Peripheral Neuropathy and Neuropathic Pain Into The Light

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Written by one of the world's leading experts on neuropathy, Professor Gérard Said, this

book is a ‘must read’ and also a handy reference book for doctors, nurses, physiotherapists,

chiropodists/podiatrists, other health professionals, and, importantly, for patients who wish

to be more informed.

As well as covering the anatomy of the nervous system and the basic pathological processes

that may affect the peripheral nerves, this book covers a whole range of neuropathic

conditions. These include, for example, Guillain‐Barré syndrome, chronic inflammatory

demyelinating polyneuropathy, vasculitic neuropathies, infectious neuropathies, diabetic and

other metabolic neuropathies, hereditary neuropathies and neuropathies in patients with

cancer.

Given the almost explosive increase in diabetes predicted over the coming years and the

high incidence of HIV infections alone, not to mention all the other possible causes of

peripheral neuropathy, no self‐respecting medical unit should be without a copy of this new

book on their shelves.

Peripheral Neuropathy & N

europathic Pain — Into the Light

Peripheral NeuropathyNeuropathic Pain&

Gérard Said

Written by one of theworld’s leading experts

The author, Professor Gérard Said, is based in the Department of Neurology atthe prestigious Hôpital de la Salpêtrière in Paris. He has devoted a lifetime tothe study of peripheral neuropathy and — alongside other great neurologicalnames — added much to the world's ever‐growing store of knowledge on thiscomplex but fascinating condition which affects so many individuals.

Into the Light

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Gérard Said MD FRCP


Into the Light

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Peripheral Neuropathy & Neuropathic Pain — Into the Light

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Contentsé~ÖÉ

Foreword î

Acknowledgements îáá

Glossary áñ

Dedication ñîááá

Chapter 1

Anatomy of the peripheral nervous system N

Chapter 2

Basic pathological processes NR

Chapter 3

Clinical manifestations and examination OP

of patients with peripheral neuropathy

Chapter 4

Guillain-Barré syndrome QN

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Chapter 5

Chronic inflammatory demyelinating polyneuropathy RR

Chapter 6

Vasculitic neuropathies ST

Chapter 7

Infectious neuropathies TT

Chapter 8

Diabetic and uraemic neuropathies NMN

Chapter 9

Neuropathies in patients with monoclonal gammopathy NOV

and malignancy

Chapter 10

Hereditary neuropathies NQN

Chapter 11

Neuropathic pain NST

Index NUP


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`~êéÉ=ÇáÉãK

Andrew KeenCEO and Founder, The Neuropathy Trust


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vii

Acknowledgements

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lìê= ãçëí= ëáåÅÉêÉ= íÜ~åâë= ~äëç= Öç= íç= káââá= _ê~ãÜáääI= aáêÉÅíçê= ~í= íÑãéìÄäáëÜáåÖI=Ñçê=ÜÉê=ÖÉåíäÉ=Äìí=Ñáêã=ÖìáÇáåÖ=Ü~åÇI=ÜÉê=íáêÉäÉëë=ïçêâ=~åÇ=ÜÉêÉåíÜìëá~ëã= ~åÇ= ÉåÅçìê~ÖÉãÉåí= áå= ÄêáåÖáåÖ= íÜáë= é~êíáÅìä~ê= éêçàÉÅí= íçÑêìáíáçåK

cáå~ääóI=ãó=éÉêëçå~ä=íÜ~åâë=Öç=íç=jêë=pìÉ=`ççâÉI=^åÖÉä~=_êççâëI=gÉ~åjìêéÜóI=~åÇ=gçóÅÉ=~åÇ=f~å=hÉÉå=ïÜçëÉ=ÉåÇäÉëë=ÉÑÑçêíë=çîÉê=íÜÉ=ä~ëí=NRóÉ~êë=Ü~îÉ=åçí=çåäó=âÉéí=qÜÉ=kÉìêçé~íÜó=qêìëí=ÖçáåÖI=Äìí=Ü~îÉ=~äëç=ÄÉÉåëç= ÜÉäéÑìä= áå= ÉåëìêáåÖ= íÜ~í= íÜáë= åÉï= éìÄäáÅ~íáçå= ÄÉÅçãÉë= ~î~áä~ÄäÉ= ~åÇ~ÅÅÉëëáÄäÉ=íç=~ääK

Andrew KeenCEO and Founder, The Neuropathy Trust


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GlossaryAcute poliomyelitis: ~å= ~ÅìíÉ= îáê~ä= ÇáëÉ~ëÉ= Å~ìëÉÇ= Äó= ~= éçäáçîáêìë= ~åÇã~êâÉÇ= ÅäáåáÅ~ääó= Äó= ÑÉîÉêI= ëçêÉ= íÜêç~íI= ÜÉ~Ç~ÅÜÉI= îçãáíáåÖI= ~åÇ= çÑíÉåëíáÑÑåÉëë=çÑ=íÜÉ=åÉÅâ=~åÇ=Ä~ÅâX=íÜÉëÉ=ã~ó=ÄÉ=íÜÉ=çåäó=ëóãéíçãë=ÉñéêÉëëÉÇáå= íÜÉ=ãáäÇ= Ñçêã=çÑ= íÜÉ= áääåÉëëK= få= íÜÉ=ëÉîÉêÉ= Ñçêã=çÑ= íÜÉ= áääåÉëëI= íÜÉêÉ= áëåÉìêçäçÖáÅ~ä=áåîçäîÉãÉåíI=~=ëíáÑÑ=åÉÅâI=éäÉçÅóíçëáë=áå=ëéáå~ä=ÑäìáÇI=~åÇ=çÑíÉåáåÑÉÅíáçå= çÑ= ÄìäÄ~êI= ãÉÇìää~ê= çê= ëéáå~ä= ãìëÅäÉ= ãçíçê= åÉìêçåëI= ïáíÜëìÄëÉèìÉåí=é~ê~äóëáë=~åÇ=~íêçéÜó=çÑ=ÅçêêÉëéçåÇáåÖ=ãìëÅäÉë=ïáíÜ=ÑêÉèìÉåíéÉêã~åÉåí=ÇÉÑçêãáíóK=oÉëéáê~íçêó= Ñ~áäìêÉ= áë=Åçããçå= êÉèìáêáåÖ=éêçäçåÖÉÇêÉëéáê~íçêó= ~ëëáëí~åÅÉK= mêÉîÉåíáçå= Äó= î~ÅÅáå~íáçå= áë= ÅçãéìäëçêóïçêäÇïáÇÉKAllodynia: ~=ÅçåÇáíáçå=áå=ïÜáÅÜ=é~áå=~êáëÉë=Ñêçã=~=ëíáãìäìë=íÜ~í=ïçìäÇ=åçíåçêã~ääó=ÄÉ=ÉñéÉêáÉåÅÉÇ=~ë=é~áåÑìäKAmyotrophy: ãìëÅìä~ê= ï~ëíáåÖ= çê= ~íêçéÜó= ïÜáÅÜ= Å~å= ÄÉ= ÇìÉ= íçÇÉåÉêî~íáçåI=ÇáëìëÉ=çê=Å~ÅÜÉñá~KAnhydrosis: ~=ÇÉÑáÅáÉåÅó=çê=~ÄëÉåÅÉ=çÑ=éÉêëéáê~íáçåKAreflexia: äçëë=çÑ=íÉåÇçå=êÉÑäÉñÉëKAscites: ~å=~ÅÅìãìä~íáçå=çÑ=ÑäìáÇ=áå=íÜÉ=éÉêáíçåÉ~ä=Å~îáíóKAstereognosia: ~å=áå~Äáäáíó=íç=áÇÉåíáÑó=çÄàÉÅíë=çê=ëÜ~éÉë=Äó=é~äé~íáçåKAtaxia: ~å= áå~Äáäáíó= íç= ÅççêÇáå~íÉ= ãìëÅäÉ= ~Åíáîáíó= ÇìêáåÖ= îçäìåí~êóãçîÉãÉåíX=íÜáë=ãçëí=çÑíÉå=êÉëìäíë=Ñêçã=ÇáëçêÇÉêë=çÑ=íÜÉ=ÅÉêÉÄÉääìã=çê=Ñêçãáãé~áêÉÇ=éêçéêáçÅÉéíáçåKAtony: äçëë=çÑ=ãìëÅäÉ=íçåÉKAxon: ~=íÜáå=ÉäçåÖ~íÉÇ=éêçÅÉëë=çÑ=~=åÉìêçå=ïÜáÅÜ=íê~åëãáíë=áãéìäëÉë=ÑêçãíÜÉ=åÉìêçåÉ=ÅÉää=ÄçÇó=íç=íÜÉ=~ñçå=íÉêãáåìëI=ïÜáÅÜ=íêáÖÖÉêë=íÜÉ=êÉäÉ~ëÉ=çÑåÉìêçíê~åëãáííÉêëKCachexia: éêçÖêÉëëáîÉ= ïÉáÖÜí= äçëëI= ~åçêÉñá~I= ~åÇ= éÉêëáëíÉåí= Éêçëáçå= çÑÄçÇó=ÅÉää=ã~ëëK=Campylobacter jejuni: ~= Ä~ÅíÉêá~ä= ëéÉÅáÉë= íÜ~í= Å~ìëÉë= ~å= ~ÅìíÉÖ~ëíêçÉåíÉêáíáë= çÑ= ëìÇÇÉå= çåëÉí= ïáíÜ= Åçåëíáíìíáçå~ä= ëóãéíçãë= Eã~ä~áëÉIãó~äÖá~I=~êíÜê~äÖá~I=~åÇ=ÜÉ~Ç~ÅÜÉF=~åÇ=Åê~ãéáåÖ=~ÄÇçãáå~ä=é~áåX=éçíÉåíá~äëçìêÅÉë=çÑ=Üìã~å=áåÑÉÅíáçå=áåÅäìÇÉ=éçìäíêó=~åÇ=Å~ííäÉK=Cardiomegaly: áåÅêÉ~ëÉÇ=îçäìãÉ=çÑ=íÜÉ=ÜÉ~êíK

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Cardiomyopathy: ~=ÇáëçêÇÉê=çÑ=íÜÉ=Å~êÇá~Å=ãìëÅäÉKCastleman disease: ~= ê~êÉ= ÇáëçêÇÉê= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~= åçåJÅ~åÅÉêçìëÖêçïíÜ=çÑ=äóãéÜçÅóíÉë=íÜ~í=ã~ó=ÇÉîÉäçé=~í=~=ëáåÖäÉ=ëáíÉ=çê=íÜêçìÖÜçìí=íÜÉÄçÇóK=qÜáë=ÇáëÉ~ëÉ=áë=ëáãáä~ê=íç=äóãéÜçã~KCauda equina syndrome: ~=ëÉêáçìë=åÉìêçäçÖáÅ~ä=ÅçåÇáíáçå=íÜ~í=ã~åáÑÉëíëïáíÜ=é~áåI=é~ê~ÉëíÜÉëá~I=~åÇ=ïÉ~âåÉëë=áåîçäîáåÖ=íÜÉ=iO=íç=pP=åÉêîÉ=êççíëã~âáåÖ= ìé= íÜÉ= Å~ìÇ~= Éèìáå~X= íÜÉêÉ= ã~ó= ~äëç= ÄÉ= Ää~ÇÇÉê= ~åÇ= ÄçïÉäëéÜáåÅíÉê=ÇóëÑìåÅíáçåKCausalgia: ~= ÄìêåáåÖ= é~áåI= çÑíÉå= ïáíÜ= íêçéÜáÅ= ëâáå= ÅÜ~åÖÉëI= ÇìÉ= íçéÉêáéÜÉê~ä=åÉêîÉ=áåàìêóK=Connective tissue disorders: ~=Öêçìé=çÑ=~ìíçáããìåÉ=ÇáëçêÇÉêë= áå=ïÜáÅÜáåÑä~ãã~íáçå=~ÑÑÉÅíë=çêÖ~åë=Åçåí~áåáåÖ=ÅçååÉÅíáîÉ=íáëëìÉKCorti organ: ëéÉÅá~äáëÉÇ= ÉéáíÜÉäáìã= áå= íÜÉ= Ñäççê= çÑ= íÜÉ= ÅçÅÜäÉ~ê= ÇìÅíÅçåí~áåáåÖ=íÜÉ=~ìÇáíçêó=êÉÅÉéíçê=ÅÉääëKCryoglubulinaemia: ~= éêÉÅáéáí~íáçå= çÑ= áããìåçÖäçÄìäáåë= ~í= äçïíÉãéÉê~íìêÉëKCutis laxa: ~å=áåÅêÉ~ëÉÇ=Éä~ëíáÅáíó=çÑ=íÜÉ=ëâáåKCytomegalovirus (CMV): ~=ãÉãÄÉê=çÑ=~=Öêçìé=çÑ= ä~êÖÉ=ëéÉÅáÉëJëéÉÅáÑáÅÜÉêéÉëJíóéÉ= îáêìëÉë= ïáíÜ= ~= ïáÇÉ= î~êáÉíó= çÑ= ÇáëÉ~ëÉ= ÉÑÑÉÅíëK= fí= Å~ìëÉëëÉêáçìë=áääåÉëë=áå=áããìåçëìééêÉëëÉÇ=éÉçéäÉ=áåÅäìÇáåÖ=íÜçëÉ=~í=~=ä~íÉ=ëí~ÖÉçÑ=áåÑÉÅíáçå=ïáíÜ=Üìã~å=áããìåçÇÉÑáÅáÉåÅó=îáêìëI=~åÇ=áå=íÜçëÉ=ÄÉáåÖ=íêÉ~íÉÇïáíÜ= áããìåçëìééêÉëëáîÉ= ÇêìÖë= ~åÇ= íÜÉê~éóI= ÉëéÉÅá~ääó= ~ÑíÉê= çêÖ~åíê~åëéä~åí~íáçåK= `js= áåÑÉÅíáçå= éêçÇìÅÉë= ìåáèìÉ= ä~êÖÉ= ÅÉääë= ïáíÜáåíê~åìÅäÉ~ê=áåÅäìëáçåëX=íÜÉ=îáêìë=Å~å=Å~ìëÉ=~=î~êáÉíó=çÑ=ÅäáåáÅ~ä=ëóåÇêçãÉëIÅçääÉÅíáîÉäó= âåçïå= ~ë= ÅóíçãÉÖ~äáÅ= áåÅäìëáçå= ÇáëÉ~ëÉI= ~äíÜçìÖÜ= ãçëíáåÑÉÅíáçåë=~êÉ=ãáäÇ=çê=ëìÄÅäáåáÅ~äKDactilitis: ~å=áåÑä~ãã~íáçå=çÑ=~å=ÉåíáêÉ=ÇáÖáíI=~ë=áå=êÜÉìã~íçáÇ=~êíÜêáíáëKDelayed-type hypersensitivity reaction: E~äëç= Å~ääÉÇ= íóéÉ= fsÜóéÉêëÉåëáíáîáíó= êÉ~ÅíáçåF= ~= ÇÉä~óÉÇ= ÅÉääJãÉÇá~íÉÇ= áããìåÉ= êÉëéçåëÉ= áåïÜáÅÜ=àQH=ÜÉäéÉê=qJäóãéÜçÅóíÉë=êÉÅçÖåáëÉ=~=ÑçêÉáÖå=~åíáÖÉå=éêÉëÉåíÉÇÄó=~=ã~ÅêçéÜ~ÖÉK=få=çêÇÉê=íç=ÖÉí=êáÇ=çÑ=áí=íÜÉó=~Åíáî~íÉ=å~íìê~ä=âáääÉê=ÅÉääëI~åíáÖÉåJëéÉÅáÑáÅ= ÅóíçíçñáÅ= äóãéÜçÅóíÉë= ~åÇ= ã~ÅêçéÜ~ÖÉëI= ïÜáÅÜ= áå= íìêåêÉäÉ~ëÉ= ÅóíçâáåÉëI= ~åÇ= Ñçêã= ãìäíáåìÅäÉ~íÉÇ= ÅÉääë= ~åÇ= Öê~åìäçã~ëK= qÜáëâáåÇ=çÑ=êÉ~Åíáçå=çÅÅìêë=áå=íìÄÉêÅìäçáÇ=äÉéêçëó=~åÇ=áå=êÉîÉêë~ä=êÉ~ÅíáçåëKDermatome: ~å=~êÉ~=çÑ=ëâáå=ëìééäáÉÇ=Äó=ëÉåëçêó=åÉìêçåë=íÜ~í=~êáëÉ=Ñêçã~=ëéáå~ä=çê=~=Åê~åá~ä=åÉêîÉ=Ö~åÖäáçåK


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Dysaesthesia: ~Äåçêã~ä=ëÉåë~íáçåKDysautonomia: ~=ÇáëíìêÄ~åÅÉ=çÑ=~ìíçåçãáÅ=ÑìåÅíáçåëKDyscrasia: ~=ÄäççÇ=ÇáëÉ~ëÉ=çê=ÇáëçêÇÉêKEndoneurium: ~=ëé~ÅÉ= äçÅ~íÉÇ= áåëáÇÉ= íÜÉ=éÉêáåÉìêáìãK= fí=Åçåí~áåë=åÉêîÉÑáÄêÉëI=ÄäççÇ=îÉëëÉäëI=Åçää~ÖÉå=ÑáÄêÉëI=ÑáÄêçÄä~ëíë=~åÇ=ÉåÇçåÉìêá~ä=ÑäìáÇKEosinophilia: ~å= áåÅêÉ~ëÉÇ= åìãÄÉê= çÑ= ÉçëáåçéÜáäáÅ= éçäóãçêéÜçåìÅäÉ~êÅÉääëKEpineurium: ÅçååÉÅíáîÉ=íáëëìÉ=ëìêêçìåÇáåÖ=åÉêîÉ=Ñ~ëÅáÅäÉëKEpiscleritis: ~å= áåÑä~ãã~íçêó= ÅçåÇáíáçå= ~ÑÑÉÅíáåÖ= íÜÉ= ÉéáëÅäÉê~ä= íáëëìÉÄÉíïÉÉå= íÜÉ= ÅçåàìåÅíáî~= EíÜÉ= ÅäÉ~ê= ãìÅçìë= ãÉãÄê~åÉ= äáåáåÖ= íÜÉ= áååÉêÉóÉäáÇë=~åÇ=ëÅäÉê~F=~åÇ=íÜÉ=ëÅäÉê~=EíÜÉ=ïÜáíÉ=é~êí=çÑ=íÜÉ=ÉóÉF=íÜ~í=çÅÅìêë=áåíÜÉ=~ÄëÉåÅÉ=çÑ=~å=áåÑÉÅíáçåK=Erythema migrans: ~å=~ååìä~ê=ê~ëÜ=ïáíÜ=ÅÉåíê~ä=ÅäÉ~êáåÖK=Fascicle: åÉêîÉ=Ñ~ëÅáÅäÉX=~=ÄìåÅÜ=çÑ=åÉêîÉ=ÑáÄêÉë=ëìêêçìåÇÉÇ=Äó=~=ä~óÉê=çÑéÉêáåÉìêá~ä=ÅÉääëK=^=åÉêîÉ=êççí=çê=~=åÉêîÉ=íêìåâ=áë=ã~ÇÉ=ìé=çÑ=ëÉîÉê~ä=åÉêîÉÑ~ëÅáÅäÉëKFasciculation: ëéçåí~åÉçìë=Åçåíê~Åíáçå=çÑ=~=Öêçìé=çÑ=ãìëÅäÉ=ÑáÄêÉëKGanglion: Ççêë~ä= êççí= Ö~åÖäáçåX= ~å= ~å~íçãáÅ= ëíêìÅíìêÉ= äçÅ~íÉÇ= áå= Ççêë~äêççíëI=Åçåí~áåáåÖ=íÜÉ=ÅÉää=ÄçÇó=çÑ=ëÉåëçêó=åÉìêçåëKGastroparesis: ÇÉä~óÉÇ=ÉãéíóáåÖ=çÑ=íÜÉ=ëíçã~ÅÜKGlucose tolerance test: ~=ãÉí~ÄçäáÅ= íÉëí= íÜ~í=ãÉ~ëìêÉë= íÜÉ=~Äáäáíó=çÑ= íÜÉÄçÇó= íç=ãÉí~ÄçäáëÉ=Å~êÄçÜóÇê~íÉëK=^=é~íáÉåí= áë=~ÇãáåáëíÉêÉÇ=~=ëí~åÇ~êÇÇçëÉ=çÑ=ÖäìÅçëÉI=~åÇ=ÄäççÇ=~åÇ=ìêáåÉ=ë~ãéäÉë=~êÉ=ãÉ~ëìêÉÇ=Ñçê=ÖäìÅçëÉäÉîÉäë=~í=éÉêáçÇáÅ= áåíÉêî~äë=ÑçääçïáåÖ=~Çãáåáëíê~íáçåK=fí= áë=ìëÉÇ=íç=~ëëáëí= áåíÜÉ=Çá~Öåçëáë=çÑ=Çá~ÄÉíÉë=ãÉääáíìëKGranuloma: ~=åçÇìä~ê=~ÖÖêÉÖ~íÉ=çÑ=ãçåçåìÅäÉ~ê=ÅÉääëKGranulomatosis with polyangiitis: ~=ê~êÉ=ÄäççÇ=îÉëëÉä=ÇáëÉ~ëÉ=ïÜáÅÜ=Å~å~ÑÑÉÅí= íÜÉ=ëáåìëÉëI= äìåÖë=~åÇ=âáÇåÉóë=~ë=ïÉää=~ë=çíÜÉê=çêÖ~åëI=ìëì~ääó= áå~ëëçÅá~íáçå= ïáíÜ= ~åíáåÉìíêçéÜáä= Åóíçéä~ëãáÅ= ~åíáÄçÇáÉë= E^k`^F= áå= ÄäççÇíÉëíëKHelper T-lymphocyte: ~=ëìÄJÖêçìé=çÑ=äóãéÜçÅóíÉëI=~=íóéÉ=çÑ=ïÜáíÉ=ÄäççÇÅÉääI= íÜ~í=éä~ó=~å= áãéçêí~åí=êçäÉ= áå=íÜÉ= áããìåÉ=ëóëíÉãI=é~êíáÅìä~êäó= áå=íÜÉ~Ç~éíáîÉ=áããìåÉ=ëóëíÉãK=qÜÉó=ÜÉäé=íÜÉ=~Åíáîáíó=çÑ=çíÜÉê=áããìåÉ=ÅÉääë=ÄóêÉäÉ~ëáåÖ= qJÅÉää= ÅóíçâáåÉëK= qÜÉó= ~êÉ= ÉëëÉåíá~ä= áå= _JÅÉää= ~åíáÄçÇó= Åä~ëëëïáíÅÜáåÖI= áå= íÜÉ= ~Åíáî~íáçå= ~åÇ= ÖêçïíÜ= çÑ= ÅóíçíçñáÅ= qJÅÉääëI= ~åÇ= áåã~ñáãáëáåÖ= Ä~ÅíÉêáÅáÇ~ä= ~Åíáîáíó= çÑ= éÜ~ÖçÅóíÉë= ëìÅÜ= ~ë= ã~ÅêçéÜ~ÖÉë= íçÑçêã=Öê~åìäçã~ëK

Glossary

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Hemiplegia: é~ê~äóëáë=çÑ=çåÉ=ëáÇÉ=çÑ=íÜÉ=ÄçÇóKHyperaesthesia: ÜóéÉêëÉåëáíáîáíóKHyperalgesia: ~å= áåÅêÉ~ëÉÇ=ëÉåëáíáîáíó= íç=é~áåI=ïÜáÅÜ=ã~ó=ÄÉ=Å~ìëÉÇ=ÄóÇ~ã~ÖÉÇ=éÉêáéÜÉê~ä=åÉêîÉëKHyperpathia: ~= é~áåÑìä= ëÉåë~íáçå= áå= êÉëéçåëÉ= íç= ~= åçêã~ääó= áååçÅìçìëëíáãìäìëK=Hyponatremia: ~=ÇÉÅêÉ~ëÉÇ=ëçÇáìã=äÉîÉä=áå=íÜÉ=ÄäççÇKHypotonia: ÇÉÅêÉ~ëÉÇ=ãìëÅäÉ=íçåÉKImmunosuppressive drugs: ÇêìÖë=íÜ~í=ÇÉÅêÉ~ëÉ=ÅÉääìä~ê=áããìåáíóKIntravenous immunoglobulin (IVIG): ~= ÄäççÇ= éêçÇìÅí= ~ÇãáåáëíÉêÉÇáåíê~îÉåçìëäóK= fí= Åçåí~áåë= íÜÉ= éççäÉÇI= éçäóî~äÉåíI= áããìåçÖäçÄìäáå= dÉñíê~ÅíÉÇ=Ñêçã=íÜÉ=éä~ëã~=çÑ=çîÉê=NMMM=ÄäççÇ=ÇçåçêëK=qÜÉ=ÉÑÑÉÅí=çÑ=fsfdëä~ëíë=ÄÉíïÉÉå=O=ïÉÉâë=~åÇ=P=ãçåíÜëKIridocyclitis: ~å=áåÑä~ãã~íáçå=çÑ=íÜÉ=áêáë=~åÇ=Åáäá~êó=ÄçÇó=çÑ=íÜÉ=ÉóÉK=qÜÉ=áêáëáë=íÜÉ=ÅçäçìêÉÇ=é~êí=çÑ=íÜÉ=ÉóÉK=qÜÉ=Åáäá~êó=ÄçÇó=áë=íÜÉ=Öêçìé=çÑ=ãìëÅäÉë~åÇ=íáëëìÉë=íÜ~í=ã~âÉ=ÑäìáÇ=áå=íÜÉ=ÉóÉ=~åÇ=Åçåíêçä=ãçîÉãÉåí=ÜÉäéáåÖ=íÜÉÉóÉ=íç=ÑçÅìëK=qÜáë=ÅçåÇáíáçå= áë=~äëç=âåçïå=~ë=ìîÉáíáë=~åÇ= áêáíáëK= fí=Å~å=ÄÉÅ~ìëÉÇ= Äó= íÜÉ= ÉóÉÛë= ÉñéçëìêÉ= íç= ÅÉêí~áå= ÅÜÉãáÅ~äë= ~åÇ= î~êáçìë~ìíçáããìåÉ=ÇáëçêÇÉêëKIxodes: ~=ÖÉåìë=çÑ=Ü~êÇJÄçÇáÉÇ=íáÅâëKLagophthalmos: ~å=áå~Äáäáíó=íç=ÅäçëÉ=íÜÉ=ÉóÉäáÇë=ÅçãéäÉíÉäó=ïÜáÅÜ=ÉñéçëÉëíÜÉ=ÉóÉ=íç=ìäÅÉê~íáçåKLepromae: ëéÉÅáÑáÅ=äÉéêçã~íçìë=ëâáå=äÉëáçåëKLeucopenia: ~=ÇÉÅêÉ~ëÉÇ=åìãÄÉê=çÑ=ïÜáíÉ=ÄäççÇ=ÅÉääëKLeukocytoclasia: ÇÉëíêìÅíáçå=çÑ=ïÜáíÉ=ÄäççÇ=ÅÉääëKLhermitte’s phenomenon: ÉäÉÅíêáÅ~ä= ëÉåë~íáçå= íÜ~í= êìåë= Ççïå= íÜÉ= Ä~Åâ~åÇ= áåíç= íÜÉ= äáãÄë= ÉäáÅáíÉÇ= Äó= ÄÉåÇáåÖ= íÜÉ= ÜÉ~Ç= Ñçêï~êÇK= lêáÖáå~ääóÇÉëÅêáÄÉÇ=áå=ãìäíáéäÉ=ëÅäÉêçëáëKLight-chain immunoglobulin: íÜÉ=ëã~ääÉê=çÑ=íÜÉ=íïç=íóéÉë=çÑ=éçäóéÉéíáÇÉÅÜ~áåë=áå=áããìåçÖäçÄìäáåëI=ÅçåëáëíáåÖ=çÑ=~å=~åíáÖÉåJÄáåÇáåÖ=éçêíáçå=ïáíÜ=~î~êá~ÄäÉ=~ãáåç=~ÅáÇ=ëÉèìÉåÅÉI=~åÇ=~=Åçåëí~åí=êÉÖáçå=ïáíÜ=~å=~ãáåç=~ÅáÇëÉèìÉåÅÉ=íÜ~í=áë=êÉä~íáîÉäó=ìåÅÜ~åÖáåÖKLymphadenitis: áåÑä~ãã~íáçå=çÑ=~=äóãéÜ=åçÇÉKLymphoedema: ëïÉääáåÖ=íÜ~í=ÇÉîÉäçéë=~ë=~=êÉëìäí=çÑ=~å=áãé~áêÉÇ=äóãéÜ~íáÅëóëíÉãK=


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Macrophages: ïÜáíÉ= ÄäççÇ= ÅÉääë= E~Åíáî~íÉÇ= ãçåçÅóíÉëF= íÜ~í= éêçíÉÅí= íÜÉÄçÇó=~Ö~áåëí=áåÑÉÅíáçå=~åÇ=ÑçêÉáÖå=ëìÄëí~åÅÉë=Äó=ÄêÉ~âáåÖ=íÜÉã=Ççïå=áåíç~åíáÖÉåáÅ=éÉéíáÇÉë=êÉÅçÖåáëÉÇ=Äó=ÅáêÅìä~íáåÖ=qJÅÉääëKMaculae: ~=ëéçí=çÑ=ëâáå=ÇáëÅçäçê~íáçåKMeningoradiculitis: ãÉåáåÖáíáë= ~ëëçÅá~íÉÇ= ïáíÜ= áåÑä~ãã~íçêó= äÉëáçåë= çÑåÉêîÉ=êççíë=áå=íÜÉ=ëìÄ~ê~ÅÜåçáÇ=ëé~ÅÉKMitochondriopathies: ëéçê~ÇáÅ= çê= áåÜÉêáíÉÇ= ãìí~íáçåë= áå= åìÅäÉ~ê= çêãáíçÅÜçåÇêá~ä= ak^= äçÅ~íÉÇ= ÖÉåÉëK= fåÜÉêáíÉÇ= ãìí~íáçåë= çÑ= ãáíçÅÜçåÇêá~äak^=~êÉ=~ëëçÅá~íÉÇ=ïáíÜ=~=î~êáÉíó=çÑ=éçäóëóëíÉãáÅ=ã~åáÑÉëí~íáçåëKMonoclonal gammopathy: ~å= áåÅêÉ~ëÉÇ= éêçÇìÅíáçå= çÑ= çåÉ= íóéÉ= çÑáããìåçÖäçÄìäáå=Äó=~=ëáåÖäÉ=ÅäçåÉ=çÑ=ÅÉääëK=qÜÉ=~Äåçêã~ä=éêçíÉáå=éêçÇìÅÉÇáë=Å~ääÉÇ=~=é~ê~éêçíÉáå=çê=~å=j=ÅçãéçåÉåí=~åÇ=ã~ó=ÄÉ=ÅçãéçëÉÇ=çÑ=ïÜçäÉáããìåçÖäçÄìäáå=ãçäÉÅìäÉë=çê=ëìÄìåáíëI=äáÖÜí=ÅÜ~áåë=çê=ÜÉ~îó=ÅÜ~áåëKMononeuritis multiplex: ã~åáÑÉëí~íáçåë=ÇìÉ=íç=åÉêîÉ=äÉëáçåë=áå=ìåêÉä~íÉÇéçêíáçåë=çÑ=íÜÉ=ÄçÇóKMorton's neuroma: ~= ëÜ~êéI= ÄìêåáåÖ= é~áåI= Åçããçåäó= ÄÉíïÉÉå= íÜÉ= PêÇ~åÇ=QíÜ=ãÉí~í~êë~ä=ÜÉ~ÇëI=ïÜáÅÜ=áë=ïçêëÉ=ïáíÜ=ÇáêÉÅí=éêÉëëìêÉ=~åÇ=ÄÉííÉêïáíÜ=êÉëíKMultinucleated giant cells: ä~êÖÉ=ÅÉääë=ïáíÜ=ëÉîÉê~ä=åìÅäÉá= êÉëìäíáåÖ= ÑêçãíÜÉ= Ñìëáçå= çÑ= ~Åíáî~íÉÇ= ã~ÅêçéÜ~ÖÉë= áå= íÜÉ= ëÉííáåÖ= çÑ= ~= ÇÉä~óÉÇJíóéÉÜóéÉêëÉåëáíáîáíó=êÉ~ÅíáçåKMultiple sclerosis (MS): ~å= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ= ~ìíçáããìåÉÇáëçêÇÉê=~ÑÑÉÅíáåÖ=ãçîÉãÉåíI=ëÉåë~íáçåI=~åÇ=ÄçÇáäó=ÑìåÅíáçåëK=fí=áë=Å~ìëÉÇÄó=ÇÉëíêìÅíáçå=çÑ=íÜÉ=ãóÉäáå=áåëìä~íáçå=ÅçîÉêáåÖ=åÉêîÉ=ÑáÄêÉë=áå=íÜÉ=ÅÉåíê~äåÉêîçìë=ëóëíÉã=~åÇ=çéíáÅ=åÉêîÉëK=Muscle spindles: ëéÉÅá~äáëÉÇ=ëÉåëçêó=ëíêìÅíìêÉë=ïáíÜáå=ëâÉäÉí~ä=ãìëÅäÉëIÅçåëáëíáåÖ= çÑ= ëã~ää= ãìëÅäÉ= ÑáÄêÉë= ïÜáÅÜ= Çç= åçí= ÅçåíêáÄìíÉ= íç= éçïÉêÖÉåÉê~íáçåI= Äìí= é~êíáÅáé~íÉ= áå= ãìëÅäÉ= Åçåíêçä= Äó= ÉåëìêáåÖ= ~= ÅçåíáåìçìëëÉåëçêó=ÑÉÉÇÄ~ÅâKMydriasis: ÉñÅÉëëáîÉ=Çáä~í~íáçå=çÑ=íÜÉ=éìéáäKMyopathy: ~=ãìëÅäÉ=ÇáëçêÇÉê=çÑ=~=ÖÉåÉíáÅ=çê=ãÉí~ÄçäáÅ=çêáÖáåKNecrosis: íáëëìÉ=ÇÉëíêìÅíáçåKNeuritis: ~å=áåÑä~ãã~íçêó=äÉëáçå=çÑ=~=åÉêîÉKNeurofibroma: ~=ÄÉåáÖåI=åçåJÉåÅ~éëìä~íÉÇ= íìãçìê= íÜ~í= êÉëìäíë= Ñêçã=íÜÉÇáëçêÇÉêäó= éêçäáÑÉê~íáçå= çÑ= pÅÜï~åå= ÅÉääë= ~åÇ= íÜ~í= áåÅäìÇÉë= éçêíáçåë= çÑåÉêîÉ=ÑáÄêÉëK

Glossary

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Neurolymphomatosis: áåÑáäíê~íáçå= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉã= ÄóäóãéÜçã~=~åÇ=åçåJíìãçêçìë=äóãéÜçÅóíÉëK=Nicotinic receptors: êÉÅÉéíçêë=íÜ~í=~êÉ=ëíáãìä~íÉÇ= áåáíá~ääó=~åÇ=ÄäçÅâÉÇ=~íÜáÖÜ=ÇçëÉë=Äó= íÜÉ=~äâ~äçáÇI=åáÅçíáåÉX= íÜÉó=~êÉ=ã~áåäó= ÑçìåÇ=çå=~ìíçã~íáÅÖ~åÖäáçå=ÅÉääëKNodes of Ranvier: ~=ëÜçêí= áåíÉêî~ä= áå= íÜÉ=ãóÉäáå=ëÜÉ~íÜ=çÑ=~=åÉêîÉ= ÑáÄêÉIçÅÅìêêáåÖ=ÄÉíïÉÉå=É~ÅÜ=íïç=ëìÅÅÉëëáîÉ=ëÉÖãÉåíë=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜX~í= íÜÉ= åçÇÉI= íÜÉ= ~ñçå= áë= áåîÉëíÉÇ= çåäó= Äó= ëÜçêíI= ÑáåÖÉêJäáâÉ= Åóíçéä~ëãáÅéêçÅÉëëÉë=çÑ=íÜÉ=íïç=åÉáÖÜÄçìêáåÖ=pÅÜï~åå=ÅÉääëKNucleus pulposus: íÜÉ= ÅÉåíê~ä= éçêíáçå= çÑ= íÜÉ= áåíÉêîÉêíÉÄê~ä= Çáëâ= íÜ~í= áëã~ÇÉ=ìé=çÑ=~=ÖÉä~íáåçìë=ëìÄëí~åÅÉKOnconeural antibodies: çåÅçåÉìê~ä= ~åíáÄçÇáÉë= ~êÉ= ÇáêÉÅíÉÇ= ~Ö~áåëíáåíê~ÅÉääìä~ê=~åíáÖÉåëK=qÜÉó=~êÉ=ÜáÖÜäó=ëéÉÅáÑáÅ=ã~êâÉêë=çÑ=~=é~ê~åÉçéä~ëíáÅ~ÉíáçäçÖó=áå=é~íáÉåíë=ïáíÜ=åÉìêçäçÖáÅ~ä=ëóãéíçãëKOphthalmoplegia: é~ê~äóëáë=çÑ=çÅìäçãçíçê=ãìëÅäÉëKOrchitis: ~å=áåÑä~ãã~íáçå=çÑ=íÜÉ=íÉëíÉëKOsteoarthropathy: ~=ÇáëçêÇÉê=~ÑÑÉÅíáåÖ=ÄçåÉë=~åÇ=àçáåíëKOsteolysis: Çáëëçäìíáçå=çê=ÇÉÖÉåÉê~íáçå=çÑ=ÄçåÉ=íáëëìÉK=Osteophytosis: ~Äåçêã~ä=éêçÇìÅíáçå=çÑ=ÄçåÉ=~ë=áå=~êíÜêçëáëKPancoast tumour: íìãçìêë=íÜ~í=Ñçêã=~í=íÜÉ=ÉñíêÉãÉ=íçé=çÑ=ÉáíÜÉê=íÜÉ=êáÖÜíçê=äÉÑí=äìåÖK=qÜÉó=íÉåÇ=íç=áåî~ÇÉ=äçïÉê=êççíë=çÑ=íÜÉ=Äê~ÅÜá~ä=éäÉñìë=~åÇ=íÜÉëóãé~íÜÉíáÅ=ÅÜ~áåKPandysautonomia: ëóãéíçãë=çÑ=ïáÇÉëéêÉ~Ç=~åÇ=ëÉîÉêÉ=ëóãé~íÜÉíáÅ=~åÇé~ê~ëóãé~íÜÉíáÅ=Ñ~áäìêÉKPapilloedema: çéíáÅ=ÇáëÅ=ëïÉääáåÖ=íÜ~í=áë=Å~ìëÉÇ=Äó=áåÅêÉ~ëÉÇ=áåíê~Åê~åá~äéêÉëëìêÉK=Paraesthesia: é~áåäÉëë=ëéçåí~åÉçìë=ëÉåë~íáçåKParaneoplastic: ÅÜ~åÖÉë=éêçÇìÅÉÇ=áå=íáëëìÉ=êÉãçíÉ=Ñêçã=~=íìãçìê=çê=áíëãÉí~ëí~ëÉëKPericarditis: áåÑä~ãã~íáçå=çÑ=íÜÉ=éÉêáÅ~êÇáìãI=íÜÉ=çìíÉê=ÑáÄêçìë=ÉåîÉäçéÉ=çÑíÜÉ=ÜÉ~êíKPerineurium: íÜÉ= ÅçååÉÅíáîÉ= íáëëìÉ= ëÜÉ~íÜ= ëìêêçìåÇáåÖ= É~ÅÜ= ÄìåÇäÉ= çÑåÉêîÉ=ÑáÄêÉë=EÑ~ëÅáÅäÉF=áå=~=éÉêáéÜÉê~ä=åÉêîÉK=Peripheral neuropathy: ëáÖåë= ~åÇ= ëóãéíçãë= êÉä~íÉÇ= íç= äÉëáçåë= çÑ= íÜÉéÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉãKPerivascular cuffing: ïÜáíÉ=ÅÉääë=ëìêêçìåÇáåÖ=ëã~ää=ÄäççÇ=îÉëëÉäëK


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Plasmacytoma: ~= ÑçÅ~ä= åÉçéä~ëã= Åçåí~áåáåÖ= éä~ëã~= ÅÉääë= íÜ~í= ã~óÇÉîÉäçé=áå=íÜÉ=ÄçåÉ=ã~êêçïI=~ë=áå=ãìäíáéäÉ=ãóÉäçã~I=çê=çìíëáÇÉ=íÜÉ=ÄçåÉã~êêçïI=~ë=áå=íìãçìêë=çÑ=íÜÉ=îáëÅÉê~KPlasmid: ~=ëã~ääI=ÅáêÅìä~êI=ÇçìÄäÉJëíê~åÇÉÇ=ak^=ãçäÉÅìäÉ=íÜ~í= áë=ÇáëíáåÅíÑêçã=~=ÅÉääÛë=ÅÜêçãçëçã~ä=ak^K=mä~ëãáÇë=å~íìê~ääó=Éñáëí=áå=Ä~ÅíÉêá~ä=ÅÉääëKPleocytosis: íÜÉ=éêÉëÉåÅÉ=çÑ=~=ÖêÉ~íÉê=åìãÄÉê=çÑ=ÅÉääë=íÜ~å=åçêã~äI=~ë=áåíÜÉ=ÅÉêÉÄêçëéáå~ä=ÑäìáÇKPoikilodermia: ÑçÅ~ä=~íêçéÜó=~åÇ=íÉä~åÖáÉÅí~ëáÉë=çÑ=íÜÉ=ëâáåKPolyarteritis nodosa: ~=î~ëÅìä~ê=ÇáëÉ~ëÉ=íÜ~í=~ÑÑÉÅíë=íÜÉ=Åçää~ÖÉå=áå=ëã~ääJ~åÇ=ãÉÇáìãJëáòÉÇ=ÄäççÇ=îÉëëÉäë=áåÇìÅáåÖ=åÉÅêçëáë=çÑ=íÜÉ=~êíÉêá~ä=ï~ää=~åÇäìãÉå= çÅÅäìëáçå= ïáíÜ= ëìÄëÉèìÉåí= áëÅÜ~ÉãáÅ= äÉëáçåë= çÑ= ~ÑÑÉÅíÉÇ= çêÖ~åëKmçäóëóëíÉãáÅ= ~åÇ= Åçåëíáíìíáçå~ä= ã~åáÑÉëí~íáçåë= áåÅäìÇÉ= ãìäíáÑçÅ~äåÉìêçé~íÜóI=ëâáå=äÉëáçåëI=Ö~ëíêçáåíÉëíáå~ä=äÉëáçåë=~åÇ=âáÇåÉó=äÉëáçåëK=içëëçÑ= ïÉáÖÜíI= ÑÉîÉê= ~åÇ= ~êíÜêáíáë= ~êÉ= ~äëç= ÅçããçåK= aá~Öåçëáë= êÉëíë= çåÜáëíçäçÖáÅ~ä= ÇÉãçåëíê~íáçå= çÑ= î~ëÅìäáíáë= áå= íáëëìÉ= Äáçéëó= ëéÉÅáãÉåëKjçêí~äáíó=ï~ë=ÜáÖÜ=ÄÉÑçêÉ=íÜÉ=ìëÉ=çÑ=ÅçêíáÅçëíÉêçáÇëKPolycythaemia: ~å=áåÅêÉ~ëÉÇ=åìãÄÉê=çÑ=ÄäççÇ=ÅÉääëKProprioception: íÜÉ=~Äáäáíó=íç=ëÉåëÉ=ÄçÇó=éçëáíáçåI=éçëíìêÉI=Ä~ä~åÅÉI=~åÇãçíáçåKPseudoathetotic movements: ëäçï=áåîçäìåí~êó=ãçîÉãÉåíë=çÑ=íÜÉ=ÑáåÖÉêëçÅÅìêêáåÖ=~í=êÉëíI=~ëëçÅá~íÉÇ=ïáíÜ=~=éêçÑçìåÇ=äçëë=çÑ=éêçéêáçÅÉéíáçåKPtosis: ÇêççéáåÖ=çÑ=íÜÉ=ÉóÉäáÇKRetinitis: áåÑä~ãã~íáçå=çÑ=íÜÉ=êÉíáå~KRheumatoid arthritis: ~= ÅÜêçåáÅ= ~ìíçáããìåÉ= áåÑä~ãã~íçêó= ÇáëÉ~ëÉ= íÜ~íÅ~ìëÉë= áåÑä~ãã~íáçå= ~åÇ= ÇÉÑçêãáíó= çÑ= íÜÉ= àçáåíëK= m~áåI= ëïÉääáåÖI= ~åÇëíáÑÑåÉëë= áå= íÜÉ= àçáåíëI= ãçëí= çÑíÉå= áåîçäîáåÖ= íÜÉ= Ü~åÇëI= ~êÉ= íÜÉ= ã~áåã~åáÑÉëí~íáçåë=~í=çåëÉíK=Ribosome: ~= Öê~åìäÉ= ÑçêãÉÇ= çÑ= êáÄçåìÅäÉçéêçíÉáåX= ~= ëáíÉ= çÑ= éêçíÉáåëóåíÜÉëáëI=ìåÇÉê=íÜÉ=áåÑäìÉåÅÉ=çÑ=íÜÉ=åìÅäÉ~ê=ãÉëëÉåÖÉêI=êáÄçåìÅäÉáÅ=~ÅáÇKRomberg’s sign: ~=äçëë=çÑ=Ä~ä~åÅÉ=ïÜÉå=íÜÉ=ÉóÉë=~êÉ=ÅäçëÉÇKSarcoidosis: ~=ÇáëÉ~ëÉ=~ëëçÅá~íÉÇ=ïáíÜ=Öê~åìäçã~ë=íÜ~í=áåî~êá~Ääó=~ÑÑÉÅíëíÜÉ=äìåÖëK=få=~=ãáåçêáíó=çÑ=é~íáÉåíë=ë~êÅçáÇçëáë=Å~å=ÄÉÅçãÉ=éçäóëóëíÉãáÅ~åÇ= áåîçäîÉ= çíÜÉê= çêÖ~åë= áåÅäìÇáåÖ= ãìëÅäÉë= ~åÇ= íÜÉ= ÅÉåíê~ä= åÉêîçìëëóëíÉãKSchmidt-Lanterman incisures: ÑìååÉäJëÜ~éÉÇ= áåíÉêêìéíáçåë= áå= íÜÉ= êÉÖìä~êëíêìÅíìêÉ=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜ=çÑ=åÉêîÉ=ÑáÄêÉëK

Glossary

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Schwann cells: ÅÉääë=íÜ~í=Ñçêã=~=Åçåíáåìçìë=ÉåîÉäçéÉ=~êçìåÇ=É~ÅÜ=åÉêîÉÑáÄêÉ= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîÉëK= ^= pÅÜï~åå= ÅÉää= ÉåÑçäÇë= çåÉ= çê= ãçêÉìåãóÉäáå~íÉÇ= ~ñçåëK= pÅÜï~åå= ÅÉääë= éêçÇìÅÉ= ~= ãÉãÄê~åçìë= Éñé~åëáçåíÜ~í=ïáåÇë=~êçìåÇ=ä~êÖÉê=~ñçåë=íç=Ñçêã=íÜÉ=ãóÉäáå=ëÜÉ~íÜKSchwannoma: ~= åÉçéä~ëã= çêáÖáå~íáåÖ= Ñêçã= pÅÜï~åå= ÅÉääëK= qÜÉó= ãçëíäóçÅÅìê=áå=íÜÉ=ëâáåI=Äìí=íÜÉó=~êÉ=~äëç=ÑçìåÇ=áå=åÉêîÉ=íêìåâë=~åÇ=êççíëKSpastic paraparesis: ïÉ~âåÉëë=çÑ=íÜÉ=äçïÉê=äáãÄë=ïáíÜ=áåÅêÉ~ëÉÇ=ãìëÅäÉíçåÉ=~åÇ=íÉåÇçå=êÉÑäÉñÉë=áå=êÉä~íáçå=íç=äÉëáçåë=çÑ=íÜÉ=éóê~ãáÇ~ä=íê~Åí=áå=íÜÉÅÉåíê~ä=åÉêîçìë=ëóëíÉãK=Spirochaetes: Ä~ÅíÉêá~=íÜ~í=Ü~îÉ=~=ÇáëíáåÅíáîÉ=ëéáê~ä=ëÜ~éÉKSyringomyelia: ~= ÇáëçêÇÉê= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= Ç~ã~ÖÉ= íç= íÜÉ= ëéáå~ä= ÅçêÇIÅ~ìëÉÇ=Äó= Ñçêã~íáçå=çÑ=~= ÑäìáÇJÑáääÉÇ=Å~îáíó=ïáíÜáå= íÜÉ=ëéáå~ä=ÅçêÇI=ÇìÉ= íçíê~ìã~I= íìãçìêëI=çê=ÅçåÖÉåáí~ä=ÇÉÑÉÅíëX= íÜÉ=Å~îáíó=ÄÉÖáåë= áå= íÜÉ=ÅÉêîáÅ~äêÉÖáçå=~åÇ=ëäçïäó=Éñé~åÇëK=fí=êÉëìäíë=áå=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáíë=ÅÜ~ê~ÅíÉêáëÉÇÄó= ~= ÇáëëçÅá~íÉÇ= ëÉåëçêó= äçëë= Eäçëë= çÑ= é~áå= ~åÇ= íÉãéÉê~íìêÉ= ëÉåë~íáçåIïáíÜ=éêÉëÉêî~íáçå=çÑ= íÜÉ=ëÉåëÉ=çÑ= íçìÅÜFI=ëÉÖãÉåí~ä=ãìëÅìä~ê=ïÉ~âåÉëë~åÇ=~íêçéÜóK=qÜçê~ÅáÅ=ëÅçäáçëáë=áë=çÑíÉå=éêÉëÉåíKSystemic lupus erythematosus (SLE): ~å= ~ìíçáããìåÉ= ëóëíÉãáÅ= ÇáëÉ~ëÉíÜ~í=Å~å=~ÑÑÉÅí=îáêíì~ääó=~åó=çêÖ~åK=pib=ãçëí=çÑíÉå=Ü~êãë=íÜÉ=àçáåíëI=ëâáåIÄäççÇ=îÉëëÉäëI=âáÇåÉóI=éÉêáÅ~êÇáìã=~åÇ=íÜÉ=åÉêîçìë=ëóëíÉãK=qÜÉ=ÅçìêëÉ=çÑíÜÉ=ÇáëÉ~ëÉ=áë=ìåéêÉÇáÅí~ÄäÉK=qÜÉ=ÇáëÉ~ëÉ=çÅÅìêë=åáåÉ=íáãÉë=ãçêÉ=çÑíÉå=áåïçãÉå=íÜ~å=áå=ãÉåK=T2-weighted images: ~= íÉÅÜåáèìÉ= çÑ= ã~ÖåÉíáÅ= êÉëçå~åÅÉ= áã~ÖáåÖ= íÜ~íáÇÉåíáÑáÉë=ÜóéÉêëáÖå~äë= áå= íÜÉ=ïÜáíÉ=ã~ííÉê=çÑ= íÜÉ=Äê~áå= áå=ãçëí=Å~ëÉë=çÑãìäíáéäÉ=ëÅäÉêçëáëK=T-cells: àQH=ÜÉäéÉê=qJäóãéÜçÅóíÉë=íÜ~í=~êÉ= áåîçäîÉÇ=áå=íÜÉ=ÇÉä~óÉÇJíóéÉÜóéÉêëÉåëáíáîáíó=êÉ~ÅíáçåKThrombocytosis: ~å=áåÅêÉ~ëÉÇ=åìãÄÉê=çÑ=íÜêçãÄçÅóíÉë=áå=íÜÉ=ÄäççÇKTransverse myelitis: ~å=~ÅìíÉ=~íí~Åâ=çÑ=ëéáå~ä=ÅçêÇ=áåÑä~ãã~íáçå=êÉëìäíáåÖáå=ÅçãéäÉíÉ=é~ê~éäÉÖá~KTropism: ~ííê~ÅíáçåKType 1 diabetes mellitus: ~å= ~ìíçáããìåÉ= ÇáëÉ~ëÉ= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~åáå~Äáäáíó=íç=ãÉí~ÄçäáëÉ=Å~êÄçÜóÇê~íÉëI=éêçíÉáåI=~åÇ=Ñ~í=ÄÉÅ~ìëÉ=çÑ=~ÄëçäìíÉáåëìäáå=ÇÉÑáÅáÉåÅóK=qóéÉ=N=Çá~ÄÉíÉë=Å~å=çÅÅìê=~í=~åó=~ÖÉI=Äìí=áíë=áåÅáÇÉåÅÉáë=ãçêÉ=Åçããçå=áå=ÅÜáäÇêÉåK=råÅçåíêçääÉÇ=íóéÉ=N=Çá~ÄÉíÉë=áë=ÅÜ~ê~ÅíÉêáëÉÇÄó=ÉñÅÉëëáîÉ=íÜáêëíI=áåÅêÉ~ëÉÇ=ìêáå~íáçåI=~å=áåÅêÉ~ëÉÇ=ÇÉëáêÉ=íç=É~íI=äçëë=çÑïÉáÖÜíI=ÇáãáåáëÜÉÇ=ëíêÉåÖíÜI=~åÇ=ã~êâÉÇ=áêêáí~ÄáäáíóK

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Type 2 diabetes: Çá~ÄÉíÉë=ãÉääáíìëI=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=~= ä~íÉ=~ÖÉ=çÑ=çåëÉíEPM=óÉ~êë=çê=çäÇÉêFI=áåëìäáå=êÉëáëí~åÅÉI=ÜáÖÜ=äÉîÉäë=çÑ=ÄäççÇ=ëìÖ~êI=~åÇ=äáííäÉçê=åç=åÉÉÇ=Ñçê=ëìééäÉãÉåí~ä=áåëìäáåKUveitis: ~å=áåÑä~ãã~íáçå=çÑ=íÜÉ=ãáÇÇäÉ=ä~óÉê=çÑ=íÜÉ=ÉóÉI=Å~ääÉÇ=íÜÉ=ìîÉ~=çêìîÉ~ä=íê~ÅíKVascular endothelial growth factor (VEGF): ~= éÉéíáÇÉ= êÉäÉ~ëÉÇ= Ñêçãî~ëÅìä~ê= ÉåÇçíÜÉäá~ä= ÅÉääë= áå= êÉëéçåëÉ= íç= Üóéçñá~I= áëÅÜ~Éãá~I= çêÜóéçÖäóÅ~Éãá~K=sbdc=éêçãçíÉë=éêçäáÑÉê~íáçå=çÑ=ÄäççÇ=îÉëëÉäëK=sbdc= áëêÉäÉ~ëÉÇ=íç=ã~áåí~áå=íÜÉ=ëìêîáî~ä=çÑ=íÜÉ=ãáÅêçî~ëÅìä~íìêÉ=çÑ=~=íáëëìÉKVasculitis: áåÑä~ãã~íçêó= äÉëáçåë= çÑ= ÄäççÇ= îÉëëÉä= ï~ääë= äÉ~ÇáåÖ= íçÇÉëíêìÅíáçå=çÑ=íÜÉ=îÉëëÉä=ï~ää=~åÇ=çÅÅäìëáçå=çÑ=íÜÉ=äìãÉåKVoltage-gated channels: áçå=ÅÜ~ååÉäë=íÜ~í=çéÉå=~åÇ=ÅäçëÉ=áå=êÉëéçåëÉ=íç~=ÅÜ~åÖÉ= áå= íÜÉ=ÉäÉÅíêáÅ~ä=éçíÉåíá~ä= ~Åêçëë= íÜÉ=éä~ëã~=ãÉãÄê~åÉ=çÑ= íÜÉÅÉääX= îçäí~ÖÉJÖ~íÉÇ=ëçÇáìã=ÅÜ~ååÉäë=~êÉ= áãéçêí~åí= Ñçê=ÅçåÇìÅíáåÖ=~ÅíáçåéçíÉåíá~äë=~äçåÖ=åÉêîÉ=ÅÉää=éêçÅÉëëÉëK

Glossary

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This book is dedicated to the memory of

Miss Dawn Louise Ind,

Mrs Olive Briggs

and Miss Dorothea Klyne.

All were neuropathy patients with a

vision of a better future for others.

Dedication

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1

Anatomy of the peripheral nervous system

Chapter 1

Overview

This chapter is about the different components and functions of the peripheral

nervous system which links the central nervous system (brain and spinal cord) to

muscles and to sensory receptors for activation of muscle contraction and

perception of sensation. The anatomy, motor and sensory territories supplied by

plexuses and nerve trunks, and cranial nerves are detailed. The anatomy and

function of the sympathetic and parasympathetic nervous systems are also

outlined. Microscopic anatomy details the morphology and role of myelinated and

unmyelinated nerve fibres.

Introduction

qÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=EmkpF=Ü~ë=íïç=ÅçãéçåÉåíëW=íÜÉ=ëçã~íáÅéÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=ïÜáÅÜ= áåÅäìÇÉë=ëÉåëçêó=~åÇ=ãçíçê=åÉìêçåë=~åÇåÉêîÉ= ÑáÄêÉëI= ~åÇ= íÜÉ= ~ìíçåçãáÅ= åÉêîçìë= ëóëíÉã= ïÜáÅÜ= ÅçãéêáëÉë= íÜÉëóãé~íÜÉíáÅ=~åÇ=é~ê~ëóãé~íÜÉíáÅ=ëóëíÉãë=NK=qÜÉ=ëçã~íáÅ=éÉêáéÜÉê~ä=åÉêîçìëëóëíÉã=Åçåíêçäë=ãìëÅäÉ=Åçåíê~Åíáçå=~åÇ=éÉêÅÉéíáçå=çÑ=ëÉåë~íáçåI=ïÜáäÉ= íÜÉ~ìíçåçãáÅ=åÉêîçìë=ëóëíÉã=ïçêâë=~ìíçã~íáÅ~ääó=ïáíÜçìí=ÅçåëÅáçìë=ÅçåíêçäK

Somatic peripheral nervous system

b~ÅÜ=ëéáå~ä=åÉêîÉ=êÉëìäíë=Ñêçã=íÜÉ=Ñìëáçå=çÑ=íïç=êççíëK=qÜÉ=îÉåíê~ä=êççíáë= ÑçêãÉÇ= Äó= íÜÉ= ~ÖÖêÉÖ~íáçå= çÑ= ëéáå~ä= ãçíçê= êççíäÉíë= Eãçíçê= åÉìêçå

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~ñçåëFK=qÜÉ=ÅÉää=ÄçÇóI=çê=éÉêáâ~êóçåI=çÑ= íÜÉ=ãçíçê= ÑáÄêÉ= áë= äçÅ~íÉÇ= áå= íÜÉ~åíÉêáçê=Üçêå=çÑ=íÜÉ=ëéáå~ä=ÅçêÇK=qÜÉ=Ççêë~ä=êççí=Åçåí~áåë=íÜÉ=Ççêë~ä=êççíÖ~åÖäáçåI=ïÜÉêÉ=ÅÉää=ÄçÇáÉë=çÑ=~ää=ëÉåëçêó=åÉìêçåë=çÑ=íÜÉ=mkp=~êÉ=ÑçìåÇKqÜÉëÉ=íïç=êççíë=ìåáíÉ=íç=Ñçêã=íÜÉ=ãáñÉÇ=ëéáå~ä=åÉêîÉK=qÜÉêÉ=~êÉ=ÉáÖÜí=é~áêëçÑ=ÅÉêîáÅ~ä=ëéáå~ä=åÉêîÉëI=íïÉäîÉ=é~áêë=çÑ=íÜçê~ÅáÅ=ëéáå~ä=åÉêîÉëI=ÑáîÉ=é~áêëçÑ=äìãÄ~ê=ëéáå~ä=åÉêîÉëI=ÑáîÉ=é~áêë=çÑ=ë~Åê~ä=ëéáå~ä=åÉêîÉë=~åÇ=~=ÑÉï=é~áêëçÑ=ÅçÅÅóÖÉ~ä=åÉêîÉëK=

Cervical plexus

qÜÉ= ãçíçê= ÑáÄêÉ= ÉåÇáåÖë= çÑ= íÜÉ= ÅÉêîáÅ~ä= éäÉñìë= áååÉêî~íÉ= ÅÉêîáÅ~äãìëÅäÉë=~åÇ=íÜÉ=Çá~éÜê~ÖãK=póãé~íÜÉíáÅ=ëìÇçãçíçê=~åÇ=î~ëçãçíçê=ÑáÄêÉëé~ëë=íÜêçìÖÜ=íÜáë=éäÉñìë=íç=ÄäççÇ=îÉëëÉäë=~åÇ=Öä~åÇëK=

Brachial plexus

qÜÉ=Äê~ÅÜá~ä=éäÉñìë=áë=ÑçêãÉÇ=Äó=íÜÉ=~ëëÉãÄäó=çÑ=`RI=`SI=`TI=Ù=~åÇqN=îÉåíê~ä=êççíëK=qÜêÉÉ=íêìåâë=EëìéÉêáçêI=ãáÇÇäÉ=~åÇ=áåÑÉêáçêF=çêáÖáå~íÉ=ÑêçãíÜÉ=Äê~ÅÜá~ä=éäÉñìëK=b~ÅÜ=çÑ=íÜÉëÉ=íêìåâë=ÇáîáÇÉë=áåíç=~=îÉåíê~ä=~åÇ=~=Ççêë~äÄê~åÅÜ=~åÇ=íÜêÉÉ=ÅçêÇë=~êÉ=ÑçêãÉÇ=Eä~íÉê~äI=éçëíÉêáçê=~åÇ=ãÉÇá~äFI=ïÜáÅÜÑáå~ääó=äÉ~Ç=íç=íÜÉ=ëéáå~ä=åÉêîÉë=çÑ=íÜÉ=ìééÉê=äáãÄëK=qÜÉ=ëìéÉêáçê=Äê~ÅÜá~äéäÉñìë= áë= îÉêó= îìäåÉê~ÄäÉ= ÇìêáåÖ= ÄáêíÜ= íê~ìã~= ïáíÜ= ëìÄëÉèìÉåí= ÇÉäíçáÇIÄáÅÉéëI= Äê~ÅÜá~äáë= ~åÇ= Äê~ÅÜáçê~Çá~äáë= é~ê~äóëáëK= içïÉê= éäÉñìëÅçãéêÉëëáçå=Äó=~=ÅÉêîáÅ~ä=êáÄ=EÙI=qNFI=çê= áåÑáäíê~íáçå=Äó=ÅÉääë=çêáÖáå~íáåÖÑêçã=~=ÄêÉ~ëí=çê=éìäãçå~êó=Å~åÅÉêI=ã~ó=Å~ìëÉ=é~ê~äóëáë=çÑ=íÜÉ=ëã~ää=Ü~åÇãìëÅäÉëK

Nerves of the upper limbs

qÜÉ=åÉêîÉë=çÑ=íÜÉ=ìééÉê=äáãÄë=~êÉ=~ë=ÑçääçïëW

√ íÜÉ= ãìëÅìäçÅìí~åÉçìë= åÉêîÉ= E`RI= `SF= áååÉêî~íÉë= ãìëÅäÉëéêÉÇçãáå~åíäó=áåîçäîÉÇ=áå=ÑäÉñáçå=çÑ=íÜÉ=~êãX=

√ íÜÉ= ëÅ~éìä~ê= åÉêîÉ= E`RF= áååÉêî~íÉë= ãìëÅäÉë= Ñçê= ÉäÉî~íáçå= ~åÇ~ÇÇìÅíáçå=çÑ=íÜÉ=ëÅ~éìä~X


2


√ íÜÉ=ëìéê~ëÅ~éìä~ê=åÉêîÉ=E`RJ`SF=áååÉêî~íÉë=íÜÉ=ëìéê~ëéáå~íìë=~åÇáåÑê~ëéáå~íìë= ãìëÅäÉë= Ñçê= äáÑí= ~åÇ= çìíï~êÇ= êçí~íáçå= çÑ= íÜÉ= ~êãIêÉëìäíáåÖ=áå=~ÄÇìÅíáçå=çÑ=NRø=~åÇ=ÉñíÉêå~ä=êçí~íáçå=çÑ=íÜÉ=~êãX

√ íÜÉ= ~ñáää~êó= åÉêîÉ= E`RJ`SF= áååÉêî~íÉë= íÜÉ= ÇÉäíçáÇ= ~åÇ= íÉêÉë= ãáåçêãìëÅäÉë= Ñçê= ~ÄÇìÅíáçå= çÑ= íÜÉ= ~êã= íç= íÜÉ= Üçêáòçåí~ä= ~åÇ= çìíï~êÇêçí~íáçå=çÑ=íÜÉ=~êãX=

√ íÜÉ= ê~Çá~ä= åÉêîÉ= E`SJÙF= áååÉêî~íÉë= íÜÉ= íêáÅÉéëI= ~åÅçåÉìëIÄê~ÅÜáçê~Çá~äáëI= ÉñíÉåëçê= Å~êéá= ê~Çá~äáëI= ÉñíÉåëçê= ÇáÖáíçêìã= ~åÇëìéáå~íçê= ãìëÅäÉë= Ñçê= ÉñíÉåëáçå= ~åÇ= ÑäÉñáçå= çÑ= íÜÉ= ÉäÄçï= ~åÇëìéáå~íáçå=çÑ= íÜÉ=ÑçêÉ~êãX=ãìëÅäÉë=Ñçê= íÜÉ=ÉñíÉåëáçå=~åÇ=ÑäÉñáçå=çÑíÜÉ= ÉäÄçïI= ëìéáå~íáçå= çÑ= íÜÉ= ÑçêÉ~êãI= ÉñíÉåëáçå= çÑ= íÜÉ= ïêáëí= ~åÇÑáåÖÉêëI=~åÇ=~ÄÇìÅíáçå=çÑ= íÜÉ= íÜìãÄK=pÉåëçêó= áååÉêî~íáçå= áë= íç= íÜÉéçëíÉêáçê= ìééÉê= ~êã= ~åÇ= ÑçêÉ~êãI= ~åÇ= íÜÉ= éçëíÉêáçê= íÜìãÄ= ~åÇä~íÉê~ä=O½=ÑáåÖÉêëX

√ íÜÉ=ãÉÇá~å=åÉêîÉ=E`RJqNF=áååÉêî~íÉë=ãìëÅäÉë=áåîçäîÉÇ=áå=ÑäÉñáçå=çÑíÜÉ=ÑáåÖÉêëI=~ÄÇìÅíáçå=~åÇ=çééçëáíáçå=çÑ=íÜÉ=íÜìãÄI=~åÇ=éêçå~íáçå=çÑíÜÉ=ÑçêÉ~êãK=pÉåëçêó=áåååÉêî~íáçå=áë=íç=íÜÉ=é~äã=~åÇ=ÑáåÖÉêë=N=íç=P~åÇ=íÜÉ=ä~íÉê~ä=Ü~äÑ=çÑ=íÜÉ=ÑçìêíÜ=ÑáåÖÉêX

√ íÜÉ= ìäå~ê= åÉêîÉ= EÙJqNF= áååÉêî~íÉë= ãìëÅäÉë= ÅçåíêçääáåÖ= ~ÄÇìÅíáçå~åÇ=~ÇÇìÅíáçå=çÑ= íÜÉ= ÑáåÖÉêë=~åÇ=ïêáëí= ÑäÉñáçåX=ëÉåëçêó= áååÉêî~íáçåëìééäáÉë=íÜÉ=Ççêë~ä=~åÇ=é~äã~ê=ãÉÇá~ä=Ñ~ÅÉë=çÑ=íÜÉ=Ü~åÇI=~åÇ=Ü~äÑ=çÑÑáåÖÉêë=Q=~åÇ=RK=

Thoracic nerves

qÜÉ=NO=é~áêë=çÑ=íÜçê~ÅáÅ=åÉêîÉë=ÖáîÉ=êáëÉ=íç=íÜÉ=Åìí~åÉçìë=áååÉêî~íáçå=çÑíÜÉ=íÜçê~ÅáÅ=ÇÉêã~íçãÉëK=jçíçê=ÑáÄêÉë=ëìééäó=áååÉêî~íáçå=çÑ=íÜÉ=ãìëÅäÉëçÑ=íÜÉ=íÜçê~ÅáÅ=~åÇ=~ÄÇçãáå~ä=ï~ääëK=

Lumbosacral plexus

qÜÉ= äìãÄ~ê= éäÉñìë= áë= ÅçãéçëÉÇ= çÑ= éêáã~êó= Äê~åÅÜÉë= çÑ= íÜÉ= ~åíÉêáçêêççíë=iNI=iOI=iP=~åÇ=iQK=fäáçÖ~ëíêáÅI=áäáçáåÖìáå~ä=~åÇ=ÖÉåáíçÑÉãçê~ä=åÉêîÉëçêáÖáå~íÉ=Ñêçã=íÜÉ=iN=êççí=~åÇ=áååÉêî~íÉ=íê~åëîÉêëÉ=~åÇ=çÄäáèìÉ=~ÄÇçãáå~äãìëÅäÉëK= cÉãçê~äI= çÄíìê~íçê= ~åÇ= ä~íÉê~ä= ÑÉãçê~ä= Åìí~åÉçìë= åÉêîÉë= ~êÉÑçêãÉÇ=Ñêçã=íÜÉ=êÉã~áåáåÖ=êççíëK=qÜÉëÉ=åÉêîÉë=~êÉ=êÉëéçåëáÄäÉ=Ñçê=ÑäÉñáçå

Chapter 1 Anatomy of the peripheral nervous system

3


~åÇ=~ÇÇìÅíáçå=çÑ=íÜÉ=íÜáÖÜI=äÉÖ=ÉñíÉåëáçåI=~åÇ=ëÉåëçêó=áååÉêî~íáçå=çÑ=íÜÉ~åíÉêáçê=íÜáÖÜ=~åÇ=äÉÖK=

qÜÉ=ë~Åê~ä=~åÇ=ÅçÅÅóÖÉ~ä=éäÉñìëÉë=~êÉ=ÑçêãÉÇ=Ñêçã=êççíë=ÅçãáåÖ=ÑêçãiQ= íç= pQK= qÜÉáê= ã~áå= íÉêãáå~ä= Äê~åÅÜÉë= ~êÉ= íÜÉ= ëìéÉêáçê= EiQJpNF= ~åÇáåÑÉêáçê=ÖäìíÉ~ä=åÉêîÉë=EiRJpOFI=íÜÉ=éçëíÉêáçê=ÑÉãçê~ä=Åìí~åÉçìë=åÉêîÉ=EpNJpPFI=íÜÉ=ëÅá~íáÅ=åÉêîÉ=EiQJpPF=~åÇ=áíë=Çáîáëáçå=áåíç=íÜÉ=íáÄá~ä=~åÇ=ÅçããçåéÉêçåÉ~ä=åÉêîÉëI=~åÇ=íç=íÜÉ=éìÇÉåÇ~ä=åÉêîÉK=qÜÉó=êÉëìäí=áå=ÉñíÉåëáçå=çÑ=íÜÉíÜáÖÜI= äÉÖ=~åÇ=ÑççíI=~åÇ=ÜÉäé= íç=ÅäçëÉ= íÜÉ=Ää~ÇÇÉê=~åÇ=êÉÅí~ä=ëéÜáåÅíÉêëI~åÇ=ëìééäó=ëÉåëçêó=áååÉêî~íáçå=çÑ=íÜÉ=íÜáÖÜ=~åÇ=éÉêá~å~ä=êÉÖáçåK=

Nerves of the lower limbs

qÜÉ=åÉêîÉë=çÑ=íÜÉ=äçïÉê=äáãÄë=~êÉ=~ë=ÑçääçïëW

√ íÜÉ=ÑÉãçê~ä=åÉêîÉ=EiOJiQF=ãçíçê=ÑáÄêÉë=ëìééäó=íÜÉ=áäáçéëç~ëI=ë~êíçêáìë~åÇ= èì~ÇêáÅÉéë= ÑÉãçêìë= ãìëÅäÉë= êÉëìäíáåÖ= áå= ÑäÉñáçå= ~åÇ= çìíï~êÇêçí~íáçå= çÑ= íÜÉ= äçïÉê= äÉÖ= ~åÇ= ÉñíÉåëáçå= çÑ= íÜÉ= äçïÉê= äÉÖ= çîÉê= íÜÉíÜáÖÜX=ëÉåëçêó= áååÉêî~íáçå= áë= íç= íÜÉ=~åíÉêáçê= íÜáÖÜ=~åÇ=~åíÉêáçê=~åÇãÉÇá~ä=ëìêÑ~ÅÉë=çÑ=íÜÉ=äÉÖ=~åÇ=ÑççíX=

√ íÜÉ=ä~íÉê~ä=ÑÉãçê~ä=Åìí~åÉçìë=åÉêîÉ=áë=éìêÉäó=ëÉåëçêó=~åÇ=áååÉêî~íÉëíÜÉ=~åíÉêáçê=~åÇ=ä~íÉê~ä=ëìêÑ~ÅÉë=çÑ=íÜÉ=íÜáÖÜX=

√ íÜÉ=çÄíìê~íçê=åÉêîÉ= EiOJiQF=Åçåíêçäë=~ÇÇìÅíáçå=~åÇ= êçí~íáçå=çÑ= íÜÉíÜáÖÜX=Åìí~åÉçìë=áååÉêî~íáçå=áë=çå=íÜÉ=áåíÉêå~ä=íÜáÖÜX=

√ íÜÉ= ëìéÉêáçê= ÖäìíÉ~ä= åÉêîÉ= EiQJpNF= Åçåíêçäë= ~ÄÇìÅíáçå= ~åÇ= áåï~êÇêçí~íáçå=çÑ=íÜÉ=íÜáÖÜ=~åÇ=ÑäÉñáçå=çÑ=íÜÉ=ìééÉê=äÉÖ=íç=íÜÉ=ÜáéX=

√ íÜÉ=áåÑÉêáçê=ÖäìíÉ~ä=åÉêîÉ=EiQJpNF=ãçíçê=ÑáÄêÉë=Åçãã~åÇ=ÉñíÉåëáçå=çÑíÜÉ=íÜáÖÜ=~í=íÜÉ=Üáé=~åÇ=çìíï~êÇ=êçí~íáçå=çÑ=íÜÉ=íÜáÖÜX=

√ íÜÉ= ëÅá~íáÅ= åÉêîÉ= EiQJpPF= ãçíçê= ÑáÄêÉë= ëìééäó= íÜÉ= ÄáÅÉéë= ÑÉãçêáëIëÉãáíÉåÇáåçëìë=~åÇ=ëÉãáãÉãÄê~åçëìë=ãìëÅäÉë=êÉëìäíáåÖ=áå=ÑäÉñáçåçÑ= íÜÉ= äçïÉê= äÉÖI=~åÇ=~äëç=ãìëÅäÉë=ÇÉéÉåÇÉåí=ìéçå= íÜÉ= íáÄá~ä= ~åÇéÉêçåÉ~ä=Äê~åÅÜÉë=íÜ~í=~êÉ=íÉêãáå~ä=Äê~åÅÜÉë=çÑ=íÜÉ=ëÅá~íáÅ=åÉêîÉX=

√ íÜÉ= éçëíÉêáçê= ÑÉãçê~ä= Åìí~åÉçìë= åÉêîÉ= EpNJpPF= ëìééäáÉë= ëÉåëçêóáååÉêî~íáçå= çÑ= íÜÉ= éçëíÉêáçê= íÜáÖÜI= ä~íÉê~ä= éÉêáåÉìã= ~åÇ= äçïÉêÄìííçÅâX=

√ íÜÉ=íáÄá~ä=åÉêîÉ=EiQJpOF=ãçíçê=ÑáÄêÉë=Åçåíêçä=éä~åí~ê=ÑäÉñáçåI=áåîÉêëáçåçÑ= íÜÉ= Ñççí= ~åÇ= íçÉ= ÑäÉñáçåK= pÉåëçêó= áååÉêî~íáçå= áë= ÇÉîçíÉÇ= íç= íÜÉä~íÉê~ä=Å~äÑI=ÑççíI=ÜÉÉä=~åÇ=ëã~ää=íçÉX=


4


√ íÜÉ=Åçããçå=éÉêçåÉ~ä=åÉêîÉ=EiQJpNF=Äê~åÅÜÉë=íç=íÜÉ=ÇÉÉé=éÉêçåÉ~äåÉêîÉ=ïÜáÅÜ=ÖáîÉë=êáëÉ=íç=ãçíçê=ÑáÄêÉë=ëìééäóáåÖ=íÜÉ=íáÄá~äáë=~åíÉêáçêIÉñíÉåëçê= Ü~ääìÅáë= äçåÖìëI= ÉñíÉåëçê= ÇáÖáíçêìã= äçåÖìëI= ÉñíÉåëçêÇáÖáíçêìã= ÄêÉîáë= ãìëÅäÉëI= ~åÇ= íç= íÜÉ= ëìéÉêÑáÅá~ä= éÉêçåÉ~ä= åÉêîÉIïÜáÅÜ=ÖáîÉë=êáëÉ=íç=ãçíçê=ÑáÄêÉë=ëìééäóáåÖ=íÜÉ=éÉêçåÉìë=äçåÖìë=~åÇÄêÉîáë= ãìëÅäÉë= êÉëìäíáåÖ= áå= Ñççí= ÇçêëáÑäÉñáçå= ~åÇ= íçÉ= ÉñíÉåëáçåKpÉåëçêó= áååÉêî~íáçå= ÅçãéêáëÉë= íÜÉ= ä~íÉê~ä= é~êí= çÑ= íÜÉ= äÉÖ= ~åÇ= íÜÉÇçêë~ä=~ëéÉÅí=çÑ=íÜÉ=ÑççíK=

Cranial nerves

qÜÉ=Ñáêëí=íïç=é~áêë=çÑ=Åê~åá~ä=åÉêîÉë=~êÉ=ÇÉêáîÉÇ=Ñêçã=íÜÉ=ÅÉêÉÄêìãI=ïÜáäÉíÜÉ=çíÜÉê=íÉå=çêáÖáå~íÉ=Ñêçã=íÜÉ=Äê~áå=ëíÉãK=`ê~åá~ä=åÉêîÉ=f=Ô=íÜÉ=çäÑ~ÅíçêóåÉêîÉ=Ô=~åÇ=Åê~åá~ä=åÉêîÉ=ff=Ô=íÜÉ=çéíáÅ=åÉêîÉ=Ô=Çç=åçí=ÄÉäçåÖ=íç=íÜÉ=mkpKqÜÉó=~êÉ=~Åíì~ääó=Éñé~åëáçåë=çÑ=íÜÉ=ÅÉåíê~ä=åÉêîçìë=ëóëíÉã=E`kpFK=qÜÉ=çíÜÉêíÉå=Åê~åá~ä=åÉêîÉë=ÉåíÉê=íÜÉ=mkp=~ÑíÉê=äÉ~îáåÖ=íÜÉáê=åìÅäÉá=áå=íÜÉ=Äê~áåëíÉãK

Oculomotor nerves — cranial nerves III, IV and VI

`ê~åá~ä=åÉêîÉë=fs=~åÇ=sf= áååÉêî~íÉ=Éñíê~çÅìä~ê=ãìëÅäÉë=çåäóK=qÜÉ=íÜáêÇÅê~åá~ä= åÉêîÉ= áååÉêî~íÉë= Éñíê~çÅìä~ê= ãìëÅäÉëI= íÜÉ= äÉî~íçê= é~äéÉÄê~ÉëìéÉêáçêáë=ãìëÅäÉ=~åÇ=éìéáä=ÅçåëíêáÅíçê=ãìëÅäÉK=`çãéäÉíÉ=é~ê~äóëáë=çÑ=íÜÉíÜáêÇ=Åê~åá~ä=åÉêîÉ=áåÇìÅÉë=éíçëáëI=ïÜáÅÜ=ã~ó=íçí~ääó=ÜáÇÉ=íÜÉ=~ÑÑÉÅíÉÇ=ÉóÉI~åÇ=~êÉ~ÅíáîÉ=éìéáä=Çáä~í~íáçå=EãóÇêá~ëáëFK=

`ê~åá~ä=åÉêîÉ=fs=ëìééäáÉë=íÜÉ=ëìéÉêáçê=çÄäáèìÉ=ãìëÅäÉI=~ääçïáåÖ=íÜÉ=ÉóÉíç= äççâ=Ççïå=~åÇ= áåï~êÇëI=ÉëéÉÅá~ääó=ïÜÉå=ÅäáãÄáåÖ=ëí~áêë=çê= êÉ~ÇáåÖ=~Äççâ=áå=ÄÉÇK=`ê~åá~ä=åÉêîÉ=sf=áååÉêî~íÉë=íÜÉ=ä~íÉê~ä=êÉÅíìë=ãìëÅäÉI=ïÜáÅÜÇáêÉÅíë=íÜÉ=áéëáä~íÉê~ä=ÉñíÉêå~ä=Ö~òÉK=

The trigeminal nerve — cranial nerve V

qÜÉ=íêáÖÉãáå~ä=åÉêîÉ=ÅçåîÉóë=íÜÉ=ëÉåëáÄáäáíó=çÑ=íÜÉ=Ñ~ÅÉI=ëáåìëÉëI=íÉÉíÜ~åÇ=íÜÉ=~åíÉêáçê=é~êí=çÑ=íÜÉ=çê~ä=Å~îáíóK=fí=áë=ÇáîáÇÉÇ=áåíçW=

√ íÜÉ=çéÜíÜ~äãáÅ=Äê~åÅÜ=Ô=éìêÉ=ëÉåëçêó=áååÉêî~íáçåX=


5


√ íÜÉ=ã~ñáää~êó=Äê~åÅÜ=Ô=éìêÉ=ëÉåëçêó=áååÉêî~íáçåX=~åÇ=√ íÜÉ= ã~åÇáÄìä~ê= Äê~åÅÜ= Ô= ëÉåëçêó= ~åÇ= ãçíçê= áååÉêî~íáçå= çÑ

ã~ëíáÅ~íáçå=~åÇ=íÜÉ=íÉåëçê=íóãé~åá=ãìëÅäÉëK=

qÜÉ=ëÉåëçêó=é~êí=çÑ=íÜÉ=çéÜíÜ~äãáÅ=Çáîáëáçå=çÑ=íÜÉ=Åê~åá~ä=åÉêîÉ=s=EsNFáååÉêî~íÉë= íÜÉ= ÅçêåÉ~= ~åÇ= ÉóÉÄ~ää= ~åÇ= Ñçêãë= íÜÉ= ~ÑÑÉêÉåí= äáãÄ= çÑ= íÜÉÅçêåÉ~ä=êÉÑäÉñK=qÜÉ=ã~ñáää~êó=Äê~åÅÜ=áååÉêî~íÉë=íÜÉ=ãáÇJé~êí=çÑ=íÜÉ=Ñ~ÅÉ=~åÇíÜÉ=ã~åÇáÄìä~ê=Äê~åÅÜ=çÑ=íÜÉ=äçïÉê=Ñ~ÅÉK=

The facial nerve — cranial nerve VII

qÜÉ=ãçíçê=ÑáÄêÉë=çÑ=íÜÉ=Ñ~Åá~ä=åÉêîÉ=~êÉ=ÇáëíêáÄìíÉÇ=íç=íÜÉ=ãìëÅäÉë=íÜ~íÅçåíêáÄìíÉ=íç=Ñ~Åá~ä=ÉñéêÉëëáçåI=ãìëÅäÉë=çÑ=íÜÉ=ëÅ~äé=~åÇ=~ää=Ñ~Åá~ä=ãìëÅäÉëI~åÇ= íÜÉ= çêÄáÅìä~êáë= çÑ= íÜÉ= ÉóÉK= m~ê~ëóãé~íÜÉíáÅ= ÑáÄêÉë= áååÉêî~íÉ= íÜÉä~Åêáã~ä= Öä~åÇ= ~åÇ= ë~äáî~êó= Öä~åÇëK= pÉåëçêó= í~ëíÉ= ÑáÄêÉë= ÅçãÉ= Ñêçã= íÜÉ~åíÉêáçê=íïç=íÜáêÇë=çÑ=íÜÉ=íçåÖìÉ=~åÇ=íÜÉ=ëçÑí=é~ä~íÉK=

The vestibulocochlear nerve — cranial nerve VIII

qÜÉ=ìíêáÅäÉI=ë~ÅÅìäÉ=~åÇ=ëÉãáÅáêÅìä~ê=Å~å~äë=íêáÖÖÉê=ëáÖå~äë=åÉÅÉëë~êóÑçê=ÅççêÇáå~íáçåI=Ä~ä~åÅÉ=~åÇ=ãçîÉãÉåí=çÑ=íÜÉ=ÜÉ~Ç=~åÇ=åÉÅâI=ïÜáÅÜ=~êÉÅçåîÉóÉÇ=íÜêçìÖÜ=íÜÉ=îÉëíáÄìä~ê=ÅçãéçåÉåíK=bñé~åëáçåë=çÑ=íÜÉ=éÉêáéÜÉê~äëéáê~ä=Ö~åÖäáçå=áååÉêî~íÉ=íÜÉ=Ü~áê=ÅÉääë=~äçåÖ=íÜÉ=ÅçÅÜäÉ~ê=ÇìÅí=çÑ=íÜÉ=`çêíáçêÖ~åK= eÉ~êáåÖ= áåÑçêã~íáçå= Ñêçã= íÜÉ= `çêíá= çêÖ~å= áë= ÅçåîÉóÉÇ= íç= íÜÉÅçÅÜäÉ~ê= åìÅäÉá= íÜêçìÖÜ= íÜÉ= ÅçÅÜäÉ~ê= ÅçãéçåÉåí= çÑ= íÜÉ= ÉáÖÜíÜ= Åê~åá~äåÉêîÉK=

The glossopharyngeal nerve — cranial nerve IX

qÜÉ= ÖäçëëçéÜ~êóåÖÉ~ä= åÉêîÉ= áååÉêî~íÉë= íÜÉ= ëíóäçéÜ~êóåÖÉìë= ãìëÅäÉ~åÇ= é~êíáÅáé~íÉë= áå= íÜÉ= áååÉêî~íáçå= çÑ= íÜÉ= éÜ~êóåÖÉ~ä= ãìëÅäÉë= Ñçêëï~ääçïáåÖK=pÉåëçêó=ÑáÄêÉë=ÅçåîÉó=í~ëíÉ=ëÉåëáíáîáíó=çÑ=íÜÉ=éçëíÉêáçê=íÜáêÇ=çÑíÜÉ=íçåÖìÉ=~åÇ=éçëíÉêáçê=é~êí=çÑ=íÜÉ=ëçÑí=é~ä~íÉK=


6


The vagus nerve — cranial nerve X

qÜÉ=éÉêáéÜÉê~ä=ãçíçê=åÉìêçåë=çÑ=íÜÉ=î~Öìë=åÉêîÉ=áååÉêî~íÉ=íÜÉ=ãìëÅäÉëçÑ= íÜÉ= ëçÑí= é~ä~íÉI= éÜ~êóåñ= ~åÇ= ä~êóåñI= ~ääçïáåÖ= ëéÉÉÅÜ= ~åÇ= ëï~ääçïáåÖKmêÉÖ~åÖäáçåáÅ= é~ê~ëóãé~íÜÉíáÅ= ~ñçåë= ÉåëìêÉ= íÜÉ= ~ìíçåçãáÅé~ê~ëóãé~íÜÉíáÅ=áååÉêî~íáçå=çÑ=íÜÉ=ÜÉ~êíI=äìåÖ=~åÇ=Ö~ëíêçáåíÉëíáå~ä=íê~Åí=íçíÜÉ=ÇÉëÅÉåÇáåÖ=ÅçäçåK=

The accessory nerve — cranial nerve XI

qÜÉ= ÅçìêëÉ= çÑ= íÜÉ= Åê~åá~ä= é~êí= çÑ= íÜÉ= ÉäÉîÉåíÜ= Åê~åá~ä= åÉêîÉ= êìåë~äçåÖëáÇÉ=íÜÉ=ä~êóåÖÉ~ä=~åÇ=éÜ~êóåÖÉ~ä=Äê~åÅÜÉë=çÑ=íÜÉ=î~Öìë=åÉêîÉ=~åÇíÜÉ= åÉêîÉë= íç= íÜÉ= ëçÑí= é~ä~íÉK= qÜÉ= ëéáå~ä= é~êí= áë= ÑçêãÉÇ= çÑ= ëéáå~ä= ãçíçêåÉìêçåë= Ñêçã=íÜÉ= ä~íÉê~ä= Ñáêëí= Ñçìê=çê= ÑáîÉ=ëÉÖãÉåíë=çÑ= íÜÉ=ÅÉêîáÅ~ä=ëéáå~äÅçêÇK=qÜÉ=ëéáå~ä=~ÅÅÉëëçêó=åÉêîÉ=áååÉêî~íÉë=íÜÉ=ëíÉêåçÅäÉáÇçã~ëíçáÇ=~åÇíÜÉ=ìééÉê=íïç=íÜáêÇë=çÑ=íÜÉ=íê~éÉòáìë=ãìëÅäÉëI=êÉëìäíáåÖ=áå=íÜÉ=êçí~íáçå=çÑíÜÉ=ÜÉ~Ç=~åÇ=ëÜçìäÇÉê=ÉäÉî~íáçåK

The hypoglossal nerve — cranial nerve XII

qÜÉ=ÜóéçÖäçëë~ä=åÉêîÉ=áååÉêî~íÉë=~ää=íÜÉ=íçåÖìÉ=ãìëÅäÉëK=

Autonomic nervous system

qÜÉ= ~ìíçåçãáÅ= åÉêîçìë= ëóëíÉã= E^kpF= Åçåëáëíë= çÑ= ÉÑÑÉêÉåí= ÑáÄêÉë= íÜ~íáååÉêî~íÉ= çêÖ~åë= ëìÅÜ= ~ë= íÜÉ= ÜÉ~êíI= ëãççíÜ= ãìëÅäÉI= ÉñçÅêáåÉ= ~åÇÉåÇçÅêáåÉ= Öä~åÇëI= ~åÇ= ãÉí~ÄçäáÅ= íáëëìÉëI= áããìåÉ= ÅÉääëI= ÉíÅKX= íÜáëáååÉêî~íáçå=áåîçäîÉë=~=íïçJåÉìêçå=é~íÜï~óK=^ÑÑÉêÉåí=~Åíáîáíó=ã~ó=íê~îÉä=îá~íÜÉ=Åê~åá~ä=mkp=EÉKÖK=î~Öìë=åÉêîÉF=ÅçåíêçääÉÇ=Äó=åÉìêçåë=áå=íÜÉ=Äê~áåëíÉãIçê= ~äçåÖ= íÜÉ= ëçã~íáÅ= mkp= EÉKÖK= åÉìêçåë= ÅçåîÉóáåÖ= ÑáåÖÉê= åçÅáÅÉéíáçåFÅçåíêçääÉÇ= Äó= åÉìêçåë= çÑ= íÜÉ= ëéáå~ä= ÅçêÇK= qÜìëI= ëóãé~íÜÉíáÅ= ~åÇé~ê~ëóãé~íÜÉíáÅ= éêÉÖ~åÖäáçåáÅ= åÉìêçåëI= ïÜáÅÜ= ~êÉ= íÜÉ= Ñáêëí= é~êí= çÑ= íÜÉÉÑÑÉêÉåí=é~íÜï~óI=~êÉ=~Åíáî~íÉÇ=íÜêçìÖÜ=áåíÉêåÉìêçåë=~åÇ=ïáää=ëóå~éëÉ=ïáíÜéçëíÖ~åÖäáçåáÅ= åÉìêçåë= íÜ~í= ïáää= áå= íìêå= éêçÇìÅÉ= ~= êÉÑäÉñ= êÉëéçåëÉ= EÉKÖKÅçåíê~Åíáçå= çÑ= î~ëÅìä~ê= ëãççíÜ= ãìëÅäÉ= áå= íÜÉ= êÉÖìä~íáçå= çÑ= ÄäççÇéêÉëëìêÉFK=


7


Sympathetic nervous system

qÜÉ= ëóãé~íÜÉíáÅ= åÉêîçìë= ëóëíÉã= éêÉé~êÉë= íÜÉ= ÄçÇó= Ñçê= ÉãÉêÖÉåÅóêÉëéçåëÉëK=mêÉÖ~åÖäáçåáÅ=åÉìêçå=ÅÉää=ÄçÇáÉë=çÑ=íÜÉ=ëóãé~íÜÉíáÅ=^kpI=çêíÜçê~ÅçäìãÄ~ê=ëóëíÉãI=~êÉ= äçÅ~íÉÇ= áå= íÜÉ= ä~íÉê~ä=Üçêå=çÑ= íÜÉ=ëéáå~ä=ÅçêÇKqÜÉó= äÉ~îÉ= íÜÉ= ëéáå~ä= ÅçêÇ= îá~= îÉåíê~ä= êççíë= ~åÇ= íÉêãáå~íÉ= ÉáíÜÉê= áå= íÜÉä~íÉê~ä=Ö~åÖäáçåáÅ=ÅÜ~áå=íê~îÉääáåÖ=ïáíÜ=ëéáå~ä=åÉêîÉë=çê=áå=íÜÉ=éêÉJîÉêíÉÄê~äÖ~åÖäáçåáÅ= ÅÜ~áåK= mêÉÖ~åÖäáçåáÅ= åÉìêçåë= êÉäÉ~ëÉ= ~ÅÉíóäÅÜçäáåÉ= E^`ÜF= ~ëíÜÉ= ã~áå= åÉìêçíê~åëãáííÉê= áå= íÜÉ= Ö~åÖäáçåáÅ= ëóå~éëÉI= ïÜáÅÜ= ~Åíáî~íÉëåáÅçíáåáÅ= êÉÅÉéíçêë= çå= Ö~åÖäáçå= ÅÉääëI= ~ääçïáåÖ= ~= ê~éáÇ= êÉëéçåëÉKmçëíÖ~åÖäáçåáÅ= ëóãé~íÜÉíáÅ= åÉìêçåë= êÉäÉ~ëÉ= íÜÉ= åÉìêçíê~åëãáííÉêåçêÉéáåÉéÜêáåÉ= E~ÇêÉåÉêÖáÅ= êÉëéçåëÉF= íÜ~í= ÄáåÇë= íç= ~äéÜ~= ~åÇ= ÄÉí~êÉÅÉéíçêë=çå=í~êÖÉíÉÇ=íáëëìÉëK=eçïÉîÉêI=éçëíÖ~åÖäáçåáÅ=ÑáÄêÉë=í~êÖÉíÉÇ=íçíÜÉ=ëïÉ~í=Öä~åÇë=êÉäÉ~ëÉ=^`Ü=~ë=~=åÉìêçíê~åëãáííÉêK

Parasympathetic nervous system

qÜáë=ëóëíÉã=~áãë=íç=êÉÖìä~íÉ=íÜÉ=êÉé~áê=ÑìåÅíáçåëI=é~êíáÅìä~êäó=ÇáÖÉëíáîÉ~åÇ= Éäáãáå~íáçå= ÑìåÅíáçåëK= mêÉÖ~åÖäáçåáÅ= åÉìêçå= ÅÉää= ÄçÇáÉë= çÑ= íÜÉé~ê~ëóãé~íÜÉíáÅ=^kpI=çê=Åê~åáçë~Åê~ä=ëóëíÉãI=~êÉ=äçÅ~íÉÇ=áå=íÜÉ=åìÅäÉá=çÑíÜÉ=íÜáêÇI=ëÉîÉåíÜI=åáåíÜ=~åÇ=íÉåíÜ=Åê~åá~ä=åÉêîÉë=~åÇ= áå=íÜÉ= áåíÉêãÉÇá~íÉÖêÉó=ã~ííÉê=çÑ=íÜÉ=ë~Åê~ä=ëéáå~ä=ÅçêÇ=pO=íç=pQI=~åÇ=íÉêãáå~íÉ=áå=îáëÅÉê~ä=çêáåíê~ãìê~ä=Ö~åÖäáçåëI=åÉ~ê=íÜÉ=áååÉêî~íÉÇ=çêÖ~åK=

mêÉÖ~åÖäáçåáÅ= åÉìêçåë= êÉäÉ~ëÉ= ^`Ü= ~ë= íÜÉ= ã~áå= åÉìêçíê~åëãáííÉê= áåíÜÉ=Ö~åÖäáçåáÅ=ëóå~éëÉI=ïÜáÅÜ=~Åíáî~íÉë=åáÅçíáåáÅ=êÉÅÉéíçêë=çå=Ö~åÖäáçåÅÉääëI= ~ääçïáåÖ= ~= ê~éáÇ= êÉëéçåëÉK= mçëíÖ~åÖäáçåáÅ= é~ê~ëóãé~íÜÉíáÅåÉìêçåë= ~äëç= êÉäÉ~ëÉ= ^`Ü= íÜ~í= ÄáåÇë= íç= ãìëÅ~êáåáÅ= êÉÅÉéíçêë= çå= íÜÉí~êÖÉíÉÇ=íáëëìÉK=

Microscopic anatomy of peripheral nerves OI=P

qÜÉ= åÉêîÉ= íêìåâ= áë= ÅçãéçëÉÇ= çÑ= ëÉîÉê~ä= Ñ~ëÅáÅäÉëI= É~ÅÜ= çÑ= ïÜáÅÜ= áëÇÉÑáåÉÇ= Äó= ÅóäáåÇêáÅ~ä= ä~óÉêë= çÑ= Ñä~ííÉåÉÇ= ëèì~ãçìë= ÅÉääë= ÑçêãáåÖ= íÜÉéÉêáåÉìêáìãI=~åÇ=ÉãÄÉÇÇÉÇ=áå=~=Åçää~ÖÉåçìë=ã~íêáñI=íÜÉ=ÉéáåÉìêáìã=ïÜáÅÜÅçåí~áåë=ÄäççÇ=îÉëëÉäë= EcáÖìêÉ=NFK=kÉêîÉ= ÑáÄêÉë=ÅçåëáëíáåÖ=çÑ=~ñçåë=~åÇíÜÉáê= ~ëëçÅá~íÉÇ= pÅÜï~åå= ÅÉääë= äáÉ= íçÖÉíÜÉê= áå= íÜÉëÉ= Ñ~ëÅáÅäÉë= ~åÇ= ~êÉ


8


ÖáîÉå=ãÉÅÜ~åáÅ~ä=ëìééçêí=Äó=ÉåÇçåÉìêá~ä= ÑáÄêçìë=Åçää~ÖÉå=~åÇ=ãÉí~ÄçäáÅëìééçêí=Ñêçã=~=åÉíïçêâ=çÑ=ëã~ää=ÄäççÇ=îÉëëÉäë=EcáÖìêÉ=OFK

The motor neurons and axons

qÜÉ=ÅÉää=ÄçÇó=çÑ=ãçíçê=åÉìêçåë= áë= äçÅ~íÉÇ= áå= íÜÉ=~åíÉêáçê=Üçêå=çÑ= íÜÉëéáå~ä=ÅçêÇ=~åÇ=áå=íÜÉ=ãçíçê=åìÅäÉá=çÑ=Åê~åá~ä=åÉêîÉëK=jçíçê=~ñçåë=áëëìáåÖÑêçã=ãçíçê=åÉìêçåë=~êÉ=~ää=ãóÉäáå~íÉÇK

Sensory neurons

qÜÉ=ÅÉää=ÄçÇáÉë=çÑ=ëÉåëçêó=åÉìêçåë=~êÉ=äçÅ~íÉÇ=çìíëáÇÉ=íÜÉ=̀ kpI=áå=Ççêë~äêççí=Ö~åÖäá~K=qïç=~ñçåë=~êÉ=áëëìÉÇ=Ñêçã=íÜÉ=ÅÉää=ÄçÇóW=çåÉ=~=ÅÉåíê~ä Äê~åÅÜ


9

Figure 1. Cross-section of the sural nerve sampled by biopsy

to show the multifascicular constitution of a peripheral nerve.

The asterisk is positioned in the epineurial nerve compartment

which surrounds nerve fascicles. The arrow points to the

perineurium, which surrounds and limits nerve fascicles. The

crosses are positioned in the endoneurial space, which

contains nerve fibres and connective tissue. (Plastic section at

1μm thickness. Thionin staining. Bar: 1mm.)

*

x

x


ïÜáÅÜ=ÉåíÉêë= íÜÉ=ëéáå~ä=ÅçêÇ=ïáíÜ= íÜÉ=Ççêë~ä= êççíX= íÜÉ=çíÜÉê=~=éÉêáéÜÉê~äÄê~åÅÜ=ïÜáÅÜ=íê~îÉäë=íÜêçìÖÜ=íÜÉ=Ççêë~ä=êççíI=íç=íÜÉ=éäÉñìë=~åÇ=éÉêáéÜÉê~äåÉêîÉë=íç=êÉ~ÅÜ=ëÉåëçêó=êÉÅÉéíçêë=áå=íÜÉ=ëâáåI=ãìëÅäÉë=~åÇ=íÉåÇçåëK

Schwann cells

pÅÜï~åå=ÅÉääë=ïê~é=ã~åó=ä~óÉêë=çÑ=íáÖÜíäó=é~ÅâÉÇ=ÅÉää=ãÉãÄê~åÉ=~êçìåÇ~= ëáåÖäÉ= ëÉÖãÉåí= çÑ= ~= ëáåÖäÉ= ~ñçå= íç= Ñçêã= íÜÉ= ãóÉäáå= ëÜÉ~íÜ= EcáÖìêÉ= PFKråãóÉäáå~íÉÇ=ëÉåëçêó=ÑáÄêÉë=~êÉ=ëã~ääÉê=~ñçåëI=äÉëë=íÜÉå=Oμã=áå=Çá~ãÉíÉêKqÜÉó=~êÉ=ëìêêçìåÇÉÇ=Äó=~=ëáåÖäÉ=ä~óÉê=çÑ=pÅÜï~åå=ÅÉää=Åóíçéä~ëãáÅ=éêçÅÉëëK^=ëáåÖäÉ=pÅÜï~åå=ÅÉää=ïê~éë=ëÉîÉê~ä=ìåãóÉäáå~íÉÇ=~ñçåëK


10

Figure 2. The endoneurium with myelinated and unmyelinated

fibres. A one-micron-thick section of a plastic-embedded nerve

biopsy specimen showing the myelinated nerve fibres. The

unmyelinated fibres cannot be studied by light microscopy.

However, they can be seen on this section as pointed out by thin

white arrows. The density of myelinated nerve fibres (large

arrows) is 7000 to 12,000 per mm2 of endoneurial area. The

nerve fibre density decreases with age. The density of

unmyelinated nerve fibres ranges from 15,000 to 35,000 per

mm2. An endoneurial blood vessel is marked with an asterisk.

(Bar: 10μm.)

*


Nodes of Ranvier

jçíçêI= éêÉÖ~åÖäáçåáÅ= ~åÇ= ~ééêçñáã~íÉäó= çåÉ= íÜáêÇ= çÑ= ëÉåëçêó= åÉìêçåë~êÉ= ãóÉäáå~íÉÇ= Äó= pÅÜï~åå= ÅÉääëK= pÅÜï~åå= ÅÉääë= ïê~é= ã~åó= ä~óÉêë= çÑíáÖÜíäó=é~ÅâÉÇ=ÅÉää=ãÉãÄê~åÉ=~êçìåÇ=~=ëáåÖäÉ=ëÉÖãÉåí=çÑ=~=ëáåÖäÉ=~ñçåI=áåÅçåíê~ëí=íç=`kp=ãóÉäáå~íáçå=ïÜÉêÉ=çäáÖçÇÉåÇêçÅóíÉë=ïê~é=ãìäíáéäÉ=ä~óÉêëçÑ= íáÖÜíäó= é~ÅâÉÇ= ãÉãÄê~åÉ= çÑ= ~= ëáåÖäÉ= ëÉÖãÉåí= ~åÇ= ëÉîÉê~ä= ~Çà~ÅÉåí~ñçåëK= qÜÉ= ëé~ÅÉ= ÄÉíïÉÉå= ~Çà~ÅÉåí= ãóÉäáå~íÉÇ= ëÉÖãÉåíë= Eçê= åçÇÉë= çÑo~åîáÉêF=EcáÖìêÉ=QF=Åçåí~áåë=~=ÜáÖÜ=ÅçåÅÉåíê~íáçå=çÑ=ëçÇáìã=îçäí~ÖÉJÖ~íÉÇÅÜ~ååÉäë=çå=íÜÉ=~ñçå=ãÉãÄê~åÉ=~åÇ=~ääçïë=íÜÉ=êÉáåáíá~íáçå=çÑ= íÜÉ=~ÅíáçåéçíÉåíá~ä=ÇìêáåÖ=áíë=ëéêÉ~ÇáåÖ=çå=íÜÉ=åÉêîÉ=ÑáÄêÉI=Ñêçã=íÜÉ=ÅÉää=ÄçÇó=íç=íÜÉåÉñí=ëóå~éëÉW=íÜáë=íóéÉ=çÑ=ÅçåÇìÅíáçå=áë=Å~ääÉÇ=ë~äí~íçêó=ÅçåÇìÅíáçåÒK


11

Figure 3. Isolated myelinated fibres. A group of myelinated nerve fibres are isolated

by teasing the endoneurial content with fine needles under the microscope. The

myelin sheath is grey to black, depending on its thickness, after post-fixation in

osmium tetroxide. The nodes of Ranvier are designated by arrows. (Bar: 50μm.)

Figure 4. Electron micrograph of a longitunal section of a node

of Ranvier. The reflection of the myelin layers on each side of the

node are indicated by arrows. The asterisks identify the axon.

(Uranyl and lead citrate staining.)

*

*

*


Myelin sheath proteins

qÜÉ=ãóÉäáå=ëÜÉ~íÜ=Åçåí~áåë=ëÉîÉê~ä=éêçíÉáåëI=ëçãÉ=íÜÉ=ë~ãÉ=~ë=ÑçìåÇáå= íÜÉ= pÅÜï~åå= ÅÉää= éä~ëã~= ãÉãÄê~åÉ= ~åÇ= çíÜÉêë= áåëÉêíÉÇ= ÇìêáåÖÅçãé~Åíáçå=çÑ=íÜÉ=ä~óÉêë=çÑ=ÅÉää=ãÉãÄê~åÉ=íç=Ñçêã=íÜÉ=ëÜÉ~íÜK=qÜÉ=ãóÉäáåéêçíÉáå= òÉêç= EjmwI= mMF= ÅçãéêáëÉë= RMJSMB= çÑ= íÜÉ= ãóÉäáå= éêçíÉáå= ÄóïÉáÖÜíK= qÜÉ= ëã~ää= éÉêáéÜÉê~ä= ãóÉäáå= éêçíÉáå= OO= EmjmOOF= ã~âÉë= ìé= äÉëëíÜ~å= RB= çÑ= íÜÉ= íçí~ä= éêçíÉáå= Äìí= áë= ÉëëÉåíá~ä= Ñçê= íÜÉ= ÅçêêÉÅí= ~åÇ= ëí~ÄäÉÑçêã~íáçå=çÑ=~=ãóÉäáå=ëÜÉ~íÜK=fåÅêÉ~ëÉë=áå=èì~åíáíó=çê=~Äåçêã~äáíáÉë=áå=íÜÉëíêìÅíìêÉ= çÑ= íÜáë= éêçíÉáå= ìåÇÉêäáÉ= ~= ÅçåëáÇÉê~ÄäÉ= åìãÄÉê= çÑ= áåÜÉêáíÉÇéÉêáéÜÉê~ä= åÉìêçäçÖáÅ~ä= ÇáëÉ~ëÉëK= cçìåÇ= çåäó= áå= íÜÉ= é~ê~åçÇÉ= ~åÇpÅÜãáÇíJi~åíÉêã~å=áåÅáëìêÉëI=ãóÉäáåJ~ëëçÅá~íÉÇ=ÖäóÅçéêçíÉáå=Ej^dF=áë=~åáãéçêí~åí= ãóÉäáå= éêçíÉáåI= ~äíÜçìÖÜ= ~= îÉêó= ãáåçê= ÅçãéçåÉåí= Äó= ïÉáÖÜíI~ÅÅçìåíáåÖ=Ñçê=çåäó=MKNB=çÑ=íÜÉ=éêçíÉáåK=qÜáë=áë=~å=~ÇÜÉëáçå=éêçíÉáå=ïáíÜÑáîÉ=áããìåçÖäçÄìäáå=Ççã~áåë=íÜ~í= áë=çåäó=ÑçìåÇ=áå=åçåJÅçãé~ÅíÉÇ=ãóÉäáå~í=íÜÉ=åçÇÉë=çÑ=o~åîáÉê=~åÇ=pÅÜãáÇíJi~åíÉêã~å=áåÅáëìêÉëK=j^d=ã~ó=ÄÉáãéçêí~åí=ÇìêáåÖ=íÜÉ=éêçÅÉëë=çÑ=ãóÉäáå=Ñçêã~íáçå=~åÇ=ã~ó=~äëç=éä~ó=~=êçäÉáå=íÜÉ=ã~áåíÉå~åÅÉ=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜK=

Classification of nerve fibres: size, myelination, conductionvelocity and function (Figure 5)

qÜáë=ëóëíÉã=ÅçãéêáëÉë=íÜêÉÉ=Öêçìéë=çÑ=ÑáÄêÉë=EÎ=_=~åÇ=`FW

√ Öêçìé= Î= íÜÉ= ä~êÖÉëí= ÑáÄêÉë= ïáíÜ= Ñ~ëíÉëí= ÅçåÇìÅíáçå= îÉäçÅáíáÉë= ~êÉãóÉäáå~íÉÇ=ëçã~íáÅ=~ÑÑÉêÉåí=~åÇ=ÉÑÑÉêÉåí=ÑáÄêÉëW

n Öêçìé=^=~ÑÑÉêÉåí=ÑáÄêÉëW=J= Öêçìé= fI= íÜÉ= ä~êÖÉëí= ïáíÜ= Çá~ãÉíÉêë= çÑ= NMJOMμãI= ~êÉ= ëÉåëçêó

~ÑÑÉêÉåíë= Ñêçã= íÜÉ=ãìëÅäÉ=ëéáåÇäÉë= Ef~F=~åÇ= íÉåÇçå=çêÖ~åë= EfÄFïáíÜ=ëéÉÉÇë=çÑ=RMJNMMãLëX=

J= Öêçìé=ff=ERJNRμãI=OMJTMãLëF= áåÅäìÇÉ=ÑáÄêÉë=Ñêçã=íÜÉ=ëÉÅçåÇ~êóÉåÇáåÖë=çå=áåíê~Ñìë~ä=ãìëÅäÉ=ÑáÄêÉë=ïáíÜáå=íÜÉ=ãìëÅäÉ=ëéáåÇäÉ=~åÇÅìí~åÉçìë=~ÑÑÉêÉåí=êÉÅÉéíçêëX=

J= Öêçìé= fff= ~êÉ= íÜÉ= ëã~ääÉëí= ãóÉäáå~íÉÇ= ÑáÄêÉë= ~åÇ= ä~êÖÉêìåãóÉäáå~íÉÇ= çåÉë= ïáíÜ= Çá~ãÉíÉêë= çÑ= NJTμã= ~åÇ= ëéÉÉÇë= çÑ= RJ


12


PMãLëK=qÜÉëÉ=áåÅäìÇÉ=åçÅáÅÉéíáîÉ=ÑáÄêÉëI=ëÉåëçêó=~ÑÑÉêÉåíë=Ñêçãëã~ää=ÄäççÇ=îÉëëÉä=ï~ääë=~åÇ=Ü~áê=ÑçääáÅäÉëX

n Öêçìé=^=ÉÑÑÉêÉåí=ÑáÄêÉëWJ= α EVJOMμãI=RMJNMMãLëF=~êÉ=ëâÉäÉíçãçíçê=ÑáÄêÉëXJ= β EVJNRμãI=RMJURãLëF=~êÉ=ëâÉäÉíçãçíçê=~åÇ=ÑìëáãçíçêXJ= γ EQKRJUKRμãI=OMJQMãLëF=~êÉ=ÑìëáãçíçêX=

√ Öêçìé=_=~êÉ=ãóÉäáå~íÉÇ=éêÉJÖ~åÖäáçåáÅ=~ìíçåçãáÅ=ÑáÄêÉëX=√ Öêçìé=`=~êÉ=íÜÉ=ëã~ääÉëíI=ëäçïÉëí=ìåãóÉäáå~íÉÇ=ÑáÄêÉë=EîáëÅÉê~ä=~åÇ

ëçã~íáÅ=~ÑÑÉêÉåí=~åÇ=éçëíJÖ~åÖäáçåáÅ=~ìíçåçãáÅ=ÉÑÑÉêÉåíëFK=


13

Figure 5. Electron micrograph of a cross-section of a nerve

specimen to show the myelinated and unmyelinated fibres, and

the Schwann cells. The myelinated fibres are indicated by large

arrows and the unmyelinated axons by thin arrows. The

asterisks depict the nuclei of Schwann cells.

*

**


References

NK d~êÇåÉê= baI= _ìåÖÉ= omK= dêçëë= ~å~íçãó= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉãK= fåW

mÉêáéÜÉê~ä= kÉìêçé~íÜóI= QíÜ= ÉÇáíáçåK= aóÅâ= mgI= qÜçã~ë= mhI= bÇëK= bäëÉîáÉê= p~ìåÇÉêëI

OMMRW=NNJPPK

OK qÜÉ=mÉêáéÜÉê~ä=kÉêîÉK=ak=i~åÇçåI=bÇK=içåÇçåW=`Ü~éã~å=~åÇ=e~ääI=NVTSK

PK pí~~ä=Î=î~å=dáàå=gI=pé~~åë=cK jçåçåÉìêçé~íÜáÉëK=t_=p~ìåÇÉêëI=NVVVK


14

√ Spinal roots exchange fibres in the cervical and brachial plexuses.

√ The cranial nerves provide motor innervation of oculomotor muscles,

masticatory muscles, the pharynx, the larynx and tongue muscles.

√ Sensory innervation of the face is provided by the trigeminal nerve.

√ Nerve trunks destined to the upper limbs are issued from the brachial plexus;

those destined to the lower limbs from the lumbosacral plexus.

√ The autonomic nervous system is composed of sympathetic and

parasympathetic systems.

√ The myelinated fibres have larger axons, always surrounded by a myelin

sheath made by Schwann cells.

√ The unmyelinated fibres are made of smaller axons. One Schwann cell

harbours several unmyelinated axons.

Ü Key points


15

Basic pathological processes

Chapter 2

Overview

This chapter gives a brief summary of the lesions of nerve fibres that occur in

different conditions. Axonal degeneration with the Wallerian degeneration

component and the dying-back phenomenon are discussed, as is the demyelinating

process in some conditions. Also covered is the reparation of lesions of nerve fibres

which occurs by sprouting of the axon in axonal neuropathies, by remyelination in

demyelinating processes. Finally, general conditions that can affect nerves are

outlined, including infection, malignancy and vasculitis.

Introduction

^=î~êáÉíó=çÑ=é~íÜçäçÖáÅ~ä=éêçÅÉëëÉë=Å~å=~ÑÑÉÅí= íÜÉ=éÉêáéÜÉê~ä=åÉêîçìëëóëíÉã= ~åÇ= áåÇìÅÉ= äÉëáçåë= çÑ= åÉêîÉ= ÑáÄêÉë= êÉëéçåëáÄäÉ= Ñçê= ëáÖåë= ~åÇëóãéíçãë=çÑ=åÉìêçé~íÜó=NI=OK=qÜÉëÉ=éêçÅÉëëÉë=ïáää=ÄÉ=ÄêáÉÑäó=Éñéä~áåÉÇ=áåíÜáë=ëÉÅíáçå=~åÇ=ÅçîÉêÉÇ=áå=ãçêÉ=ÇÉí~áä=áå=ÅçêêÉëéçåÇáåÖ=ÅÜ~éíÉêëK=

Pathological processes affecting the nerve fibres

qïç=ã~áå=íóéÉë=çÑ=ÇÉÖÉåÉê~íáçå=çÑ=åÉêîÉ=ÑáÄêÉë=Ü~îÉ=ÄÉÉå=áÇÉåíáÑáÉÇ=Ô~ñçå~ä=~åÇ=ëÉÖãÉåí~äK


Axonal degeneration

Wallerian degenerationfå= íÜáë= éêçÅÉëëI= íÜÉ= ~ñçå= áë= íÜÉ= éêáã~êó= í~êÖÉíI= áåÇìÅáåÖ= ëÉÅçåÇ~êó

ÇÉÖÉåÉê~íáçå=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜI=ïÜÉå=~=ãóÉäáå~íÉÇ=ÑáÄêÉ=áë=~ÑÑÉÅíÉÇK=qÜÉáåàìêó=Å~ìëáåÖ=~ñçå~ä=ÇÉÖÉåÉê~íáçå=ã~ó=~Åí=~í=íÜÉ=äÉîÉä=çÑ=íÜÉ=ÅÉää=ÄçÇó=çÑíÜÉ=åÉìêçåI=áå=ãçíçê=åÉìêçåë=äçÅ~íÉÇ=áå=íÜÉ=~åíÉêáçê=Üçêå=ÅÉää=áå=íÜÉ=ëéáå~äÅçêÇ=Eáå=~ãóçíêçéÜáÅ=ä~íÉê~ä=ëÅäÉêçëáë=Ñçê=Éñ~ãéäÉFI=çê=ãçêÉ=Çáëí~ääó=ìéçåíÜÉ=éÉêáéÜÉê~ä=~ñçåK=

tÜÉå=íÜÉ=ëÉåëçêó=ëóëíÉã= áë=ÅçåÅÉêåÉÇI= íÜÉ=ÅÉää=ÄçÇó=çÑ= íÜÉ=ëÉåëçêóåÉìêçå= áë= äçÅ~íÉÇ= áå=Ççêë~ä= êççí=Ö~åÖäá~I=ïÜÉêÉ= íÜÉáê= äÉëáçåë=Å~å= áåÇìÅÉ~ñçå~ä= ÇÉÖÉåÉê~íáçå= çÑ= íÜÉ= ÅÉåíê~ä= ~åÇ= éÉêáéÜÉê~ä= ~ñçå~ä= éêçÅÉëëÉë= áåÇçêë~ä=êççíëK=^ñçå~ä=ÇÉÖÉåÉê~íáçå=Çáëí~ä=íç=íÜÉ=äÉëáçå=áë=ÇìÉ=íç=ëÉé~ê~íáçåçÑ= íÜÉ= Åóíçéä~ëã= Ñêçã= íÜÉ= åìÅäÉìëK= qÜÉ= Ñáêëí= ÇÉëÅêáéíáçå= çÑ= íÜÉ= äÉëáçåëáåÇìÅÉÇ= Äó= ÑçÅ~ä= íê~ìã~= íç= ~= éÉêáéÜÉê~ä= åÉêîÉ= áë= Å~ääÉÇ= t~ääÉêá~åÇÉÖÉåÉê~íáçå=~ÑíÉê=t~ääÉê=ENURMF=ïÜç=éÉêÑçêãÉÇ=íÜÉ=ÉñéÉêáãÉåíK

táíÜáå=~= ÑÉï=Üçìêë=~ÑíÉê=åÉêîÉ=ÅêìëÜI= íÜÉ=Çáëí~ä= ~ñçå=ëÜçïë=ëáÖåë=çÑÇÉÖÉåÉê~íáçå=~åÇ=Äó=OQ=Üçìêë=íÜÉ=ãóÉäáå=ëÜÉ~íÜ=ëí~êíë=íç=ëéäáíK=_ó=OJP=Ç~óëíÜÉ=ãóÉäáå=ëÜÉ~íÜ=ÄÉÅçãÉë=ÄêçâÉå=áåíç=ä~êÖÉ=çîçáÇë=ïáíÜáå=íÜÉ=pÅÜï~ååÅÉääëK=qÜÉ=ëáòÉ=çÑ=íÜÉ=ãóÉäáå=çîçáÇë=~åÇ=Ä~ääë=ÇÉÅêÉ~ëÉ=Öê~Çì~ääó=ïáíÜ=íáãÉÄìí=ãóÉäáå=ÇêçéäÉíë=Å~å=ëíáää=ÄÉ=ëÉÉå=OJP=ãçåíÜë=~ÑíÉê=íÜÉ=åÉêîÉ=äÉëáçåK=

páãìäí~åÉçìë= íç= ãóÉäáå= ÇÉÖÉåÉê~íáîÉ= ÅÜ~åÖÉëI= pÅÜï~åå= ÅÉääëéêçäáÑÉê~íÉ= íç= éêÉé~êÉ= íÜÉ= íìÄÉ= íÜ~í= ïáää= ÖìáÇÉ= íÜÉ= êÉÖÉåÉê~íáåÖ= ~ñçåëéêçìíë= ïÜáÅÜ= çêáÖáå~íÉ= Ñêçã= íÜÉ= éêçñáã~ä= ëíìãé= çÑ= íÜÉ= Ç~ã~ÖÉÇ= ~ñçåKoÉÖÉåÉê~íáåÖ= ~ñçå= ëéêçìíë= ~ééÉ~ê= îÉêó= É~êäóI= ~= ÑÉï= Ç~óëI= ~ÑíÉê= åÉêîÉÅêìëÜK= qÜÉó= ïáää= éêçÖêÉëë= ~í= íÜÉ= ê~íÉ= çÑ= Ñê~Åíáçåë= çÑ= ~= ãáääáãÉíêÉ= íç= çåÉãáääáãÉíêÉ=éÉê=Ç~ó= íç= êÉ~ÅÜ= íÜÉáê= í~êÖÉíK=oÉÖÉåÉê~íáåÖ=~ñçåë=~êÉ=ëã~ääÉêíÜ~å=íÜÉ=çêáÖáå~ä=~ñçå=~åÇ=ëí~êí=íç=ãóÉäáå~íÉ=çåäó=~ÑíÉê=êÉ~ÅÜáåÖ=íÜÉ=ÅêáíáÅ~äëáòÉ=çÑ=NJOμ áå=Çá~ãÉíÉêK=^ÑíÉê=~=ÑÉï=ãçåíÜë=íÜÉ=êÉÖÉåÉê~íÉÇ=~ñçåë=ÑçêãÅäìëíÉêë=çÑ=ëã~ää=ãóÉäáå~íÉÇ=ÑáÄêÉë=ïÜáÅÜ=Å~å=ÄÉ=áÇÉåíáÑáÉÇ=çå=åÉêîÉ=ÅêçëëJëÉÅíáçåK

få= éê~ÅíáÅÉI= t~ääÉêá~å= ÇÉÖÉåÉê~íáçå= çÅÅìêë= ã~áåäó= áå= íê~ìã~íáÅ= åÉêîÉäÉëáçåëI= åÉêîÉ= ÅçãéêÉëëáçå= çê= åÉêîÉ= áëÅÜ~Éãá~K= ^åçíÜÉê= íóéÉ= çÑ= ~ñçå~ä


16


ÇÉÖÉåÉê~íáçå=Ô=íÜÉ=ÇóáåÖJÄ~Åâ=éêçÅÉëë=Ô=áë=ãçëí=Åçããçå=áå=~=åìãÄÉêçÑ=åÉìêçé~íÜáÉëK

Axonopathy: dying-back axonal degeneration_ÉÅ~ìëÉ= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉã= ~ñçåë= ä~Åâ= êáÄçëçãÉëI= íÜÉ

ëìééäó= çÑ= ÉëëÉåíá~ä= ëíêìÅíìê~ä= éêçíÉáåë= ~åÇ= ÉåòóãÉë= íç= Çáëí~ä= êÉÖáçåëEÇÉêáîÉÇ= Ñêçã= íÜÉ=åÉìêçå~ä=éÉêáâ~êóçåF=ãìëí=ÄÉ= íê~åëéçêíÉÇ= íÜêçìÖÜ= íÜÉ~ñçå= Ñçê= ÖêÉ~í= Çáëí~åÅÉëK= kÉìêçåë= ïáíÜ= äçåÖ= ~ñçåë= ~êÉ= é~êíáÅìä~êäóîìäåÉê~ÄäÉ=íç=íçñáÅ=çê=ãÉí~ÄçäáÅ=ÇáëíìêÄ~åÅÉë=íÜ~í=ÅçãéêçãáëÉ=ëóåíÜÉëáë=çêíê~åëéçêí=çÑ=~ñçå~ä=éêçíÉáåëK= få=íçñáÅ=åÉìêçé~íÜáÉëI=Çá~ÄÉíáÅ=åÉìêçé~íÜáÉë~åÇ=åÉìêçé~íÜáÉë=ÇìÉ= íç= îáí~ãáå=ÇÉÑáÅáÉåÅáÉëI= ëÉåëçêó=~åÇ=ãçíçê=ÇÉÑáÅáíéêÉÇçãáå~íÉ= áå=Çáëí~ä= äçïÉê=ÉñíêÉãáíáÉë= áå= êÉä~íáçå= íç=ÇÉÖÉåÉê~íáçå=çÑ= íÜÉíÉêãáå~ä=é~êíë=çÑ=íÜÉ=äçåÖÉëí=~ñçåëK=qÜáë=éÜÉåçãÉåçå=Å~ääÉÇ=ÇóáåÖJÄ~ÅâÒÅ~å=ÄÉ=ÇìÉ=íç=áãé~áêãÉåí=çÑ=íÜÉ=ëìééäó=çÑ=íÜÉ=ãÉí~ÄçäáÅ=êÉèìáêÉãÉåíë=ÑêçãíÜÉ= ÅÉää= ÄçÇóK= qÜÉ= ÇóáåÖJÄ~Åâ= ÇÉÖÉåÉê~íáçå= áë= çÑíÉå= ÑçääçïÉÇ= Äó= ~ñçå~äêÉÖÉåÉê~íáçå=Äó=ëéêçìíáåÖ=çÑ=íÜÉ=éêçñáã~ä=ëíìãé=EcáÖìêÉë=NJPFK=

Chapter 2 Basic pathological processes

17

Figure 1. Diagram to illustrate dying-back degeneration. The top row illustrates a normal

sensory myelinated fibre with the cell body (arrow). The second row illustrates segmental

demyelination (arrow) proximal to distal axonal degeneration with fragmentation of the

myelin sheath forming ovoids and balls of myelin containing fragments of axon

(arrowheads). The third row shows remyelination of the demyelinated area (arrow) and

axonal regeneration by sprouting (arrowhead). The regenerating axon is not yet myelinated.

The last row shows a later stage of axonal regeneration with division of the regenerating

axon and myelination of axon sprouts (arrowheads).



18

Figure 2. Dying-back degeneration of a nerve fibre

in experimental acrylamide intoxication. Consecutive

segments of a nerve fibre isolated from the sciatic

nerve of a rat intoxicated by acrylamide in drinking

water for a month. The proximal end of the isolated

segment is at the top left of the panel, the distal end

at the bottom right. The myelin sheath is stained

black by post-fixation in osmium tetroxide. A and B

denotes normal nodes of Ranvier. Distal to node D

the myelin sheath shows changes characteristic of

Wallerian degeneration.


Segmental demyelination

pÉÖãÉåí~ä=ÇÉãóÉäáå~íáçå=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=äçëë=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜÄÉíïÉÉå=íïç=åçÇÉë=çÑ=o~åîáÉêK=pÉÖãÉåí~ä=ÇÉãóÉäáå~íáçå=ã~ó=êÉëìäí=Ñêçã~å=áããìåÉ=ãÉÅÜ~åáëã=~ë=áå=íÜÉ=dìáää~áåJ_~êê¨=ëóåÇêçãÉ=çê=Ñêçã=~=íçñáÅãÉÅÜ~åáëã=~ë=áå=ÇáéÜíÜÉêáíáÅ=åÉìêçé~íÜóK=qÜÉ=ìåÇÉêäóáåÖ=~ñçå=áë=ëé~êÉÇ~åÇ= áå= ãçëí= áåëí~åÅÉë= ëÉÖãÉåí~ä= ÇÉãóÉäáå~íáçå= áë= ÑçääçïÉÇ= ÄóêÉãóÉäáå~íáçå= ïáíÜ= êÉéä~ÅÉãÉåí= çÑ= íÜÉ= çêáÖáå~ä= áåíÉêåçÇÉ= Äó= ëÉîÉê~äëÜçêíÉêI= åÉïäó= ÑçêãÉÇ= áåíÉêåçÇÉëK= qÜÉ= éêçÅÉëë= çÑ= ÇÉãóÉäáå~íáçåJêÉãóÉäáå~íáçå= Å~å= ÄÉ= ÅçãéäÉíÉÇ= ïáíÜáå= ~= ÑÉï= Ç~óë= çê= ïÉÉâë= ~ë= áå= íÜÉdìáää~áåJ_~êê¨= ëóåÇêçãÉK= aÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜáÉë= ~êÉ= çÑíÉå~ëëçÅá~íÉÇ= ïáíÜ= ëÉÅçåÇ~êó= ~ñçå~ä= ÇÉÖÉåÉê~íáçå= ïÜáÅÜ= ÇÉä~óë= çêéêÉîÉåíë= ÅäáåáÅ~ä= êÉÅçîÉêóK= få= Çá~ÄÉíáÅ= åÉìêçé~íÜóI= ïÜáÅÜ= áë= íÜÉ= ãçëíÅçããçå=åÉìêçé~íÜó=áå=íÜÉ=ïçêäÇI=ëÉÖãÉåí~ä=ÇÉãóÉäáå~íáçå=áë=~ëëçÅá~íÉÇïáíÜ=ÇóáåÖJÄ~Åâ=~ñçå~ä=ÇÉÖÉåÉê~íáçåK

Figure 3. Axonal regeneration. One-micron-thick plastic

section of a nerve biopsy specimen from a patient with an

axonal neuropathy. Two clusters of regenerating axon sprouts

are indicated by arrows. (Bar: 10μm.)


19


Pathological processes affecting nerve blood vessels

s~ëÅìäáíáëI= ïÜáÅÜ= ~ééäáÉë= íç= áåÑä~ãã~íçêó= äÉëáçåë= çÑ= îÉëëÉä= ï~ääëIÅçããçåäó= ~ÑÑÉÅíë= åÉêîÉ= ÄäççÇ= îÉëëÉäëI= ~ë= áå= éçäó~êíÉêáíáë= åçÇçë~IáåÇìÅáåÖ= åÉêîÉ= áëÅÜ~Éãá~K= _äççÇ= îÉëëÉäë= ~ÑÑÉÅíÉÇ= Äó= î~ëÅìäáíáë= ~êÉäçÅ~íÉÇ= áå= íÜÉ= ÉéáåÉìêáìãK= lÅÅäìëáçå= çÑ= íÜÉ= îÉëëÉä= äìãÉå= éêçîçâÉëáëÅÜ~Éãá~= çÑ= åÉêîÉ= ÑáÄêÉë= ~åÇ= ã~ëëáîÉ= ~ñçå~ä= ÇÉÖÉåÉê~íáçåK= EmäÉ~ëÉêÉÑÉê=íç=`Ü~éíÉê=S=Ô=s~ëÅìäáíáÅ=åÉìêçé~íÜáÉë=Ô=Ñçê=ãçêÉ=ÇÉí~áäKF

Abnormal deposits in the endoneurium

båÇçåÉìêá~ä=~ãóäçáÇ=ÇÉéçëáíë=Å~ìëÉ=ã~àçê=~åÇ=áêêÉîÉêëáÄäÉ=Ç~ã~ÖÉ=íçåÉáÖÜÄçìêáåÖ=ãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇ=åÉêîÉ=ÑáÄêÉë=êÉëéçåëáÄäÉ= ÑçêäáÑÉJíÜêÉ~íÉåáåÖ= Ñ~ãáäá~ä=çê= äáÖÜíJÅÜ~áå=~ãóäçáÇ=éçäóåÉìêçé~íÜáÉëK= EmäÉ~ëÉêÉÑÉê=íç=`Ü~éíÉê=NM=Ô=eÉêÉÇáí~êó=åÉìêçé~íÜáÉë=Ô=Ñçê=ãçêÉ=ÇÉí~áäKF

Invading malignant cells

j~äáÖå~åí= ÅÉääë= Å~å= ~äëç= áåî~ÇÉ= åÉêîÉ= ÉåîÉäçéÉë= ~åÇ= éêçîçâÉÇÉÖÉåÉê~íáçå= çÑ= åÉêîÉ= ÑáÄêÉëK= iÉëáçåë= ~êÉ= ãìÅÜ= ãçêÉ= Åçããçå= ~í= íÜÉêççí= äÉîÉä= íÜ~å= áå= åÉêîÉ= íêìåâëK= EmäÉ~ëÉ= êÉÑÉê= íç= `Ü~éíÉê= V= ÔkÉìêçé~íÜáÉë=áå=é~íáÉåíë=ïáíÜ=ãçåçÅäçå~ä=Ö~ããçé~íÜó=~åÇ=ã~äáÖå~åÅóÔ=Ñçê=ãçêÉ=ÇÉí~áäKF

Infection of the peripheral nervous system

iÉéêçëó=áë=íÜÉ=ã~áå=áåÑÉÅíáçìë=åÉìêçé~íÜóK=pÅÜï~åå=ÅÉääë=~êÉ=íÜÉ=ã~áåí~êÖÉí=çÑ= íÜÉ= áåÑÉÅíáîÉ=~ÖÉåíI=jóÅçÄ~ÅíÉêáìã= äÉéê~ÉK=iÉéêçëó=Ü~ë=ÄÉÉåÉê~ÇáÅ~íÉÇ= áå=ÇÉîÉäçéÉÇ=ÅçìåíêáÉë=Äìí= áë=ëíáää= ê~ãé~åí= áå=ã~åó=~êÉ~ë=çÑ^ëá~I= ^ÑêáÅ~= ~åÇ= pçìíÜ= ^ãÉêáÅ~K= kÉìêçé~íÜó= ~äëç= çÅÅìêë= áå= ióãÉÇáëÉ~ëÉI= ïÜáÅÜ= áë= ÇìÉ= íç= _çêêÉäá~= ÄìêÖÇçêÑÉêáI= ~åÇ= áå= Üìã~åáããìåçÇÉÑáÅáÉåÅó= îáêìë= EefsF= ~åÇ= Üìã~å= qJäóãéÜçíêçéáÅ= îáêìë= íóéÉ= fEeqisJNF=êÉíêçîáê~ä=áåÑÉÅíáçåëI=Äìí=áå=íÜÉëÉ=ÅçåÇáíáçåë=íÜÉ=áåÑÉÅíáîÉ=~ÖÉåí


20


áë=åçí=ÇÉíÉÅíÉÇ= áå= íÜÉ=åÉêîÉëK= EmäÉ~ëÉ=êÉÑÉê= íç=`Ü~éíÉê=T=Ô=fåÑÉÅíáçìëåÉìêçé~íÜáÉë=Ô=Ñçê=ãçêÉ=ÇÉí~áäKF

References

NK= háåÖ=oI=dáåëÄÉêÖ=iK=qÜÉ=åÉêîÉ=ÄáçéëóW=áåÇáÅ~íáçåëI=íÉÅÜåáÅ~ä=~ëéÉÅíëI=~åÇ=ÅçåíêáÄìíáçåK

e~åÇÄ=`äáå=kÉìêçä=OMNPX=NNRW=NRRJTMK=

OK= háåÖ=oK=^íä~ë=çÑ=éÉêáéÜÉê~ä=åÉêîÉ=é~íÜçäçÖóK=^êåçäÇ=mìÄäáëÜÉêë=iíÇI=NVVVK=


21

√ Nerve section induces Wallerian degeneration. Dying-back degeneration of

axons occurs in length-dependent metabolic or toxic polyneuropathies.

√ Segmental demyelination is characterised by loss of the myelin sheath

between two nodes of Ranvier.

√ Healing of nerve lesions and subsequent clinical recovery occurs by axonal

sprouting and remyelination.

√ Diabetes and neurotoxic drugs are the main causes of neuropathies

associated with dying-back axonal degeneration.

√ Segmental demyelination occurs in Guillain-Barré syndrome and chronic

inflammatory demyelinating polyneuropathy.

√ Lesions of nerve blood vessels, abnormal endoneurial deposits, malignant

infiltration and inflammatory lesions can induce massive axonal degeneration

of nerve trunks.

Ü Key points



22


23

Clinical manifestations and examination

of patients with peripheral neuropathy

Chapter 3

Overview

This chapter breaks down the symptoms of peripheral neuropathy into motor

(mostly weakness), sensory (tingling, numbness and pain) and autonomic

manifestations such as fainting.

The different clinical presentations of neuropathy are discussed and the role of

investigations such as basic blood tests, nerve conduction studies,

electromyography (EMG), and skin, muscle and nerve biopsies are explained.

Symptoms of peripheral neuropathy

^ë= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉã= Ü~ë= ãçíçêI= ëÉåëçêó= ~åÇ= ~ìíçåçãáÅÅçãéçåÉåíëI= íÜÉáê= áåîçäîÉãÉåí= Å~å= äÉ~Ç= íç= î~êáçìë= ëóãéíçãë= ÇìÉ= íçåÉìêçé~íÜóK=

Motor manifestations

qÜÉëÉ= áåÅäìÇÉ= åÉÖ~íáîÉ= ã~åáÑÉëí~íáçåëI= ïÜáÅÜ= ~êÉ= ÇìÉ= íç= äçëë= çÑÑìåÅíáçåI= çê= éçëáíáîÉ= ã~åáÑÉëí~íáçåë= êÉä~íÉÇ= íç= ÜóéÉê~Åíáîáíó= çÑ= Ç~ã~ÖÉÇéÉêáéÜÉê~ä=ãçíçê=åÉìêçåëK


Negative manifestations: motor deficit

Weaknessa~ã~ÖÉÇ= éÉêáéÜÉê~ä= ãçíçê= åÉìêçåë= äçëÉ= ÑìåÅíáçåI= êÉëìäíáåÖ= áå

ïÉ~âåÉëë= ~åÇ= ãìëÅäÉ= ~íêçéÜó= ïáíÜ= ÅçãéäÉíÉ= çê= é~êíá~ä= é~ê~äóëáë= ~åÇ~ãóçíêçéÜóK= qÜÉ= ÇáëíêáÄìíáçå= ~åÇ= íÉãéç= çÑ= éêçÖêÉëëáçå= çÑ= íÜÉ= ãçíçêÇÉÑáÅáí=~êÉ=ÉñíêÉãÉäó=î~êá~ÄäÉK=qÜÉ=çåëÉí=çÑ=íÜÉ=é~ê~äóëáë=Å~å=ÄÉ=ëìÇÇÉåI~ë= áå= íê~ìã~íáÅ= åÉêîÉ= äÉëáçåëK= få= çíÜÉê= Å~ëÉëI= ~ë= áå= ëçãÉ= ÅÜêçåáÅåÉìêçé~íÜáÉëI=ïÉ~âåÉëë=éêçÖêÉëëÉë=çîÉê=óÉ~êëK= få=ëìÅÜ=Å~ëÉë=ãìëÅäÉ~íêçéÜó=ã~ó=ÄÉÅçãÉ=çÄîáçìë=äçåÖ=ÄÉÑçêÉ=íÜÉ=çåëÉí=çÑ=ïÉ~âåÉëëK=qÜáë=áëçÑíÉå=íÜÉ=Å~ëÉ=áå=ÜÉêÉÇáí~êó=ÇáëçêÇÉêë=çÑ=íÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉãK`çåîÉêëÉäóI=áå=Å~ëÉë=ïáíÜ=~å=~ÅìíÉJçåëÉí=ãçíçê=ÇÉÑáÅáíI=ãìëÅäÉ=~íêçéÜóáë=çÄëÉêîÉÇ=Ç~óë=íç=ãçåíÜë=ä~íÉê=~ÑíÉê=é~ê~äóëáëK

tÉ~âåÉëë= Å~å= ~ÑÑÉÅí= ~åó= åÉêîÉ= íÉêêáíçêó= áå= íÜÉ= äáãÄëI= íÜÉ= íêìåâ= çêÅê~åá~ä= åÉêîÉëK= tÜÉå= éêÉÇçãáå~íáåÖ= áå= Çáëí~ä= äçïÉê= äáãÄëI= íÜÉ= é~íáÉåíÅçãéä~áåë= çÑ= íêáééáåÖ= çå= êìÖë= çê= ÇáÑÑáÅìäíó= áå= ÅäáãÄáåÖ= ëí~áêëK= tÜÉåéêÉÇçãáå~íáåÖ= áå= éêçñáã~ä= äçïÉê= äáãÄë= íÜÉ= é~íáÉåí= ïáää= ÉñéÉêáÉåÅÉÇáÑÑáÅìäíó= áå=ëí~åÇáåÖ=ìéK=tÜÉå=íÜÉ=ìééÉê= äáãÄë=~êÉ=~ÑÑÉÅíÉÇ=éêçñáã~ääóIíÜÉ=é~íáÉåí=ïáää=åçíáÅÉ=ÇáÑÑáÅìäíó= áå= äáÑíáåÖ=~êãë=ìé=íç=Çç=íÜÉáê=Ü~áêK=tÜÉåÜ~åÇ=ãìëÅäÉë=~êÉ=~ÑÑÉÅíÉÇ=íÜÉ=é~íáÉåí=ïáää=ÉñéÉêáÉåÅÉ=ÇáÑÑáÅìäíó=áå=íìêåáåÖâÉóëI=ïêáíáåÖ=çê=çéÉåáåÖ=ÄçííäÉëK=jáåáã~ä=ïÉ~âåÉëë=çÑ= íÜÉ=ìééÉê= äáãÄëÅ~å= áåÇìÅÉ= íêÉãçêI= çÄîáçìë= ïÜÉå= íÜÉ= é~íáÉåí= çìíëíêÉíÅÜÉë= íÜÉ= ÑáåÖÉêëKtÉ~âåÉëë=ã~ó=~ÑÑÉÅí=~åó=é~êí=çÑ=íÜÉ=ÄçÇó=áåÅäìÇáåÖ=êÉëéáê~íçêó=ãìëÅäÉëIíÜÉ= íçåÖìÉ= ~åÇ= Ñ~Åá~ä= ãìëÅäÉëK= aÉéÉåÇáåÖ= çå= íÜÉ= ê~íÉ= çÑ= éêçÖêÉëëáçå~åÇ= áåíÉåëáíó= çÑ= íÜÉ= ÇÉÑáÅáíI= êÉëéáê~íçêó= Ñ~áäìêÉ= ã~ó= êÉèìáêÉ= êÉëéáê~íçêó~ëëáëí~åÅÉ=áå=~å=áåíÉåëáîÉ=Å~êÉ=ìåáíK

qÜÉ= éçïÉê= çÑ= ~= ãìëÅäÉ= çê= ãìëÅäÉ= Öêçìé= î~êáÉë= ïáÇÉäó= ~åÇ= áëÇÉéÉåÇÉåí= ìéçå= ~ÖÉ= ~åÇ= ÖÉåÇÉêK= jìëÅäÉ= éçïÉê= áë= ìëì~ääó= Öê~ÇÉÇ~ÅÅçêÇáåÖ=íç=íÜÉ=jÉÇáÅ~ä=`çìåÅáä=oÉëÉ~êÅÜ=pÅ~äÉ=Eq~ÄäÉ=NFK=


24


Denervation muscle atrophy jìëÅäÉ= ï~ëíáåÖ= çÅÅìêë= áå= ÇÉåÉêî~íÉÇ= ãìëÅäÉëK= fí= áë= çÄîáçìë= áå= ãçëí

Å~ëÉë=Äìí=ÉñÅÉëëáîÉ=ëìÄÅìí~åÉçìë=Ñ~í=ã~ó=ÜáÇÉ=íÜÉ=ï~ëíáåÖ=ÉëéÉÅá~ääó=áåéêçñáã~ä=äáãÄëK=t~ëíáåÖ=çÑ=ëã~ää=Ü~åÇ=ãìëÅäÉë=áë=ÅçåëéáÅìçìë=ÉëéÉÅá~ääóïÜÉå=ìåáä~íÉê~ä=çê=~ëóããÉíêáÅ~ä=EcáÖìêÉ=NFK=tÜÉå=ÇÉåÉêî~íÉÇI=íÜÉ=ÇÉäíçáÇãìëÅäÉ=~åÇLçê=íÜÉ=èì~ÇêáÅÉéë=ãìëÅäÉ=äçëÉ=Äìäâ=ê~éáÇäóK=

Chapter 3 Clinical manifestations and examination of patients with peripheral neuropathy

25

0: No contraction

1: Flicker or trace of contraction

2: Active movements possible with gravity eliminated

3: Active movements possible against gravity but not against resistance

4: Active movements possible against both gravity and resistance

5: Normal power

Table 1. Medical Council Research Scale.

Figure 1. Small hand muscle atrophy in a patient with

longstanding compression of the median nerve in the carpal

tunnel.


Positive motor manifestationsmçëáíáîÉ=ãçíçê=ëóãéíçãë=~åÇ=ëáÖåë= íÜ~í=~ÅÅçãé~åó=éÉêáéÜÉê~ä=åÉêîÉ

ÇáëÉ~ëÉI= ëìÅÜ= ~ë= Ñ~ëÅáÅìä~íáçåëI= ãóçâóãá~= ~åÇ= Åê~ãéëI= çÅÅìê= ã~áåäóÇìêáåÖ=~ÅíáîÉ=ÇÉåÉêî~íáçå=~åÇ=~ãóçíêçéÜóK=

Fibrillation and fasciculationcáÄêáää~íáçåë=~êÉ=ëéçåí~åÉçìë=ÇáëÅÜ~êÖÉë=Ñêçã=~=Öêçìé=çÑ=ëáåÖäÉ=ãìëÅäÉ

ÑáÄêÉëK=qÜÉó=êÉëìäí=áå=ãìëÅäÉ=íïáíÅÜÉë=ïÜáÅÜ=~êÉ=íçç=ëã~ää=íç=ÄÉ=îáëáÄäÉ=íçíÜÉ=å~âÉÇ=ÉóÉK=c~ëÅáÅìä~íáçåë=çÅÅìê=áå=Öêçìéë=çÑ=ãìëÅäÉ=ÑáÄêÉë=çê=áå=ïÜçäÉãçíçê= ìåáíëK= qÜÉó= ~ÅÅçãé~åó= ãìëÅäÉ= ïÉ~âåÉëë= ~åÇ= ~íêçéÜó= áå= ~åíÉêáçêÜçêå= ÅÉää= ÇáëÉ~ëÉK= c~ëÅáÅìä~íáçåëI= ïáíÜçìí= ïÉ~âåÉëë= çê= ãìëÅäÉ= ï~ëíáåÖIã~ó=ÄÉ=~=ÄÉåáÖå=éÜÉåçãÉåçåK=

Myokymia and neuromyotoniajóçâóãá~= ~ééäáÉë= íç= ìåÇìä~íáåÖ= ïçêãJäáâÉ= äçÅ~äáëÉÇ= ãçîÉãÉåíë= áå

~ÑÑÉÅíÉÇ= ãìëÅäÉëK= jóçâóãá~= áë= çÄëÉêîÉÇ= áå= Ñ~Åá~ä= ãóçâóãá~= áå= ãìäíáéäÉëÅäÉêçëáëI= ~åÇ= ÑçääçïáåÖ= êççí= äÉëáçåë= çê= åÉêîÉ= áåàìêáÉëK= kÉìêçãóçíçåá~= áëëäçï=ãìëÅäÉ=ÇÉÅçåíê~Åíáçå=áå=~=ÇÉåÉêî~íÉÇ=íÉêêáíçêóK

Segmental myoclonus pÉÖãÉåí~ä= ãóçÅäçåìë= Åçåëáëíë= çÑ= êÜóíÜãáÅ= çê= ëÉãáJêÜóíÜãáÅ= Äêáëâ

Åçåíê~Åíáçåë=çÑ=ãìëÅäÉë=áååÉêî~íÉÇ=Äó=~å=~Çà~ÅÉåí=ëéáå~ä=çê=Åê~åá~ä=åÉêîÉKjóçÅäçåìë=ÑêÉèìÉåíäó=çÅÅìêë=áå=éçëíJ_ÉääÛë=é~äëó=çê= áå=éêáã~êó=ÜÉãáÑ~Åá~äëé~ëãI=ëÉäÇçã=áå=çíÜÉê=ëáíÉëK=

Muscle cramps`ê~ãéë=~ééäó=íç=~ÄêìéíI=ìëì~ääó=é~áåÑìäI=ãìëÅäÉ=Åçåíê~ÅíáçåK=qÜÉó=~êÉ

Åçããçåäó=~ëëçÅá~íÉÇ=ïáíÜ=ÇÉåÉêî~íáçå=áå=ãçíçê=åÉìêçå=ÇáëÉ~ëÉK=qÜÉó=~êÉíÉêãáå~íÉÇ= Äó= ëíêÉíÅÜáåÖ= íÜÉ= ~ÑÑÉÅíÉÇ= ãìëÅäÉK= `ê~ãéë= ã~ó= çÅÅìê= áåÜÉ~äíÜó=éÉçéäÉ=ïáíÜçìí=éêÉÇáëéçëáåÖ=Ñ~ÅíçêëK

Tremormçëíìê~ä=~åÇ=~Åíáçå=íêÉãçêë=~êÉ=ëçãÉíáãÉë=çÄëÉêîÉÇ=áå=íÜÉ=ÅçìêëÉ=çÑ=~

ÅÜêçåáÅ=éçäóåÉìêçé~íÜóK= fí=ã~ó=ÄÉ=Çáë~ÄäáåÖK= fí= áë= íóéáÅ~ääó=çÄëÉêîÉÇ= áå=~íÉêêáíçêó= éêÉëÉåíáåÖ= ãáåáã~ä= ãçíçê= ÇÉÑáÅáíX= ãçêÉ= çÑíÉå= éçëíìê~ä= íêÉãçê~ÑÑÉÅíë=Çáëí~ä=äáãÄëK


26


Sensory manifestations

_çíÜ= äçëë= çÑ= ÑìåÅíáçå= ~åÇ= éçëáíáîÉ= ëÉåëçêó= ã~åáÑÉëí~íáçåë= Å~å= ÄÉÇáëíìêÄáåÖ=EcáÖìêÉ=OFK

Loss of function: sensory loss

Assessment during clinical examination i~êÖÉ= ãóÉäáå~íÉÇ= ëÉåëçêó= åÉêîÉ= ÑáÄêÉë= ~êÉ= ~ëëÉëëÉÇ= Äó= íÉëíáåÖ= äáÖÜí

íçìÅÜ=EÅçííçåFI=éçëáíáçå=ëÉåëÉ=çÑ=íÜÉ=ä~êÖÉ=íçÉ=~åÇ=çÑ=íÜÉ=ÑáåÖÉêëK=sáÄê~íçêóëÉåë~íáçå= áë= Éñ~ãáåÉÇ= ïáíÜ= ~= äçï= ÑêÉèìÉåÅó= íìåáåÖ= ÑçêâX= íÉãéÉê~íìêÉëÉåë~íáçå= áë= íÉëíÉÇ= Äó= ~= íÉëí= íìÄÉ= çÑ= ï~êã= çê= ÅçäÇ= ï~íÉêK= qÜÉ= íÉåÇçåêÉÑäÉñÉë=~êÉ=~äëç=ÇÉéÉåÇÉåí=çå=ä~êÖÉ=ÑáÄêÉ=ÑìåÅíáçåëK

Pain sensation m~áå=Å~å=ÄÉ=áåÇìÅÉÇ=Äó=~=ê~åÖÉ=çÑ=ëíáãìäáK=máåéêáÅâ=áë=íÜÉ=ÄÉëí=ï~ó=íç

íÉëí=é~áå=ëÉåë~íáçå=êçìíáåÉäóK=qÜÉ=êÉëìäíë=çÑ=ëÉåëçêó=íÉëíë=~êÉ=ÅçåëáÖåÉÇ=íç~= ÅÜ~êíI= ïáíÜ= çåÉ= ÅÜ~êí= Ñçê= É~ÅÜ= ëÉåë~íáçåK= nì~åíáí~íáîÉ= ëÉåëçêó= íÉëíáåÖíÉÅÜåáèìÉë= ~êÉ= ìëÉÇ= íç= ãÉ~ëìêÉ= ëÉåëçêó= íÜêÉëÜçäÇë= áå= ÅäáåáÅ~ä= éê~ÅíáÅÉIÉéáÇÉãáçäçÖáÅ=ëíìÇáÉëI=~åÇ=íÜÉê~éÉìíáÅ=íêá~äëK


27

Figure 2. Sensory manifestations.


Consequences of loss of pain sensationaáëí~ä= íêçéÜáÅ= ÅÜ~åÖÉë= ã~ó= çÅÅìê= ~ë= ~= åçåJëéÉÅáÑáÅ= ÅçåëÉèìÉåÅÉ= çÑ

ÇáÑÑÉêÉåí= ÅçåÖÉåáí~ä= çê= ~ÅèìáêÉÇ= éìêÉ= çê= éêÉÇçãáå~åíäó= ëÉåëçêóåÉìêçé~íÜáÉëI= ÉëéÉÅá~ääó= ïÜÉå= éêÉëÉêîÉÇ= ãìëÅäÉ= éçïÉê= ã~âÉë= ï~äâáåÖéçëëáÄäÉK=

Trophic ulcersmÉêÑçê~íáåÖ= ìäÅÉêë= çÑ= íÜÉ= Ñççí= êÉëìäí= Ñêçã= ãáÅêçíê~ìã~= çå= íÜÉ= éä~åí~ê

~ëéÉÅí= çÑ= íÜÉ= áåëÉåëáíáîÉ= ÑççíK= _äáëíÉêë= Å~å= ~äëç= ~ééÉ~ê= áå= ëÉåëçêóÇÉåÉêî~íÉÇ=ëâáåK=qêçéÜáÅ=ìäÅÉêë=ã~ó=ÅçãéäáÅ~íÉ=åÉìêçé~íÜó=çÑ=~åó=çêáÖáåIäÉ~ÇáåÖ=íç=ëÉåëçêó=äçëëI=ïáíÜ=éêÉëÉêî~íáçå=çÑ=ãìëÅäÉ=ëíêÉåÖíÜ=éÉêãáííáåÖ=~åçêã~ä= çê= åÉ~ê= åçêã~ä= ãçíçê= ~ÅíáîáíóK= qÜÉ= ãçëí= Åçããçå= åÉìêçé~íÜáÉëêÉëéçåëáÄäÉ=Ñçê=íêçéÜáÅ=ÅÜ~åÖÉë=áåÅäìÇÉ=Çá~ÄÉíáÅ=éçäóåÉìêçé~íÜóI=äÉéêçìëåÉìêçé~íÜóI= ~ãóäçáÇ= éçäóåÉìêçé~íÜóI= ~äÅçÜçäáÅ= éçäóåÉìêçé~íÜó= ~åÇÜÉêÉÇáí~êó=ëÉåëçêó=åÉìêçé~íÜáÉë=EcáÖìêÉ=PFK

OsteoarthropathiespÉåëçêó=åÉìêçé~íÜáÉë=ã~ó=Å~ìëÉ=çëíÉç~êíÜêçé~íÜáÉë=çÑ=íÜÉ=äçïÉê=äáãÄëK

lëíÉç~êíÜêçé~íÜó=éêÉÇçãáå~íÉë=áå=íÜÉ=ÑÉÉíI=ìé=íç=íÜÉ=~åâäÉI=ëÉäÇçã=ãçêÉéêçñáã~ääó= EcáÖìêÉ=QFK= få= äÉéêçëó=~åÇ= áå=ÜÉêÉÇáí~êó=ëÉåëçêó=åÉìêçé~íÜáÉëI


28

Figure 3. Plantar ulcers at pressure points in a patient with

sensory diabetic polyneuropathy.


íÜÉ=ÑáåÖÉêë=Å~å=ÄÉ=~ÑÑÉÅíÉÇK=få=ëóêáåÖçãóÉäá~I=éêçñáã~ä=àçáåíë=çÑ=íÜÉ=ìééÉêäáãÄë=Å~å=ÄÉ=~ÑÑÉÅíÉÇK=m~áåäÉëë=Ñê~ÅíìêÉëI=çëíÉçäóëáë=~åÇ=çëíÉçéÜóíçëáë=~êÉçÑíÉå= ~ëëçÅá~íÉÇK= cççí= ÇÉÑçêã~íáçå= ~åÇ= ~ãéìí~íáçå= çÑ= íçÉë= çÑíÉå= êÉëìäíÑêçã=áåÑÉÅíáçåI=éÉêÑçê~íáåÖ=ìäÅÉêë=~åÇ=çëíÉç~êíÜêáíáëK

Positive manifestations: spontaneous painsm~áåë=~êÉ=íÜÉ=ãçëí=ÇáëíìêÄáåÖ=ëóãéíçãë=çÑ=åÉìêçé~íÜó=EÜ ëÉÉ=Å~ëÉ

ëíìÇó=çîÉêäÉ~ÑFK=qÜÉó=~êÉ=ÇÉëÅêáÄÉÇ=~ë=ÄìêåáåÖI=íáåÖäáåÖI=éáåë=~åÇ=åÉÉÇäÉëIëíêáÅíáçå=EÑÉÉäáåÖë=çÑ=ÅçåëíêáÅíáçåFI=ï~äâáåÖ=çå=êçÅâëI= äáÖÜíåáåÖI=ÉíÅK=m~áåëÅçããçåäó=êÉîÉ~ä=ëÉåëçêó=éçäóåÉìêçé~íÜóK=m~áå=áë=çÑíÉå=ïçêëÉ=~í=åáÖÜí=~åÇÑçääçïáåÖ= ~ÅíáîáíóK= fí= áë= ~äëç= ã~ÇÉ= ïçêëÉ= Äó= Åçåí~Åí= Ô= ~ë= áå= Å~ìë~äÖá~KlÑíÉåI=ëíáãìäá=ïÜáÅÜ=~êÉ=åçí=åçêã~ääó=é~áåÑìä=~êÉ=é~áåÑìä=áå=~ÑÑÉÅíÉÇ=~êÉ~ëXíÜáë=áë=Å~ääÉÇ=~ääçÇóåá~K

içÅ~äáë~íáçå= çÑ= ëóãéíçãëI= ÉëéÉÅá~ääó= é~áåëI= ëÉåëçêó= äçëë= ~åÇ= ãçíçêÇÉÑáÅáí=~êÉ=ÅêìÅá~ä=íç=áÇÉåíáÑó=íÜÉ=åÉìêçé~íÜáÅ=é~ííÉêå=~åÇ=éÉêãáí=äçÅ~äáë~íáçåçÑ= äÉëáçåë= íç=~=ëéáå~ä= êççíI=~ë= áå=ëÅá~íáÅ~X= íç=~=åÉêîÉ= íêìåâI=~ë= áå=ãÉÇá~ååÉêîÉ=ÅçãéêÉëëáçå=áå=íÜÉ=Å~êé~ä=íìååÉäK=få=çíÜÉê=Å~ëÉëI=íÜÉ=åÉìêçé~íÜó=áëÖÉåÉê~äáëÉÇK= b~ÅÜ= çÑ= íÜÉëÉ= é~ííÉêåë= çÑ= åÉìêçé~íÜó= êÉèìáêÉë= ëéÉÅáÑáÅáåîÉëíáÖ~íáçåK=


29

Figure 4. Severe osteoarthropathy in a patient with sensory

alcoholic polyneuropathy.


Autonomic dysfunction

póãéíçãë= çÑ= ~ìíçåçãáÅ= ÇóëÑìåÅíáçå= ~êÉ= Åçããçå= Äìí= ~êÉ= çÑíÉåãáëáåíÉêéêÉíÉÇK=_ä~ÅâçìíëI=Ñ~áåíåÉëë=~åÇ=ÇáòòáåÉëë=çê=îáëì~ä=çÄëÅìê~íáçå=çåëí~åÇáåÖ= ~êÉ= ÑêÉèìÉåí= Åçãéä~áåíëK= qÜÉó= êÉÑäÉÅí= íÜÉ= ÉÑÑÉÅíë= çÑ= çêíÜçëí~íáÅÜóéçíÉåëáçå= çå= Äê~áå= éÉêÑìëáçåK= qÜÉó= ~êÉ= çÑíÉå= ~ëëçÅá~íÉÇ= ïáíÜ= ëçãÉÅÜêçåáÅ=éêçÖêÉëëáîÉ=ëÉåëçêó=åÉìêçé~íÜáÉëK=_äççÇ=éêÉëëìêÉ=~åÇ=éìäëÉ=ê~íÉ


30

Painful peripheral neuropathy

Mark is a 35-year-old right-handed former plumber who had a 5-year history of

progressive painful feet that had resulted in him being unable to continue his

occupation. His ability to walk had been severely curtailed and he spent his days

sitting at home watching TV.

On examination tone and power were normal throughout. Sensory examination

revealed a decreased pinprick sensation to three quarters of the way up the

forearm of the right arm and to the left elbow. There was decreased pinprick

sensation all the way up to the mid-thighs but he had hypersensitivity to pinprick

on the soles of his feet. Cold sensation was lost to half way up the forearm

bilaterally and half the way up the right shin, and to somewhere above the left

knee. Joint position sense was normal in the upper extremities, and reduced in the

toes, in that small excursions he did not know which way they were going, but

large excursions he did. Vibration sensation was intact. The deep tendon reflexes

were all present including ankle jerks, and the plantars were flexor. His gait was

slightly wide-based and tentative.

He was thoroughly investigated as to the cause of his neuropathy without an

underlying cause being identified. In desperation he was given a trial of steroids on

which he improved. Having failed immunosuppressive therapies he was long-term

managed on small doses of steroids and intermittent intravenous immunoglobulin

with a gradual reduction in his pain over a number of years.


ãÉ~ëìêÉãÉåí=ïÜáäëí=äóáåÖI=íÜÉå=~ÑíÉê=çåÉ=ãáåìíÉ=áå=~=ëí~åÇáåÖ=éçëáíáçå=ïáääÇÉíÉÅí=éçëíìê~ä=ÜóéçíÉåëáçå=ïáíÜ=~=ÑáñÉÇ=éìäëÉ=ê~íÉK=

kÉìêçÖÉåáÅ= ã~äÉ= áãéçíÉåÅÉ= áë= ÉñíêÉãÉäó= Åçããçå= áå= Çá~ÄÉíáÅ= ~åÇ= áå~ãóäçáÇ= éçäóåÉìêçé~íÜáÉëK= fí= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= íÜÉ= ~ÄëÉåÅÉ= çÑ= ~åÉêÉÅíáçå=~í=~åó= íáãÉ=ìåÇÉê=~åó=ÅáêÅìãëí~åÅÉëK=kÉìêçÖÉåáÅ=Ää~ÇÇÉê=ã~óéêÉëÉåí= ïáíÜ= íÜÉ= Åçãéä~áåí= çÑ= áåÅêÉ~ëáåÖ= áåíÉêî~äë= ÄÉíïÉÉå= îçáÇáåÖK`Ü~ê~ÅíÉêáëíáÅ~ääó=íÜÉ=é~íáÉåí=ïáíÜ=~=åÉìêçÖÉåáÅ=Ää~ÇÇÉê=Ü~ë=êÉëáÇì~ä=ìêáåÉïÜáÅÜ=áë=~å=áãéçêí~åí=êáëâ=Ñ~Åíçê=Ñçê=áåÑÉÅíáçå=~åÇ=ëÉéëáëK=d~ëíêçé~êÉëáëIÇá~êêÜçÉ~= ~åÇ= Åçåëíáé~íáçå= ~êÉ= ã~åáÑÉëí~íáçåë= çÑ= Ö~ëíêçáåíÉëíáå~ä~ìíçåçãáÅ=åÉìêçé~íÜó=ïÜáÅÜ=ã~ó=äÉ~Ç=íç=ã~äåìíêáíáçå=~åÇ=äçëë=çÑ=ïÉáÖÜíK=

Distribution of clinical manifestations

^ëëÉëëãÉåí=çÑ=íÜÉ=ÇáëíêáÄìíáçå=çÑ=ëáÖåë=~åÇ=ëóãéíçãë=çÑ=åÉìêçé~íÜáÉëáë= ÅêìÅá~ä= íç= ÇáêÉÅí= ÑìêíÜÉê= áåîÉëíáÖ~íáçåë= ~åÇ= é~íáÉåí= ã~å~ÖÉãÉåíK= qÜÉåÉìêçé~íÜó=Å~å=ÄÉ=ÑçÅ~ä=çê=ãìäíáÑçÅ~äI=çê=ÖÉåÉê~äáëÉÇK

Focal and multifocal neuropathies

pìÄëÉèìÉåí= áåîÉëíáÖ~íáçåë=ïáää=ÇÉéÉåÇ=çå= íÜÉ=Ç~í~=ÅçääÉÅíÉÇ=~ÑíÉê= íÜÉÑáêëí=Éñ~ãáå~íáçå=EcáÖìêÉ=RFK=^=ÑçÅ~ä=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáí=éçáåíë=íç=äÉëáçåë=çÑíÜÉ= ëéáå~ä= êççíëI= éäÉñìëÉë= çê= åÉêîÉ= íêìåâëK= få= íÜáë= êÉëéÉÅí= íÜÉ= ~ÅÅìê~íÉÇÉíÉêãáå~íáçå=çÑ=íÜÉ=ÉîÉåíë=íÜ~í=éêÉÅÉÇÉÇ=íÜÉ=çåëÉí=çÑ=ëóãéíçãë=áë=ÅêáíáÅ~äëáåÅÉ=ÑçÅ~ä=êççí=~åÇ=åÉêîÉ=äÉëáçåë=çÑíÉå=êÉëìäí=Ñêçã=íê~ìã~I=ÅçãéêÉëëáçåçê= ïçìåÇëK= `çãéêÉëëáçå= Äó= ëéáå~ä= ÇáëÅ= ÜÉêåá~íáçå= EÜÉêåá~íÉÇ= åìÅäÉìëéìäéçëìëF=áë=~=Åçããçå=Å~ìëÉ=çÑ=ê~ÇáÅìä~ê=ÇÉÑáÅáí=áå=íÜÉ=ÅÉêîáÅ~ä=~åÇ=äìãÄ~êêÉÖáçåëK=

táíÜ= êÉÖ~êÇ= íç= äÉëáçåë= çÑ= åÉêîÉ= íêìåâëI= áå= íÜÉ= ìééÉê= äáãÄë= íÜÉ= ê~Çá~äåÉêîÉ=áë=Åçããçåäó=~ÑÑÉÅíÉÇ=áå=íÜÉ=êÉÖáçå=çÑ=íÜÉ=ÜìãÉê~ä=ÖêççîÉK=qÜáë=Å~åçÅÅìê=ÇìêáåÖ=ìåÅçåëÅáçìëåÉëë=Ñêçã=~å~ÉëíÜÉëá~I=ÜÉ~îó=ÇêáåâáåÖ=çê=ÇìêáåÖéêçÑçìåÇ=ëäÉÉéK=qÜÉ=ìäå~ê=åÉêîÉ= áë=Åçããçåäó=~ÑÑÉÅíÉÇ=Äó=Éåíê~éãÉåí=~ííÜÉ=ÉäÄçïK=båíê~éãÉåí=çÑ=íÜÉ=ãÉÇá~å=åÉêîÉ=~í=íÜÉ=ïêáëí=äÉîÉäI=áå=íÜÉ=Å~êé~äíìååÉäI=áë=é~êíáÅìä~êäó=Åçããçå=áå=çäÇÉê=ïçãÉåK


31


iÉëáçåë=çÑ= íÜÉ=Äê~ÅÜá~ä=éäÉñìë=Å~å= áåÇìÅÉ=~=î~êáÉíó=çÑ=ã~åáÑÉëí~íáçåëKfåîçäîÉãÉåí=çÑ= íÜÉ=ìééÉê=éçêíáçå=~êáëáåÖ= Ñêçã=`RJ`T= áåÇìÅÉë=ïÉ~âåÉëë~åÇ= ~íêçéÜó= çÑ= íÜÉ= ëÜçìäÇÉê= ~åÇ= ìééÉê= ~êã= ãìëÅäÉëK= içïÉê= Äê~ÅÜá~äéäÉñìë= áåîçäîÉãÉåí= E~êáëáåÖ= Ñêçã= ÙI= qNF= éêçÇìÅÉë= Çáëí~ä= ïÉ~âåÉëëI~íêçéÜó=~åÇ=~=ëÉåëçêó=ÇÉÑáÅáí=áå=íÜÉ=ÑçêÉ~êã=~åÇ=Ü~åÇK=qÜÉ=ìëì~ä=Å~ìëÉëçÑ= ìééÉê= Äê~ÅÜá~ä= éäÉñìë= äÉëáçåë= áåÅäìÇÉ= íê~ìã~I= Äê~ÅÜá~ä= åÉìêáíáë= ~åÇÇ~ã~ÖÉ=Ñêçã=ê~Çá~íáçåK=içïÉê=Äê~ÅÜá~ä=éäÉñìë= äÉëáçåë=~êÉ=ìëì~ääó=ÇìÉ=íçã~äáÖå~åí=áåÑáäíê~íáçåI=ÅÉêîáÅ~ä=êáÄëI=Äê~ÅÜá~ä=åÉìêáíáë=çê=íê~ìã~K=

jìäíáÑçÅ~ä= ÇÉÑáÅáíë= ~êÉ= ëÉäÇçã= ÇìÉ= íç= ãìäíáéäÉ= êççí= áåîçäîÉãÉåíX= ãçêÉçÑíÉå= íÜÉó=~êÉ=ÇìÉ= íç=ãìäíáéäÉ= äÉëáçåë=çÑ= íÜÉ=åÉêîÉ= íêìåâ=~ë=çÅÅìêêáåÖ= áåäÉéêçëó=çê=ëóëíÉãáÅ=î~ëÅìäáíáëK=


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Figure 5. Focal and multifocal neuropathies.


Generalised neuropathy

qÜÉ=ÇáëíêáÄìíáçå=~åÇ=é~ííÉêå=çÑ=ëÉåëçêó=~åÇ=ãçíçê=ëóãéíçãë=ÖìáÇÉ=íÜÉáåîÉëíáÖ~íáçåI= ÇáÑÑÉêÉåíá~ä= Çá~Öåçëáë= ~åÇ= ã~å~ÖÉãÉåí= çÑ= éÉêáéÜÉê~äåÉìêçé~íÜáÉë= EcáÖìêÉ= SFK= få= ÖÉåÉê~äáëÉÇ= éçäóåÉìêçé~íÜáÉë= íÜÉ= é~ííÉêå= çÑëÉåëçêó=äçëë=ÇÉéÉåÇë=çå=íÜÉ=íóéÉ=çÑ=åÉêîÉ=ÑáÄêÉë=éêÉÇçãáå~åíäó=áåîçäîÉÇKtÜÉå=ëã~ää=ãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇ=åÉêîÉ=ÑáÄêÉë=~êÉ=ã~áåäó=~ÑÑÉÅíÉÇIëÉåëçêó= ~äíÉê~íáçå= êÉä~íáåÖ= íç= é~áå= ~åÇ= íÉãéÉê~íìêÉ= éÉêÅÉéíáçåéêÉÇçãáå~íÉëI= ïÜáäëí= äáÖÜí= íçìÅÜI= éçëáíáçå= ~åÇ= îáÄê~íçêó= ëÉåëÉë= ~êÉéêÉëÉêîÉÇK=`çåîÉêëÉäóI=ïÜÉå=é~íÜçäçÖó=éêÉÇçãáå~åíäó=~ÑÑÉÅíë= íÜÉ= ä~êÖÉêãóÉäáå~íÉÇ=ÑáÄêÉëI=éêçéêáçÅÉéíáçå=~åÇ=äáÖÜí=íçìÅÜ=~êÉ=~ÑÑÉÅíÉÇI=çÑíÉå=ïáíÜãçíçê= ÇÉÑáÅáíëK= qÜÉ= ÇáëíêáÄìíáçå= çÑ= ëÉåëçêó= ~åÇ= ãçíçê= ÅÜ~åÖÉë= áë= ~äëçáãéçêí~åíK


33

Figure 6. Generalised polyneuropathies.


Length-dependent polyneuropathy

Small fibre neuropathyfå=íÜáë=åÉìêçé~íÜáÅ=é~ííÉêå=ëáÖåë=~åÇ=ëóãéíçãë=ëí~êí=~åÇ=êÉã~áå=ãçêÉ

éêçåçìåÅÉÇ=áå=íÜÉ=ÑÉÉíI=íÜÉå=~ÑÑÉÅí=ãçêÉ=éêçñáã~ä=é~êíë=çÑ=íÜÉ=äçïÉê=äáãÄë~åÇ= ÉîÉåíì~ääó= íÜÉ= Çáëí~ä= ìééÉê= äáãÄëI= ïÜáÅÜ= ëìÖÖÉëíë= íÜ~í= íÜÉ= äçåÖÉëíåÉêîÉ= ÑáÄêÉë= ~êÉ= ~ÑÑÉÅíÉÇ= ÑáêëíK= pìÄëÉèìÉåíäóI= ëÜçêíÉê= ëÉåëçêó= ~ñçåë= ~êÉáåîçäîÉÇI=êÉëìäíáåÖ=áå=åÉìêçé~íÜáÅ=ã~åáÑÉëí~íáçåë=áå=ãçêÉ=éêçñáã~ä=é~êíë=çÑíÜÉ= äáãÄë= ~åÇ= ÉîÉåíì~ääó= íÜÉ= ~åíÉêáçê= íêìåâK= m~áå= ~åÇ= íÉãéÉê~íìêÉÇáëÅêáãáå~íáçå=áë=Ñáêëí=áãé~áêÉÇ=áå=íÜÉ=ÑÉÉíI=çÑíÉå=äÉ~ÇáåÖ=íç=é~áåäÉëë=íê~ìã~~åÇ= éä~åí~ê= ìäÅÉêëK= içëë= çÑ= íÉãéÉê~íìêÉ= ÇáëÅêáãáå~íáçå= íÜÉå= éêçÖêÉëëÉëìéï~êÇK= qÜáë= áë= çÑíÉå= êÉÑÉêêÉÇ= íç= ~ë= ~= äÉåÖíÜJÇÉéÉåÇÉåí= é~ííÉêå= ~åÇéêçÖêÉëëáçå= çÑ= éçäóåÉìêçé~íÜóI= ïÜáÅÜ= áë= Åçããçå= áå= Çá~ÄÉíÉëI= Äìí= ~äëççÅÅìêë=áå=~äÅçÜçäáÅ=~åÇ=~ãóäçáÇ=éçäóåÉìêçé~íÜóK=

Predominant large fibre involvementfå= íÜáë= ëÉííáåÖ= éêçéêáçÅÉéíáçå= áë= áãé~áêÉÇ= ïáíÜ= äçëë= çÑ= éçëáíáçå= ~åÇ

îáÄê~íçêó=ëÉåëÉë=áå=Çáëí~ä=äçïÉê=äáãÄëK=qÉåÇçå=êÉÑäÉñÉë=~êÉ=~ÄçäáëÜÉÇK=qÜáëé~ííÉêå= çÑ= åÉìêçé~íÜó= áë= çÄëÉêîÉÇ= áå= ~äÅçÜçäáÅ= éçäóåÉìêçé~íÜóI= áåé~ê~åÉçéä~ëíáÅ= ëÉåëçêó= éçäóåÉìêçé~íÜóI= çê= áå= ~ëëçÅá~íáçå= ïáíÜ= ÇáÑÑÉêÉåííóéÉë= çÑ= ÜÉêÉÇáí~êó= ëéáå~ä= ÅÉêÉÄÉää~ê= ~íêçéÜóK= få= `Ü~êÅçíJj~êáÉJqççíÜëóåÇêçãÉ= EÜÉêÉÇáí~êó= ãçíçê= ~åÇ= ëÉåëçêó= åÉìêçé~íÜóF= íÜÉêÉ= áë= ~éêÉÇçãáå~åí= áåîçäîÉãÉåí= çÑ= ä~êÖÉê= ãóÉäáå~íÉÇ= ÑáÄêÉë= íÜ~í= éêçÖêÉëëÑçääçïáåÖ=~=äÉåÖíÜJÇÉéÉåÇÉåí=é~ííÉêåK

Non-length-dependentfå= íÜáë= é~ííÉêå= ëáÖåë= ~åÇ= ëóãéíçãë= ~ÑÑÉÅí= éêçñáã~ä= ~ë= ïÉää= ~ë= Çáëí~ä

äáãÄëI=~åÇ=ÉîÉå=Åê~åá~ä=åÉêîÉëI=~ë=áå=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜó=çÑ=íÜÉdìáää~áåJ_~êê¨JpíêçÜä= íóéÉ= Edìáää~áåJ_~êê¨= ëóåÇêçãÉFK= qÜáë= áë= ~åáåÑä~ãã~íçêóI= éêÉëìã~Ääó= ~ìíçáããìåÉ= ÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜó~ÑÑÉÅíáåÖ=íÜÉ=éêçñáã~ä=~ë=ãìÅÜ=~ë=íÜÉ=Çáëí~ä=äáãÄë=~åÇ=Åê~åá~ä=åÉêîÉëK=

pÉåëçêó= åÉìêçåçé~íÜó= ~åÇ= ëÉåëçêó= Ö~åÖäáçåÉìêçé~íÜó= ~êÉ~ëóããÉíêáÅ~äI=ëÉåëçêóI=é~áåÑìä=~åÇ=~í~ñáÅ=åÉìêçé~íÜáÉëI=çÑíÉå=~ëëçÅá~íÉÇïáíÜ=Å~åÅÉêK=


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Course of clinical manifestations

qÜÉ=ÜáëíçêóI=åÉìêçäçÖáÅ~ä=é~ííÉêå=~åÇ=ÅçìêëÉ=çÑ=íÜÉ=åÉìêçé~íÜó=~êÉ=íÜÉãçëí=áãéçêí~åí=ÅäìÉë=íç=Çá~ÖåçëáëK=qÜÉ=Üáëíçêó=çÑ=ëóãéíçãëI=Ä~ÅâÖêçìåÇçÑ=é~íáÉåíëI=éêÉîáçìë=ãÉÇáÅ~íáçåëI=Ü~Äáíë=ÅçåÅÉêåáåÖ=~äÅçÜçäI=åìíêáíáçåI=íÜÉéêÉëÉåÅÉ=çÑ=ìåÇÉêäóáåÖ=ÇáëçêÇÉêëI=~åÇ=íÜÉ=çÅÅìêêÉåÅÉ=çÑ=ëáãáä~ê=ëóãéíçãëáå=Ñ~ãáäó=ãÉãÄÉêë=~êÉ=áãéçêí~åíK=qÜÉ=ÅçìêëÉ=çÑ=íÜÉ=åÉìêçé~íÜó=Ô=~ÅìíÉIëìÄ~ÅìíÉ=éêçÖêÉëëáîÉI=êÉä~éëáåÖ=çê=ÅÜêçåáÅ=Ô=áë=~äëç=ëáÖåáÑáÅ~åíK

Electrophysiological tests

qÜÉ= ÉäÉÅíêçÇá~ÖåçëíáÅ= Éî~äì~íáçå= áë= ~å= ÉñíÉåëáçå= çÑ= íÜÉ= éÜóëáÅ~äÉñ~ãáå~íáçåK= bäÉÅíêçéÜóëáçäçÖáÅ~ä= áåîÉëíáÖ~íáçåë= î~êó= ~ÅÅçêÇáåÖ= íç= íÜÉåÉìêçäçÖáÅ~ä= ÑáåÇáåÖë= çå= ÅäáåáÅ~ä= Éñ~ãáå~íáçåK= qÜÉ= ã~áå= éìêéçëÉ= çÑ= íÜÉÉäÉÅíêçÇá~ÖåçëíáÅ= Éî~äì~íáçå= çÑ= éÉêáéÜÉê~ä= åÉìêçãìëÅìä~ê= ÑìåÅíáçå= áë= íçÇÉíÉêãáåÉ= íÜÉ= ÇÉÖêÉÉI= ÉñíÉåí= ~åÇ= íóéÉ= çÑ= é~íÜçéÜóëáçäçÖáÅ~ä= ÅÜ~åÖÉëKkÉÉÇäÉ=ÉäÉÅíêçãóçÖê~éÜó=EbjdF=~åÇ=íÜÉ=ëíìÇó=çÑ=åÉêîÉ=~Åíáçå=éçíÉåíá~äë~åÇ= ÅçåÇìÅíáçå= ÅçåíêáÄìíÉ= íç= íÜÉ= ÇÉíÉÅíáçå= çê= ÅçåÑáêã~íáçå= çÑ= äçÅ~äáëÉÇåÉêîÉ=äÉëáçåëK=kÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉë=Å~å=ÇáÑÑÉêÉåíá~íÉ=ÄÉíïÉÉå=~ñçå~äçê=ÇÉãóÉäáå~íáåÖ=åÉìêçé~íÜáÉëI=ïÜáÅÜ=~êÉ=áãéçêí~åí=ÅäìÉë=íç=Çá~ÖåçëáëK

qÜÉ= ã~áå= ~áã= çÑ= íÜÉ= bjd= Éñ~ãáå~íáçå= áë= íç= ÇÉíÉêãáåÉ= ïÜÉíÜÉêïÉ~âåÉëë=áë=ÇìÉ=íç=åÉìêçÖÉåáÅ=áåîçäîÉãÉåí=çê=íç=~=ãìëÅäÉ=ÇáëçêÇÉê=~ë=áåãóçé~íÜóK= få=ÅçåîÉåíáçå~ä=Çá~ÖåçëíáÅ=ëíìÇáÉë= íÜÉ=ãìëÅäÉ= áë=éêçÄÉÇ=Äó=~ÅçåÅÉåíêáÅ= åÉÉÇäÉ= çê= Äó= ~å= áåëìä~íÉÇ= ãçåçéçä~ê= åÉÉÇäÉK= qÜÉ= bjdáåÅäìÇÉë=êÉÅçêÇáåÖ=~í=êÉëíI=ïÉ~â=ÉÑÑçêí=~åÇ=ã~ñáã~ä=îçäìåí~êó=Åçåíê~ÅíáçåKfí=Å~å=ÄÉ=é~áåÑìä=Ñçê=íÜÉ=é~íáÉåíK=qÜÉ=ÑáåÇáåÖë=ÇáÑÑÉê=~ÅÅçêÇáåÖ=íç=íÜÉ=ÇÉÖêÉÉçÑ= ÇÉåÉêî~íáçå= ~åÇ= íÜÉ= íÉãéçê~ä= éêçÑáäÉ= çÑ= íÜÉ= ÇáëÉ~ëÉK= qÜÉ= é~ííÉêå= çÑÇÉåÉêî~íáçå= áå= ëçãÉ= ãìëÅäÉë= ÜÉäéë= íç= áÇÉåíáÑó= íÜÉ= åÉêîÉ= êççíI= éäÉñìë= çêåÉêîÉ=íêìåâ=~ÑÑÉÅíÉÇK

kÉêîÉ= ÅçåÇìÅíáçå= ëíìÇáÉë= ~êÉ= ÅêìÅá~ä= íç= ÇáÑÑÉêÉåíá~íáåÖ= ÄÉíïÉÉå= ~å~ñçå~ä=éêçÅÉëë=ïáíÜ=äçëë=çÑ=~ñçåë=Ô=~ë=áå=t~ääÉêá~å=ÇÉÖÉåÉê~íáçå=Ô=Ñêçã~= äçëë= çÑ= ãóÉäáå= áå= ~= ÇÉãóÉäáå~íáåÖ= éêçÅÉëë= ëìÅÜ= ~ë= dìáää~áåJ_~êê¨ëóåÇêçãÉK=få=êçìíáåÉ=åÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉë=êÉëéçåëÉë=~êÉ=ÉîçâÉÇ=ÄóÉäÉÅíêáÅ~ä=ëíáãìäá=~åÇ=ÅçãéçìåÇ=êÉëéçåëÉë=~êÉ=êÉÅçêÇÉÇ=Ñêçã=ãìëÅäÉë=çêëÉåëçêó= åÉêîÉëK= qÜÉ= ÅçãéçìåÇ= ãìëÅäÉ= ~Åíáçå= éçíÉåíá~ä= E`j^mF= áë= íÜÉ


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ëìã=çÑ=ãçíçê=ìåáí=~Åíáçå=éçíÉåíá~äë=áå=íÜÉ=ãìëÅäÉK=qÜÉ=ÅçãéçìåÇ=ëÉåëçêó~Åíáçå= éçíÉåíá~ä= Epk^mF= áë= íÜÉ= ëìã= çÑ= ~Åíáçå= éçíÉåíá~äë= Ñêçã= áåÇáîáÇì~äëÉåëçêó= åÉêîÉ= ÑáÄêÉëK= qÜÉ= ~ãéäáíìÇÉëI= ëÜ~éÉë= ~åÇ= ä~íÉåÅáÉë= çÑ= ~ÅíáçåéçíÉåíá~äë=ÅÜ~åÖÉ=~ÅÅçêÇáåÖ=íç=íÜÉ=åìãÄÉê=~åÇ=ÑìåÅíáçå=çÑ=êÉäÉî~åí=åÉêîÉÑáÄêÉëK=pk^më=~êÉ=~Äçìí=NMMMJÑçäÇ=ëã~ääÉê= íÜ~å=íÜÉ=`j^më=~åÇ=ìëì~ääóêÉèìáêÉ= ~îÉê~ÖáåÖ= EêÉÅçêÇáåÖ= ãìäíáéäÉ= ÉîÉåíë= ~åÇ= í~âáåÖ= íÜÉ= ãÉ~åF= íçáåÅêÉ~ëÉ= íÜÉ= ëáÖå~äJíçJåçáëÉ= ê~íáçK= qÜÉó= ~êÉ= êÉÅçêÇÉÇ= ìëáåÖ= ëìêÑ~ÅÉ= çêåÉÉÇäÉ=ÉäÉÅíêçÇÉëK=`çåÇìÅíáçå=íáãÉ=áë=~ëëÉëëÉÇ=Äó=ãÉ~ëìêáåÖ=íÜÉ=ä~íÉåÅóÑêçã=íÜÉ=ëíáãìäìë=ëí~êí=íç=íÜÉ=~êêáî~ä=çÑ=íÜÉ=êÉëéçåëÉK=qÜÉ=~ãéäáíìÇÉ=çÑ=íÜÉpk^m=áë=ÜáÖÜäó=ëÉåëáíáîÉ=íç=~ñçå~ä=äçëëK=

`çåîÉåíáçå~ä= åÉìêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= Å~ååçí= ~ëëÉëë= íÜÉé~íÜçéÜóëáçäçÖó=çÑ=ëã~ää=ãóÉäáå~íÉÇ=çê=ìåãóÉäáå~íÉÇ=åÉêîÉ=ÑáÄêÉë=ÇìÉ=íçíÜÉáê= ÜáÖÜ= íÜêÉëÜçäÇ= íç= ÉäÉÅíêáÅ~ä= ëíáãìä~íáçå= ~åÇ= íÜÉ= ëã~ää= ~ãéäáíìÇÉë= çÑíÜÉáê= ~Åíáçå= éçíÉåíá~äëK= aÉÖêÉÉë= çÑ= ÅçåÇìÅíáçå= ëäçïáåÖ= íÜ~í= Å~ååçí= ÄÉÉñéä~áåÉÇ=Äó=ÑáÄêÉ=äçëë=çê=~ñçå~ä=~íêçéÜó=~êÉ=ÅçåëáëíÉåí=ïáíÜ=ÇÉãóÉäáå~íáçåK=

Imaging of the PNS

mêçÖêÉëë=áå=ã~ÖåÉíáÅ=êÉëçå~åÅÉ=áã~ÖáåÖ=EjofF=éÉêãáíë=áíë=ìëÉ=áå=íÜÉÇÉíÉÅíáçå=çÑ=éêáã~êó=åÉêîÉ=ëÜÉ~íÜ= íìãçìêë=ëìÅÜ=~ë=ëÅÜï~ååçã~ë=~åÇåÉìêçÑáÄêçã~ëK= jof= ÜÉäéë= íç= áÇÉåíáÑó= íÜÉ= ãçêéÜçäçÖáÅ~ä= ÅÜ~ê~ÅíÉêáëíáÅë~åÇ=ëé~íá~ä=ÉñíÉåí=çÑ=ëéáå~ä=êççíëI=~åÇ=Äê~ÅÜá~ä=~åÇ=äìãÄ~ê=éäÉñìë=äÉëáçåëíç= éçíÉåíá~ääó= ÖìáÇÉ= íÜÉ= åÉìêçëìêÖáÅ~ä= íêÉ~íãÉåíK= fã~ÖáåÖ= Å~å= ÄÉçÄí~áåÉÇ= ~í= Éåíê~éãÉåí= ëáíÉëI= Ñçê= Éñ~ãéäÉI= çÑ= íÜÉ= ãÉÇá~å= åÉêîÉ= áå= íÜÉÅ~êé~ä=íìååÉäI=çÑ=íÜÉ=ìäå~ê=åÉêîÉ=~í=íÜÉ=ÉäÄçïI=çê=áå=íÜÉ=Ñççí=áå=jçêíçåÛëåÉìêçã~K= räíê~ëçìåÇ= áã~ÖáåÖ= Å~å= ~äëç= ÄÉ= ìëÉÇ= íç= éêÉÅáëÉäó= äçÅ~äáëÉäÉëáçåë=çÑ=éÉêáéÜÉê~ä=åÉêîÉë=NK

Skin biopsy

pâáå=Äáçéëó= áë=~= íÉÅÜåáèìÉ= íÜ~í= áåîçäîÉë=~=Pãã=éìåÅÜ=Äáçéëó=çÑ=ëâáåí~âÉå=Ñêçã=ëí~åÇ~êÇáëÉÇ=ëáíÉë=çå=íÜÉ=äÉÖK=qÜÉ=íáëëìÉ=áë=áããìåçëí~áåÉÇ=ïáíÜ~åíáJéêçíÉáå=ÖÉåÉ=éêçÇìÅí= EmdmF=VKR=~åíáÄçÇáÉëK=qÜáë=ëí~áåáåÖ=~ääçïë= ÑçêíÜÉ=áÇÉåíáÑáÅ~íáçå=~åÇ=ÅçìåíáåÖ=çÑ=áåíê~JÉéáÇÉêã~ä=åÉêîÉ=ÑáÄêÉë=EfbkcFK=qÜáëíÉÅÜåáèìÉ= Ü~ë= ÄÉÉå= î~äáÇ~íÉÇ= ~ë= ~= êÉäá~ÄäÉ= ãÉíÜçÇ= Ñçê= fbkc= ÇÉåëáíó


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ÇÉíÉêãáå~íáçå= ïáíÜ= ÖççÇ= áåíê~J= ~åÇ= áåíÉêJçÄëÉêîÉê= êÉäá~Äáäáíó= áå= åçêã~äÅçåíêçäë= ~åÇ= é~íáÉåíë= ïáíÜ= ~= Çáëí~ä= ëóããÉíêáÅ~ä= éçäóåÉìêçé~íÜó= OK= fåé~êíáÅìä~êI= é~íáÉåíë= ïÜç= ~êÉ= ëìëéÉÅíÉÇ= çÑ= Ü~îáåÖ= ~= éêÉÇçãáå~åíäó= ëã~ääÑáÄêÉ= ëÉåëçêó= éçäóåÉìêçé~íÜó= ã~ó= ÄÉåÉÑáí= Ñêçã= ëâáå= Äáçéëó= ïáíÜ= fbkc~å~äóëáë=ëáåÅÉ=íÜáë=íÉëí=Å~å=ÜÉäé=ÅçåÑáêã=íÜÉ=Çá~ÖåçëáëK

fí=áë=Åçããçå=éê~ÅíáÅÉ=íç=êìå=~å=áããìåçÜáëíçÅÜÉãáÅ~ä=ëí~áå=Ñçê=íÜÉ=é~åJ~ñçå~ä=ã~êâÉê=éêçíÉáå=ÖÉåÉ=éêçÇìÅí=VKR=Emdm=VKRFK=qÜÉ=ëÉÅíáçåë=~êÉ=íÜÉåçÄëÉêîÉÇ= ~åÇ= ~å~äóëÉÇ= ïáíÜ= ÄêáÖÜíJÑáÉäÇ= ãáÅêçëÅçéó= çê= ïáíÜ= áåÇáêÉÅíáããìåçÑäìçêÉëÅÉåÅÉK= jçëí= ëíìÇáÉë= êÉéçêí= èì~åíáÑáÅ~íáçå= çÑ= fbkc= ÇÉåëáíóÇáëéä~óÉÇ= áå= ÄêáÖÜíJÑáÉäÇ= ãáÅêçëÅçéóK= kçêã~íáîÉ= î~äìÉë= Ü~îÉ= ÄÉÉåÉëí~ÄäáëÜÉÇI= é~êíáÅìä~êäó= Ñêçã= íÜÉ= Çáëí~ä= é~êí= çÑ= íÜÉ= äÉÖI= NMÅã= ~ÄçîÉ= íÜÉÉñíÉêå~ä= ã~ääÉçäìëK= pâáå= Äáçéëó= áë= çÑ= é~êíáÅìä~ê= î~äìÉ= áå= íÜÉ= Çá~Öåçëáë= çÑëã~ää=ÑáÄêÉ=åÉìêçé~íÜó=ïÜÉå=åÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉë=~êÉ=åçêã~äK=fí=ã~ó~äëç=ÄÉ=êÉéÉ~íÉÇ=íç=ëíìÇó=íÜÉ=éêçÖêÉëëáîÉ=å~íìêÉ=çÑ=íÜÉ=ÇáëÉ~ëÉK

Other diagnostic procedures

Routine blood tests

cçê=ãçëí=é~íáÉåíëI=~å=áåáíá~ä=ëÅêÉÉå=ÄäççÇ=íÉëí=ëÜçìäÇ=áåÅäìÇÉ=~=ÅçãéäÉíÉÄäççÇ=ÅçìåíI=ÉêóíÜêçÅóíÉ=ëÉÇáãÉåí~íáçå=ê~íÉI=~=ÅçãéêÉÜÉåëáîÉ=ãÉí~ÄçäáÅé~åÉä= EÄäççÇ=ÖäìÅçëÉI= êÉå~ä= ÑìåÅíáçåI= äáîÉê= ÑìåÅíáçåFI= íÜóêçáÇ= íÉëíëI=ëÉêìã_NOI=~åÇ=ëÉêìã=éêçíÉáå=ÉäÉÅíêçéÜçêÉëáëK=qÜÉëÉ=ÄäççÇ=íÉëíë=ïÉêÉ=áÇÉåíáÑáÉÇ~ë=ìëÉÑìä=áå=ÅìêêÉåí=ÉîáÇÉåÅÉJÄ~ëÉÇ=éê~ÅíáÅÉ=ÖìáÇÉäáåÉë=PK

Lumbar puncture and cerebrospinal fluid (CSF) examination

bñ~ãáå~íáçå= çÑ= íÜÉ= `pc= áë= ~å= áãéçêí~åí= ÅçåíêáÄìíáçå= íç= Çá~Öåçëáë= áåã~åó=Å~ëÉëK=`pc= Ñêçã= íÜÉ= äìãÄ~ê= êÉÖáçå=Åçåí~áåë=NRJQRãÖLÇi=éêçíÉáå~åÇ=RMJUMãÖLÇi=EOKUJQKQããçäLiF=ÖäìÅçëÉ=Eíïç=íÜáêÇë=çÑ=ÄäççÇ=ÖäìÅçëÉFKkçêã~ä=`pc=Åçåí~áåë=MJR=ãçåçåìÅäÉ~ê=ÅÉääë=éÉê=ãiK=qÜÉ=`pc=éêÉëëìêÉIãÉ~ëìêÉÇ=~í=äìãÄ~ê=éìåÅíìêÉ=EimFI=áë=NMMJNUMãã=çÑ=eOl=EUJNRãã=eÖFïáíÜ=íÜÉ=é~íáÉåí=äóáåÖ=çå=íÜÉáê=ëáÇÉ=~åÇ=OMMJPMMãã=ïáíÜ=íÜÉ=é~íáÉåí=ëáííáåÖìéK= qÜÉ= `pc= Å~å= ÄÉ= Çá~ÖåçëíáÅ= áå= ãÉåáåÖáíáëI= dìáää~áåJ_~êê¨= ëóåÇêçãÉIióãÉ= ÇáëÉ~ëÉ= ~åÇ= áå= ÅçåÇáíáçåë= ~ëëçÅá~íÉÇ= ïáíÜ= ã~äáÖå~åÅó= ïÜÉå


37


ã~äáÖå~åí= ÅÉääë= Å~å= ÄÉ= ÇÉíÉÅíÉÇ= áå= íÜÉ= `pc= QK= qÜÉ= ìëÉ= çÑ= ~íê~ìã~íáÅåÉÉÇäÉë= Ü~ë= ÄÉÉå= ëÜçïå= íç= ëáÖåáÑáÅ~åíäó= êÉÇìÅÉ= íÜÉ= áåÅáÇÉåÅÉ= çÑ= éçëíJäìãÄ~ê=éìåÅíìêÉ=ÜÉ~Ç~ÅÜÉ=EPBF=ïÜÉå=Åçãé~êÉÇ=íç=íÜÉ=ìëÉ=çÑ=ëí~åÇ~êÇëéáå~ä= åÉÉÇäÉë= E~ééêçñáã~íÉäó= PMBFK= mêçéÜóä~ÅíáÅ= ÄÉÇ= êÉëí= ~ÑíÉê= äìãÄ~êéìåÅíìêÉ=áë=åçí=ã~åÇ~íçêó=ëáåÅÉ=áí=Ü~ë=åçí=ÄÉÉå=ëÜçïå=íç=ÄÉ=çÑ=ÄÉåÉÑáí=áåëçãÉ=ëíìÇáÉëK

Indications for nerve biopsy R

mêçÖêÉëë= áå= åÉìêçéÜóëáçäçÖó= ~åÇ= åÉìêçÖÉåÉíáÅë= ~ë= ïÉää= ~ë= éêÉîáçìëåÉìêçé~íÜçäçÖáÅ~ä= ÑáåÇáåÖë= Ü~îÉ= ~ää= áãéêçîÉÇ= çìê= âåçïäÉÇÖÉ= çÑ= íÜÉé~íÜçéÜóëáçäçÖáÅ~ä= ÅÜ~ê~ÅíÉêáëíáÅë= çÑ= éÉêáéÜÉê~ä= åÉêîÉ= ÇáëçêÇÉêëK= kÉêîÉÄáçéëó= áë= ~= Çá~ÖåçëíáÅ= íççä= ïÜáÅÜ= áë= ~äëç= ~å= ÉñíÉåëáçå= çÑ= íÜÉ= éÜóëáÅ~äÉñ~ãáå~íáçå=~åÇ=áë=åçí=àìëí=~=ä~Äçê~íçêó=íÉëíK=qÜÉ=áåÇáÅ~íáçåë=Ñçê=åÉêîÉ=ÄáçéëóÜ~îÉ=ÇÉÅêÉ~ëÉÇ=Çê~ã~íáÅ~ääó=ÇìêáåÖ=íÜÉ=é~ëí=ÇÉÅ~ÇÉI=ã~âáåÖ=íÜáë= áåî~ëáîÉéêçÅÉÇìêÉ= ìååÉÅÉëë~êó= áå= íÜÉ= î~ëí= ã~àçêáíó= çÑ= é~íáÉåíë= ïáíÜ= éÉêáéÜÉê~äåÉìêçé~íÜóI=Äìí=ïÜÉå=ìëÉÇ=àìÇáÅáçìëäó=áí=Å~å=ÄÉ=áåÇáëéÉåë~ÄäÉ=áå=Çá~ÖåçëáåÖÅÉêí~áå= ÅçåÇáíáçåëK= qÜÉ= óáÉäÇ= çÑ= åÉêîÉ= Äáçéëó= ÇÉéÉåÇë= çå= ~= åìãÄÉê= çÑÑ~ÅíçêëI=áåÅäìÇáåÖ=íÜÉ=ëÉäÉÅíáçå=çÑ=é~íáÉåíëI=ÉñéÉêíáëÉ=çÑ=íÜÉ=ä~Äçê~íçêóI=~åÇíÉÅÜåáèìÉë=ìëÉÇI=É~ÅÜ=ëíÉé=ÄÉáåÖ=ÅêìÅá~ä=íç=íÜÉ=ìëÉÑìäåÉëë=çÑ=íÜÉ=éêçÅÉÇìêÉK=

tÜÉå= éÉêÑçêãÉÇ= áå= ìåëÉäÉÅíÉÇ= é~íáÉåíë= ïáíÜ= éÉêáéÜÉê~ä= åÉìêçé~íÜóIåÉêîÉ=Äáçéëó=Å~å=ÄÉ=îÉêó=Çáë~ééçáåíáåÖ=ÄÉÅ~ìëÉ=çÑ=íÜÉ=ä~Åâ=çÑ=ëéÉÅáÑáÅáíóçÑ=åÉêîÉ=ÑáÄêÉ=äÉëáçåëK=få=~ÇÇáíáçåI=é~íáÉåíë=ã~ó=Åçãéä~áå=çÑ=ÇáëÅçãÑçêí=~åÇ~ääçÇóåá~=Eé~áå=Ñêçã=ëíáãìäá=íÜ~í=åçêã~ääó=Çç=åçí=éêçÇìÅÉ=é~áåF=Ñçê=ãçåíÜëÑçääçïáåÖ=íÜÉ=éêçÅÉÇìêÉK=_ÉÑçêÉ=éÉêÑçêãáåÖ=~=åÉêîÉ=ÄáçéëóI=íÜÉ=åÉìêçé~íÜóãìëí= ÄÉ= áåîÉëíáÖ~íÉÇ= Å~êÉÑìääó= ~åÇ= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= áíë= áåÜÉêáí~åÅÉIÇáëíêáÄìíáçåI= ÅçìêëÉI= íÜÉ= ÖÉåÉê~ä= ÅçåíÉñí= áå= ïÜáÅÜ= áí= Ü~ë= ÇÉîÉäçéÉÇI~ëëçÅá~íÉÇ=`pc=~åÇ=ÉäÉÅíêçÇá~ÖåçëíáÅ=ÑáåÇáåÖëI=~åÇ=íÜÉ=~î~áä~Äáäáíó=çÑ=ak^íÉëíáåÖ=áå=íÜÉ=Å~ëÉ=çÑ=ÜÉêÉÇáí~êó=ÇáëÉ~ëÉK

få= ÜÉêÉÇáí~êó= åÉìêçé~íÜáÉëI= áí= áë= åçï= ëÉäÇçã= åÉÅÉëë~êó= íç= éÉêÑçêã= ~ãçêéÜçäçÖáÅ~ä=ëíìÇó=çÑ=~=åÉêîÉ=Äáçéëó=ëéÉÅáãÉåK=

^ÅèìáêÉÇI=Çáëí~äI=ëóããÉíêáÅ~äI=ÑáÄêÉ=äÉåÖíÜJÇÉéÉåÇÉåí=éçäóåÉìêçé~íÜáÉë~êÉ=éêÉÇçãáå~åíäó=ëÉåëçêó=~åÇ=ãçëíäó=çÑ=íçñáÅI=ÉëéÉÅá~ääó=ÇêìÖJáåÇìÅÉÇI=çêãÉí~ÄçäáÅ=çêáÖáåK= få=ëìÅÜ=Å~ëÉë=~=Äáçéëó=ëéÉÅáãÉå=çÑ=~=Çáëí~ä=åÉêîÉ=Å~å


38


áåÇáÅ~íÉ=íÜÉ=ëÉîÉêáíó=~åÇ=~Åíáîáíó=çÑ=íÜÉ=åÉìêçé~íÜó=Äìí=áë=ìåäáâÉäó=íç=ëÜçï~åó=ëéÉÅáÑáÅ=äÉëáçåëI=ÉñÅÉéí=ïÜÉå=~ãóäçáÇçëáë=áë=ëìëéÉÅíÉÇK

få= ãçåçåÉìêáíáë= ãìäíáéäÉñI= ~= åÉêîÉ= Äáçéëó= áë= êÉèìáêÉÇ= íç= ëÉ~êÅÜ= Ñçê~äíÉê~íáçåë= çÑ= î~ë~= åÉêîçêìãI= ~Äåçêã~ä= ÇÉéçëáíëI= çê= áåÑä~ãã~íçêóáåÑáäíê~íÉëI= ã~åó= çÑ= ïÜáÅÜ= ~êÉ= íêÉ~í~ÄäÉK= ^= ïÜçäÉJíÜáÅâåÉëë= åÉêîÉ= Äáçéëóãìëí=íÜÉå=ÄÉ=éÉêÑçêãÉÇ=ìåÇÉê=äçÅ~ä=~å~ÉëíÜÉëá~K=pÉ~êÅÜáåÖ=Ñçê=î~ëÅìäáíáëáë=íÜÉ=ã~áå=áåÇáÅ~íáçå=Ñçê=~=åÉêîÉ=ÄáçéëóK=qÜÉ=Çá~Öåçëáë=çÑ=î~ëÅìäáíáë=ëÜçìäÇÄÉ= ëìëéÉÅíÉÇ= ïÜÉå= ~= ãìäíáÑçÅ~ä= åÉìêçé~íÜó= áë= ~ëëçÅá~íÉÇ= ïáíÜéçäóëóëíÉãáÅ=ã~åáÑÉëí~íáçåëI=Äìí=~=ëìÄëí~åíá~ä=éêçéçêíáçå=çÑ=é~íáÉåíë=Ü~îÉáëçä~íÉÇ= åÉìêçé~íÜóI= ëçãÉíáãÉë= ïáíÜçìí= ÅäáåáÅ~ä= çê= ÄáçäçÖáÅ~ä= ëáÖåë= çÑëóëíÉãáÅ=áåÑä~ãã~íáçåK=s~ëÅìäáíáë=áë=ÑçìåÇ=áå=íÜÉ=ãìëÅäÉ=Äáçéëó=ëéÉÅáãÉåáå= íÜÉ= ë~ãÉ= éêçéçêíáçå= çÑ= é~íáÉåíë= áå= íÜáë= ëìÄÖêçìé= ~ë= áå= íÜçëÉ= ïáíÜëóãéíçã~íáÅ=ãìäíáëóëíÉãáÅ=áåîçäîÉãÉåíK=iÉéêçëó=áë=ëíáää=~=Åçããçå=Å~ìëÉçÑ= åÉìêçé~íÜó= áå= ëìÄíêçéáÅ~ä= ÇÉîÉäçéáåÖ= ÅçìåíêáÉëI= Äìí= Å~ëÉë= çÑ= äÉéêçìëåÉìêçé~íÜó= ~êÉ= ~äëç= ëÉÉå= áå= áåÇìëíêá~äáëÉÇ= ÅçìåíêáÉë= ëéçê~ÇáÅ~ääóK= tÜÉåëâáå=äÉëáçåë=~êÉ=ãáëëáåÖ=~åÇ=äÉéêçëó=ëìëéÉÅíÉÇI=éÉêÑçêãáåÖ=~=Äáçéëó=çÑ=~å~ÑÑÉÅíÉÇ=åÉêîÉ=áë=äáâÉäó=íç=ÄÉ=Çá~ÖåçëíáÅK

pÉäÉÅíáåÖ=íÜÉ=åÉêîÉ=íç=Äáçéëó=áë=îÉêó=áãéçêí~åíK=qÜÉ=åÉêîÉ=ëÜçìäÇ=ÄÉ=~ëÉåëçêó=åÉêîÉ=áå=~=íÉêêáíçêó=~ÑÑÉÅíÉÇ=Äó=íÜÉ=åÉìêçé~íÜáÅ=éêçÅÉëë=~åÇ=É~ëáäó~ÅÅÉëëáÄäÉ=íç=åÉìêçéÜóëáçäçÖáÅ~ä=ëíìÇáÉë=éêáçê=íç=íÜÉ=ÄáçéëóK=mÉêÑçêãáåÖ=~Äáçéëó=çÑ=~=åÉêîÉ=áå=~=íÉêêáíçêó=ïáíÜ=ã~êâÉÇ=ëÉåëçêó=äçëë=ÇÉÅêÉ~ëÉë=íÜÉ=êáëâçÑ=~ÇîÉêëÉ=ÉÑÑÉÅíë=~åÇ=áåÅêÉ~ëÉë=íÜÉ=ÅÜ~åÅÉ=çÑ=ÑáåÇáåÖ=ëáÖåáÑáÅ~åí=äÉëáçåëKpáãìäí~åÉçìë=ãìëÅäÉ=ë~ãéäáåÖ=áåÅêÉ~ëÉë=íÜÉ=ÅÜ~åÅÉë=çÑ=ÑáåÇáåÖ=î~ëÅìäáíáëçê=ë~êÅçáÇ=Öê~åìäçã~ëK=qÜìëI=ïÜÉå=íÜÉ=åÉìêçé~íÜáÅ=ÇÉÑáÅáí=éêÉÇçãáå~íÉëáå=Çáëí~ä=äçïÉê=äáãÄëI=~=Äáçéëó=çÑ=íÜÉ=ëìê~ä=çê=ëìéÉêÑáÅá~ä=éÉêçåÉ~ä=åÉêîÉ=Å~åÄÉ=éÉêÑçêãÉÇ=ïáíÜ=ëáãìäí~åÉçìë=ë~ãéäáåÖ=çÑ=~å=~Çà~ÅÉåí=ãìëÅäÉK=^ÑíÉê=~Äáçéëó=çÑ= íÜÉ=ëìê~ä=åÉêîÉ=çê= íÜÉ=ëìéÉêÑáÅá~ä=éÉêçåÉ~ä=åÉêîÉI=ïÉ=éêÉÑÉê= íçáããçÄáäáòÉ= íÜÉ= äÉÖ= Ñçê= TJNM= Ç~óë= íç= ~îçáÇ= ÉñÅÉëëáîÉ= íÉåëáçå= çîÉê= íÜÉáåÅáëáçåK= få= é~íáÉåíë= ïáíÜ= éêçñáã~ä= áåîçäîÉãÉåí= çÑ= íÜÉ= äçïÉê= äáãÄëI= íÜÉáåíÉêãÉÇá~íÉ=Åìí~åÉçìë=åÉêîÉ=çÑ=íÜÉ=íÜáÖÜ=Å~å=ÄÉ=ëÉäÉÅíÉÇ=~åÇ=~=Äáçéëó=çÑíÜÉ=èì~ÇêáÅÉéë=ãìëÅäÉ=éÉêÑçêãÉÇ=ÇìêáåÖ=íÜÉ=ë~ãÉ=éêçÅÉÇìêÉK=tÜÉå=íÜÉìééÉê= äáãÄë= ÄÉ~ê= íÜÉ= Äêìåí= çÑ= íÜÉ= åÉìêçé~íÜáÅ= éêçÅÉëëI= ~= Äáçéëó= çÑ= íÜÉëìéÉêÑáÅá~ä=ê~Çá~ä=åÉêîÉ=Å~å=ÄÉ=éÉêÑçêãÉÇ=~í=íÜÉ=äÉîÉä=çÑ=íÜÉ=ïêáëí=SI=TK


39


References

NK= píçää= dI= táäÇÉêJpãáíÜ= bI= _ÉåÇëòìë= jK= fã~ÖáåÖ= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉãK

e~åÇÄ=`äáå=kÉìêçä=OMNPX=NNRW=NPTJRPK

OK d›ê~åëëçå= idI= jÉääÖêÉå= pfI= iáåÇ~ä= pI= lãÇ~ä= oK= qÜÉ= ÉÑÑÉÅí= çÑ= ~ÖÉ= ~åÇ= ÖÉåÇÉê= çå

ÉéáÇÉêã~ä=åÉêîÉ=ÑáÄÉê=ÇÉåëáíóK=kÉìêçäçÖó=OMMQX=SOW=TTQJTK=

PK= båÖä~åÇ=gaI=dêçåëÉíÜ=dpI=cê~åâäáå=dI=Éí=~äK=mê~ÅíáÅÉ=m~ê~ãÉíÉêW=Éî~äì~íáçå=çÑ=Çáëí~ä

ëóããÉíêáÅ=éçäóåÉìêçé~íÜóW=êçäÉ=çÑ=~ìíçåçãáÅ=íÉëíáåÖI=åÉêîÉ=ÄáçéëóI=~åÇ=ëâáå=Äáçéëó=E~å

ÉîáÇÉåÅÉJÄ~ëÉÇ= êÉîáÉïFK= oÉéçêí= çÑ= íÜÉ= ^ãÉêáÅ~å= ^Å~ÇÉãó= çÑ= kÉìêçäçÖóI= ^ãÉêáÅ~å

^ëëçÅá~íáçå= çÑ= kÉìêçãìëÅìä~ê= ~åÇ= bäÉÅíêçÇá~ÖåçëíáÅ= jÉÇáÅáåÉI= ~åÇ= ^ãÉêáÅ~å

^Å~ÇÉãó=çÑ=mÜóëáÅ~ä=jÉÇáÅáåÉ=~åÇ=oÉÜ~Äáäáí~íáçåK=kÉìêçäçÖó OMMVÄX=TOEOFW=NTTJUQK

QK dçêÉäáÅâ= m_I= _áääÉê= gK= iìãÄ~ê= éìåÅíìêÉK= qÉÅÜåáèìÉI= áåÇáÅ~íáçåëI= ~åÇ= ÅçãéäáÅ~íáçåëK

mçëíÖê~Ç=jÉÇ NVUSX=TVW=ORTJSUK=

RK jÉääÖêÉå=pfI=kçä~åç=jI=pçããÉê=`K=qÜÉ=Åìí~åÉçìë=åÉêîÉ=ÄáçéëóW= íÉÅÜåáÅ~ä=~ëéÉÅíëI

áåÇáÅ~íáçåëI=~åÇ=ÅçåíêáÄìíáçåK=e~åÇÄ=`äáå=kÉìêçä=OMNPX=NNRW=NTNJUUK

SK p~áÇ=dK=fåÇáÅ~íáçåë=~åÇ=î~äìÉ=çÑ=åÉêîÉ=ÄáçéëóK=jìëÅäÉ=kÉêîÉ NVVVX=OOW=NSNTJVK

TK háåÖ=oI=dáåëÄÉêÖ=iK=qÜÉ=åÉêîÉ=ÄáçéëóW=áåÇáÅ~íáçåI=íÉÅÜåáÅ~ä=~ëéÉÅíë=~åÇ=ÅçåíêáÄìíáçåK

e~åÇÄ=`äáå=kÉìêçä=OMNPX=NNRW=NRRJTMK


40

√ Weakness, muscle atrophy, fasciculation, myokymia, cramps and tremor are

seen in denervated muscles.

√ Sensory loss induces trophic changes, painless osteoarthropathies, and

trophic ulcers.

√ Different patterns of neuropathy include focal and multifocal neuropathies or

generalised length-dependent polyneuropathies.

√ Electrophysiological tests are useful to assess axonal loss and nerve

conduction slowing in demyelinating processes.

√ MRI and ultrasound permit visualisation of nerve and root compression, and

tumours.

√ Nerve biopsy is indispensable in diagnosing the various conditions.

Ü Key points


41

Guillain-Barré syndrome

Chapter 4

Overview

This chapter discusses Guillain-Barré syndrome and its variants. The typical

presentation of the syndrome is outlined together with the results of investigations

and treatment. The mechanism of demyelination and the role of axonal lesions are

described, along with the changes in cerebrospinal fluid and nerve conduction. The

need for careful monitoring of autonomic disorders in some patients in intensive

care units is underlined. The modalities of treatment with intravenous

immunoglobulins and plasma exchange are also described.

Introduction

dìáää~áåJ_~êê¨= ëóåÇêçãÉ= Ed_pF= áë= ~å= ~ÅìíÉ= ãçíçê= ~åÇ= ëÉåëçêóéçäóåÉìêçé~íÜó=ïáíÜ=~=ëéçåí~åÉçìëäó=êÉîÉêëáÄäÉ=ÅçìêëÉ=áå=ãçëí=Å~ëÉëK=qÜÉçåëÉí= çÑ= íÜÉ= é~ê~äóíáÅ= éÜ~ëÉ= áë= çÑíÉå= éêÉÅÉÇÉÇ= Äó= ~= ÑäìJäáâÉ= ëóåÇêçãÉKjçíçê=ÇÉÑáÅáí=~ÑÑÉÅíë=~ää=Ñçìê=äáãÄë=ïáíÜ=ÑêÉèìÉåí=áåîçäîÉãÉåí=çÑ=Åê~åá~ä=~åÇêÉëéáê~íçêó= ãìëÅäÉëI= êÉèìáêáåÖ= êÉëéáê~íçêó= ~ëëáëí~åÅÉK= fåÅêÉ~ëÉÇÅÉêÉÄêçëéáå~ä=ÑäìáÇ=E`pcF=éêçíÉáå=ÅçåíÉåí=~åÇ=êÉîÉêëáÄáäáíó=çÑ=åÉìêçäçÖáÅ~äÇÉÑáÅáí=ÅÜ~ê~ÅíÉêáëÉ=íÜÉ=ëóåÇêçãÉK=pÉîÉê~ä=î~êá~åíë=Ü~îÉ=ÄÉÉå=~ÇÇÉÇ=íç=íÜÉÅä~ëëáÅ~ä= ÇÉëÅêáéíáçå= çÑ= íÜÉ= ëóåÇêçãÉI= ïÜáÅÜ= áå= ãçëí= Å~ëÉë= Ü~îÉ= ~Ñ~îçìê~ÄäÉ= çìíÅçãÉK= aáë~ÄäáåÖ= ëÉèìÉä~É= êÉã~áå= êÉä~íáîÉäó= ÅçããçåIÜçïÉîÉêK


Historical background

d_p= ï~ë= çêáÖáå~ääó= ÇÉëÅêáÄÉÇI= ~äãçëí= çåÉ= ÜìåÇêÉÇ= óÉ~êë= ~ÖçI= Äódìáää~áåI=_~êê¨=~åÇ=píêçÜä= ~ë=~=éÉÅìäá~ê= Ñä~ÅÅáÇ=é~ê~äóëáë= áå= íïç=ëçäÇáÉêëïÜç= Ü~Ç= ÇÉîÉäçéÉÇ= ~ÅìíÉ= é~ê~äóëáë= ïáíÜ= ~êÉÑäÉñá~I= Ñêçã= ïÜáÅÜ= íÜÉóêÉÅçîÉêÉÇ=ëéçåí~åÉçìëäó=NK=qÜÉ=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáí=áë=íóéáÅ~ääó=~ëëçÅá~íÉÇïáíÜ=áåÅêÉ~ëÉÇ=éêçíÉáå=ÅçåÅÉåíê~íáçå=áå=íÜÉ=`pc=ïáíÜ=~=åçêã~ä=ÅÉää=ÅçìåíK

d_p= áë= ~= éçëíJáåÑÉÅíáçìëI= áåÑä~ãã~íçêó= ~åÇ= ÇÉãóÉäáå~íáåÖ= éÉêáéÜÉê~äåÉìêçé~íÜó=E~äëç=Å~ääÉÇ=~ÅìíÉ=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜóFIÄìí= Ñçêãë= ïáíÜ= éêÉÇçãáå~åí= ~ñçå~ä= Ç~ã~ÖÉ= Ü~îÉ= ëìÄëÉèìÉåíäó= ÄÉÉåáÇÉåíáÑáÉÇK= pìÅÜ= ~ñçå~ä= Ñçêãë= çÑ= d_pI= Å~ääÉÇ= ~ÅìíÉ= ãçíçê= ~ñçå~äåÉìêçé~íÜó= E^j^kF= çê= ~ÅìíÉ= ãçíçê= ~åÇ= ëÉåëçêó= ~ñçå~ä= åÉìêçé~íÜóE^jp^kFI= ~êÉ= ãçêÉ= Åçããçå= áå= ëçãÉ= ÅçìåíêáÉë= ëìÅÜ= ~ë= `Üáå~= ~ÑíÉêáåÑÉÅíáçå=Äó=`~ãéóäçÄ~ÅíÉê=àÉàìåá OK

qÜÉ=ä~íÉëí=çîÉê~ää=Éëíáã~íáçå=Ñçê=íÜÉ=ÑêÉèìÉåÅó=çÑ=íÜáë=ÇáëçêÇÉê=áë=NKN=íçNKU=éÉê=NMMIMMM=éÉêëçåë=éÉê=óÉ~êK=d_p=áë=åçïI= áå= íÜÉ=éçëíJéçäáç=Éê~= áåÇÉîÉäçéÉÇ= ÅçìåíêáÉëI= íÜÉ= ãçëí= ÑêÉèìÉåí= Å~ìëÉ= çÑ= ~ÅìíÉ= é~ê~äóëáëIéçíÉåíá~ääó=äáÑÉJíÜêÉ~íÉåáåÖI=~åÇ=çÅÅ~ëáçå~ääó=äÉíÜ~ä=PK

Pathophysiology

cçääçïáåÖ= åçåJëéÉÅáÑáÅ= áåÑÉÅíáçåI= î~ÅÅáå~íáçå= çê= ~= ëìêÖáÅ~ä= éêçÅÉÇìêÉIé~íáÉåíë=ã~ó=ÇÉîÉäçé=~=d_p=ÇìÉ= íç=~ÅìíÉ=ÇÉãóÉäáå~íáçå=çÑ=ëéáå~ä= êççíë~åÇ=åÉêîÉ=íêìåâëI=ïáíÜçìí=ëáÖåáÑáÅ~åí=~ñçå~ä=áåîçäîÉãÉåí=áå=ãçëí=Å~ëÉë=QKqÜáë= ÇÉãóÉäáå~íáçå= êÉëìäíë= Ñêçã= ~å= ~ìíçáããìåÉ= éêçÅÉëë= ãÉÇá~íÉÇ= Äóã~ÅêçéÜ~ÖÉë= ~Åíáî~íÉÇ= Äó= ÜÉäéÉê= qJäóãéÜçÅóíÉë= ÇáêÉÅíÉÇ= ~Ö~áåëíÅçãéçåÉåíë= çÑ= íÜÉ= ãóÉäáå= ëÜÉ~íÜK= ^ÅìíÉ= ÇÉãóÉäáå~íáçå= Å~å= ~äëç= êÉëìäíÑêçã=~åíáÄçÇáÉë=áåÇìÅÉÇ=Äó=íÜÉ=áåÑÉÅíáçåI=ÅêçëëJêÉ~ÅíáåÖ=ïáíÜ=~åíáÖÉåë=çÑíÜÉ=ãóÉäáå=ëÜÉ~íÜK=_çíÜ=ãÉÅÜ~åáëãë=äÉ~Ç=íç=~ÅìíÉ=ÇÉãóÉäáå~íáçå=ïáíÜ=áíëÅçåëÉèìÉåÅÉ= çÑ= áãé~áêÉÇ= ÑìåÅíáçå= çÑ= ~ñçåëK= ^ÅìíÉ= ÇÉãóÉäáå~íáçå= çÑÖêçìéë= çÑ= åÉêîÉ= ÑáÄêÉë= Å~å= áåÇìÅÉ= ÅçåÇìÅíáçå= ÄäçÅâI= ~åÇ= é~ê~äóëáë= çÑÅçêêÉëéçåÇáåÖ= íÉêêáíçêáÉëK= aÉãóÉäáå~íáçå= ~åÇ= ÅçåÇìÅíáçå= ÄäçÅâ= ~êÉ= íÜÉéÜóëáçäçÖáÅ~ä=Ä~ëÉë=çÑ=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáíë=áå=d_pK=oÉÅçîÉêó=Ñêçã=d_pé~ê~äóëáë= Å~å= ÄÉ= êÉä~íáîÉäó= ê~éáÇI= í~âáåÖ= ~= ÑÉï= Ç~óë= íç= ~= ÑÉï= ïÉÉâëI~äíÜçìÖÜ= áå= ëçãÉ= Å~ëÉë= áí= ã~ó= ÉñíÉåÇ= çîÉê= ëÉîÉê~ä= ãçåíÜëK= qÜÉ


42


ÇÉãóÉäáå~íáåÖ=éêçÅÉëëI=ÜçïÉîÉêI=áë=ÅÜ~ê~ÅíÉêáëíáÅ~ääó=ëÉäÑJäáãáíáåÖ=áå=d_pI~åÇ=êÉãóÉäáå~íáçå=áë=~=ê~éáÇ=éêçÅÉëë=ïáíÜ=ëìÄëÉèìÉåí=êÉÅçîÉêó=çÑ=åçêã~äÅçåÇìÅíáçå= ~åÇ= ëìééêÉëëáçå= çÑ= ÅçåÇìÅíáçå= ÄäçÅâ= ~åÇ= é~ê~äóëáëK= páåÅÉ~ñçå~ä=Åçåíáåìáíó=~åÇ=ÑìåÅíáçå=~êÉ=éêÉëÉêîÉÇ=êÉÅçîÉêó=Å~å=ÄÉ=ÅçãéäÉíÉK=fåëçãÉ=Å~ëÉëI=ÜçïÉîÉêI=íÜÉêÉ=áë=Äóëí~åÇÉê=~ñçå~ä=Ç~ã~ÖÉ=ïÜáÅÜ=ã~ó=äÉ~Çíç=~=ãçêÉ=éêçíê~ÅíÉÇ=ÅçìêëÉ=~åÇ=ëÉèìÉä~ÉK

få= ëçãÉ= Å~ëÉë= ~ñçå~ä= äÉëáçåë= éêÉÇçãáå~íÉI= ~ë= áå= íÜÉ= ëçJÅ~ääÉÇ= ~ÅìíÉãçíçê=~ñçå~ä=åÉìêçé~íÜó=E^j^kF=çê=~ÅìíÉ=ãçíçê=~åÇ=ëÉåëçêó=åÉìêçé~íÜóE^jp^kFI= ïÜáÅÜ= Ü~îÉ= íÜÉ= ë~ãÉ= ÅçìêëÉ= ~ë= d_p= Äìí= ïáíÜ= ~= éççêÉêçìíÅçãÉK=páåÅÉ=ÇÉãóÉäáå~íáçå=áë=åçí=~=ÑÉ~íìêÉ=çÑ=íÜáë=Ñçêã=çÑ=åÉìêçé~íÜóIÉäÉÅíêçéÜóëáçäçÖáÅ~ä= áåîÉëíáÖ~íáçåë=ïáää=ëÜçï=çåäó=~ñçå~ä=Ç~ã~ÖÉ=ïáíÜçìíåÉêîÉ= ÅçåÇìÅíáçå= ëäçïáåÖ= çê= ÅçåÇìÅíáçå= ÄäçÅâK= fåíÉêãÉÇá~íÉ= Ñçêãë= ~êÉÅçããçåI=ÜçïÉîÉêK=qÜÉ=~ñçå~ä=Ñçêãë=~êÉ=ãçêÉ=Åçããçå=áå=ëçãÉ=ÅçìåíêáÉëIÉëéÉÅá~ääó= áå= `Üáå~I= ÑçääçïáåÖ= Ö~ëíêçáåíÉëíáå~ä= áåÑÉÅíáçå= ïáíÜ`~ãéóäçÄ~ÅíÉê= àÉàìåáK= qÜáë= Ä~ÅíÉêá~ä= áåÑÉÅíáçå= áë= Åçããçå= Äìí= ~Åíì~ääóáåÇìÅÉë=d_p=áå=äÉëë=íÜ~å=MKNB=çÑ=áåÑÉÅíÉÇ=éÉçéäÉK=eçëí=Ñ~Åíçêë=~êÉ=äáâÉäóíç=áåÑäìÉåÅÉ=íÜÉ=ëìëÅÉéíáÄáäáíó=íç=d_pK

Clinical aspects

páåÅÉ=íÜÉ=îáêíì~ä=Çáë~ééÉ~ê~åÅÉ=çÑ=~ÅìíÉ=éçäáçãóÉäáíáëI=d_p=áë=íÜÉ=ãçëíÅçããçå= ~ÅìíÉ= ~êÉÑäÉñáÅ= èì~Çêáé~êÉëáëK= qóéáÅ~ääóI= ~Äçìí= íïç= íÜáêÇë= çÑé~íáÉåíë=ÖáîÉ=~=Üáëíçêó=çÑ=êÉÅÉåí=~ÅìíÉ= áåÑÉÅíáçåI=~= ÑäìJäáâÉ=ëóåÇêçãÉ=çê=~ãáäÇ= êÉëéáê~íçêó= ëóåÇêçãÉX= çÅÅ~ëáçå~ääó= íÜÉ= çåëÉí= çÑ= d_p= ÑçääçïëëÉêçÅçåîÉêëáçå= íç=efs= áåÑÉÅíáçåK=mçëíJëìêÖáÅ~ä=d_pI=ïÜáÅÜ=~ÅÅçìåíë= Ñçê~ééêçñáã~íÉäó=RB=çÑ=d_p=é~íáÉåíëI=çÅÅìêë=OJP=ïÉÉâë=~ÑíÉê= íÜÉ=ëìêÖáÅ~äéêçÅÉÇìêÉK=

aáëí~ä=é~ê~ÉëíÜÉëá~ëI=åìãÄåÉëë=~åÇ=é~áå=ìëì~ääó=ÜÉê~äÇ=íÜÉ=ëí~êí=çÑ=íÜÉáääåÉëëK=tÉ~âåÉëë=Å~å=ëí~êí=Éèì~ääó=áå=íÜÉ=ìééÉê=~åÇ=äçïÉê=äáãÄëI=çê=~ÑÑÉÅííÜÉ= äçïÉê= äáãÄë= çê= íÜÉ= ìééÉê= äáãÄë= Ñáêëí= EÜ ëÉÉ= Å~ëÉ= ëíìÇó= çîÉêäÉ~ÑFKfå~ìÖìê~ä=ëÉåëçêó=ëóãéíçãë=~åÇ=é~ê~ÉëíÜÉëá~ë=~êÉ=~ëëçÅá~íÉÇ=ïáíÜ=é~áåáå=RMB=çÑ=é~íáÉåíëI=Äìí=~ë=ã~åó=~ë=VMB=çÑ=é~íáÉåíë=ÉîÉåíì~ääó=Åçãéä~áå=çÑé~áå=RK=tÉ~âåÉëë=ã~ó=~ÑÑÉÅí=íÜÉ=åÉÅâ=~åÇ=éÜ~êóåÖÉ~ä=ãìëÅäÉëK=_áä~íÉê~äëóããÉíêáÅ~ä= Ñ~Åá~ä= ïÉ~âåÉëë= Ô= Ñ~Åá~ä= ÇáéäÉÖá~= Ô= çÅÅìêë= áå= ãçêÉ= íÜ~åRMB= çÑ= Å~ëÉëK= m~êíá~ä= çê= ÅçãéäÉíÉ= é~ê~äóëáë= çÑ= çÅìäçãçíçê= ãìëÅäÉë

Chapter 4 Guillain-Barré syndrome

43


EçéÜíÜ~äãçéäÉÖá~F=çÅÅìêë= áå=PJRB=çÑ=é~íáÉåíëK=qÜÉ=ïÉ~âåÉëë= êÉ~ÅÜÉë=~ã~ñáãìã= ëí~íÉ= ~í= OJQ= ïÉÉâë= ~ÑíÉê= ëóãéíçã= çåëÉíI= ïáíÜ= éêçÖêÉëëáîÉêÉÅçîÉêó=çîÉê=ïÉÉâë=íç=ãçåíÜëK=

m~íáÉåíë= ïáíÜ= d_p= áåáíá~ääó= ÉñÜáÄáí= ÑÉï= çÄàÉÅíáîÉ= ëáÖåë= ÄÉëáÇÉëïÉ~âåÉëëK= pÉåëçêó= ëáÖåë= ~êÉ= ìëì~ääó= äáãáíÉÇ= íç= ÖäçîÉ= ~åÇ= ëíçÅâáåÖÜóéç~ÉëíÜÉëá~=íç= äáÖÜí=íçìÅÜK=mçëáíáçå=ëÉåëÉ=~åÇ=~=ÇÉÅêÉ~ëÉ=çÑ=îáÄê~íçêóëÉåë~íáçå=~êÉ=çÑíÉå=áãé~áêÉÇ=~ÑíÉê=~=ÑÉï=Ç~óëK=bñ~ãáå~íáçå=ëÜçïë=Ñä~ÅÅáÇé~ê~é~êÉëáë=çê=é~ê~éäÉÖá~I=ïáíÜ=~=äçëë=çÑ=íÉåÇçå=êÉÑäÉñÉë=~åÇ=ãáäÇ=ëÉåëçêóÅÜ~åÖÉë= áå= ãçëí= Å~ëÉëK= aóë~ìíçåçãá~= çÅÅìêë= áå= NRB= çÑ= é~íáÉåíëIáåÅäìÇáåÖ= Å~êÇá~Å= ~êêÜóíÜãá~I= ÜóéÉêíÉåëáçå= çê= ÜóéçíÉåëáçåI= áäÉìë= ~åÇìêáå~êó=êÉíÉåíáçå=SK=få=ëçãÉ=áåÇáîáÇì~äëI=êÉëéáê~íçêó=Ñ~áäìêÉ=ã~ó=çÅÅìê=É~êäóIïÜáäÉ= çíÜÉêë= ã~ó= Ü~îÉ= åç= êÉëéáê~íçêó= áåîçäîÉãÉåíI= ëç= íÜÉ= ÉñíÉåí= ~åÇÇáëíêáÄìíáçå=çÑ=íÜÉ=ëóãéíçãë=î~êó=ÖêÉ~íäó=~ãçåÖ=áåÇáîáÇì~äë=ïáíÜ=d_pK


44

Guillain-Barré syndrome

Ian is a 27-year-old painter and decorator who was admitted to the neurology

service at 22:00 having gone up to casualty with severe pain in his legs which had

developed over the course of the day. He had become unsteady on his legs. He was

admitted overnight and the junior staff could not decide what was wrong with him,

but having identified weak legs with normal reflexes had organised an MRI scan of

the spine for the following morning. On the morning ward-round the consultant

found no abnormality in the arms, but his legs were weak generally in the 4/5

range. There was a loss of vibration sensation in the toes and a minor impairment

of proprioception. His ankle jerks were absent with flexor plantar responses. He

was in significant pain. A diagnosis of GBS was made and he was started on

intravenous immunoglobulin (IVIG) at a dose of 0.4g/kg daily for 5 days. The

subsequent MRI of the complete spine was normal. His CSF was normal apart from

a mildly elevated protein level, and the peripheral electrophysiology performed

within 2 days of presentation was normal.

The pain settled over the next 4 days and his walking gradually improved. He was

discharged from hospital within a week of arrival and was able to return to work 6

weeks after his initial presentation, though he struggled with fatigue.


få= íÜÉ= Åä~ëëáÅ~ä= Ñçêã= çÑ= d_pI= äÉëáçåë= çÑ= êççíë= ~åÇ= åÉêîÉë= ~êÉÅÜ~ê~ÅíÉêáëíáÅ~ääó= ÇÉãóÉäáå~íáåÖ= ïÜáäÉ= íÜÉ= ìåÇÉêäóáåÖ= ~ñçåë= ~êÉ= êÉä~íáîÉäóëé~êÉÇK= få= íÜÉ= ~ñçå~ä= î~êá~åíI= íÜÉ= ~ñçåë= ~êÉ= éêÉÇçãáå~åíäó= áåîçäîÉÇK= qÜÉÅçìêëÉ=ëÉÉãë=ãçêÉ=ê~éáÇ=~åÇ=ëÉîÉêÉ=áå=íÜÉ=~ñçå~ä=Ñçêã=çÑ=d_pI=ïáíÜ=ãçêÉÑêÉèìÉåí= ~åÇ= ëÉîÉêÉ= êÉëéáê~íçêó= ~åÇ= Åê~åá~ä= åÉêîÉ= áåîçäîÉãÉåíKaóë~ìíçåçãá~= áë= äÉëë= Åçããçå= áå= íÜÉ= ~ñçå~ä= é~ííÉêåK= mêÉÅÉÇáåÖ`~ãéóäçÄ~ÅíÉê=àÉàìåá=áåÑÉÅíáçå=ëÉÉãë=ãçêÉ=Åçããçå=áå=íÜÉ=~ñçå~ä=î~êá~åíëíÜ~å= áå=Åä~ëëáÅ~ä=d_pK=qÜÉ=^j^k= Ñçêã= áë=~=éìêÉ=ãçíçê=ëóåÇêçãÉI=ïÜáäÉ^jp^k=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=ÄçíÜ=ãçíçê=~åÇ=ëÉåëçêó=ÇÉÑáÅáíëK=qÜÉ=ä~ííÉê= áëíÜçìÖÜí=íç=ÄÉ=íÜÉ=ãçêÉ=~Çî~åÅÉÇ=çê=ëÉîÉêÉ=Ñçêã=çÑ=~ñçå~ä=ëìÄíóéÉëK

The ophthalmoplegia, ataxia and areflexia syndrome orMiller-Fisher syndrome

jáääÉêJcáëÜÉê= ëóåÇêçãÉ= EjcpF= áë= ~= Åê~åá~ä= åÉêîÉ= î~êá~åí= çÑ= d_pIÅÜ~ê~ÅíÉêáëÉÇ=Äó=íÜÉ=éêÉëÉåÅÉ=çÑ=çéÜíÜ~äãçéäÉÖá~I=~í~ñá~=~åÇ=~êÉÑäÉñá~=TKqÜÉêÉ= áë=åç=ãçíçê= áåîçäîÉãÉåí=Äìí=ÅçåëáÇÉê~ÄäÉ= áãé~áêãÉåí=çÑ=Ä~ä~åÅÉÇìÉ= íç= áãé~áêÉÇ= éêçéêáçÅÉéíáçåK= léÜíÜ~äãçéäÉÖá~= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äóé~ê~äóëáë=çÑ=çÅìä~ê=ãìëÅäÉëK=qÜÉ=äçëë=çÑ=íÉåÇçå=êÉÑäÉñÉë=áë=êÉä~íÉÇ=íç=íÜÉáåîçäîÉãÉåí= çÑ= ä~êÖÉ= ëÉåëçêó= ãóÉäáå~íÉÇ= ÑáÄêÉëK= jcp= áë= ÖÉåÉê~ääóêÉÖ~êÇÉÇ=~ë=~=ëÉäÑJäáãáíáåÖ=ÇáëçêÇÉêX=ÜçïÉîÉêI=ëçãÉ=é~íáÉåíë=ã~ó=êÉèìáêÉêÉëéáê~íçêó=~ëëáëí~åÅÉK=`çãéäÉíÉ=êÉÅçîÉêó=áë=íÜÉ=êìäÉ=áå=jcpK

Diagnosis

qÜÉ=Çá~Öåçëáë=çÑ=d_p=~åÇ=î~êá~åíë=êÉëíë=çå= íÜÉ=ÅäáåáÅ~ä=é~ííÉêå=~åÇÉäÉÅíêçãóçÖê~éÜó= EbjdF=~åÇ=`pcK=`Ü~ê~ÅíÉêáëíáÅ~ääóI= íÜÉ=åÉìêçäçÖáÅ~äÇÉÑáÅáí= áë=åçí= ÚäÉåÖíÜJÇÉéÉåÇÉåíÛX= íÜ~í=ãÉ~åë= íÜ~í= áí=ÇçÉë=åçí=~ÑÑÉÅí= íÜÉäçåÖÉëí=ÑáÄêÉë=~åÇ=Çáëí~ä= äáãÄë=ÑáêëíK=^äëç= áí= áë=éêÉÇçãáå~åíäó=ãçíçê=ïáíÜäáííäÉ=çê=åç=ëÉåëçêó= äçëëK=^=ÇÉÅêÉ~ëÉ=áå= äáÖÜí=íçìÅÜ=ëÉåë~íáçåI=~äíÉê~íáçåçÑ=éêçéêáçÅÉéíáçå=~åÇ=äçëë=çÑ=íÉåÇçå=êÉÑäÉñÉë=~ää=éçáåí=íç=íÜÉ=áåîçäîÉãÉåíçÑ=ä~êÖÉê=ãóÉäáå~íÉÇ=ÑáÄêÉëK=

`pc= Éñ~ãáå~íáçå= ~ÑíÉê= äìãÄ~ê= éìåÅíìêÉ= áë= ~= âÉó= ÅçåíêáÄìíáçå= íçÇá~ÖåçëáëK= `pc= éêçíÉáå= ÅçåÅÉåíê~íáçå= áë= ê~áëÉÇ= áå= UMB= çÑ= é~íáÉåíëI


45


ëçãÉíáãÉë= ëÉîÉê~ä= íáãÉë= íÜÉ= ìééÉê= äáãáí= çÑ= åçêã~ä= î~äìÉëI= ïÜáäÉ= íÜÉãçåçåìÅäÉ~ê=ÅÉää=Åçìåí=áë=ìëì~ääó=åçêã~ä=~ë=ÇÉëÅêáÄÉÇ=Äó=dìáää~áå=Éí=~ä NKqÜÉ=`pc=éêçíÉáå=äÉîÉä=ã~ó=êÉã~áå=åçêã~ä=áå=~=ëã~ää=éêçéçêíáçå=çÑ=é~íáÉåíë~åÇ=ã~ó=ÄÉ=åçêã~ä=É~êäó=çå=áå=íÜÉ=áääåÉëëK

få= íÜÉ= É~êäó= ëí~ÖÉëI= é~êíáÅìä~êäó= ÄÉÑçêÉ= ~êÉÑäÉñá~= áë= éêÉëÉåíI= áí= ã~ó= ÄÉåÉÅÉëë~êó=íç=ìåÇÉêí~âÉ=ã~ÖåÉíáÅ=êÉëçå~åÅÉ=áã~ÖáåÖ=EjofF=çÑ=íÜÉ=ëéáå~äÅçêÇ= íç= ÉñÅäìÇÉ= ~= íê~åëîÉêëÉ= ãóÉäáíáë= çê= ÅçêÇ= ÅçãéêÉëëáçå= ïÜáÅÜ= Å~åãáãáÅ=íÜÉ=éêÉëÉåí~íáçå=çÑ=d_pK

qÜÉ=Åä~ëëáÅ~ä=ÑáåÇáåÖë=çÑ=åÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉë=áåÅäìÇÉ=íÜÉ=éêÉëÉåÅÉçÑ=~=é~êíá~ä=ãçíçê=ÅçåÇìÅíáçå=ÄäçÅâI=~å=~Äåçêã~ä= íÉãéçê~ä=ÇáëéÉêëáçå=çÑãçíçê= êÉëéçåëÉëI= éêçäçåÖÉÇ= Çáëí~ä= ãçíçê= ~åÇ= cJï~îÉ= ä~íÉåÅáÉëI= ~åÇêÉÇìÅíáçåë= áå= ã~ñáãìã= ãçíçê= ÅçåÇìÅíáçå= îÉäçÅáíóK= kÉêîÉ= ÅçåÇìÅíáçåëíìÇáÉë= ã~ó= ÄÉ= åçêã~ä= áåáíá~ääóI= ~åÇ= ëçãÉíáãÉë= êÉã~áå= åçêã~äëìÄëÉèìÉåíäóI=ÄÉÅ~ìëÉ=íÜÉ=íÉêêáíçêáÉë=ÉñéäçêÉÇ=Äó=ÅçåÇìÅíáçå=ëíìÇáÉë=~êÉåçí= ~ÑÑÉÅíÉÇ= Äó= íÜÉ= ÇáëÉ~ëÉK= få= íÉêêáíçêáÉë= ïáíÜ= ãçíçê= ÇÉÑáÅáíI= ÜçïÉîÉêIåÉÉÇäÉ= bjd= ïáää= ÇÉíÉÅí= åçåJëéÉÅáÑáÅ= ÇÉåÉêî~íáçåK= oÉéÉ~íÉÇÉäÉÅíêçéÜóëáçäçÖáÅ~ä=íÉëíë=~åÇ=`pc=Éñ~ãáå~íáçå=ÇìêáåÖ=íÜÉ=ÅçìêëÉ=çÑ=íÜÉé~ê~äóíáÅ=çê=êÉÅçîÉêó=éÜ~ëÉ=çÑ= íÜÉ=ÇáëÉ~ëÉ=~êÉ=åçí=åÉÅÉëë~êóI=ÉñÅÉéí= ÑçêêÉëÉ~êÅÜ=éìêéçëÉëK

Pathology

qÜÉ=é~íÜçäçÖó=çÑ=d_p=Ü~ë=ÄÉÉå=ÇÉëÅêáÄÉÇ=áå=íÜÉ=ëÉãáå~ä=ÑáåÇáåÖë=Äó^ëÄìêó= Éí= ~ä UK= iÉëáçåë= éêÉÇçãáå~íÉ= çå= ëéáå~ä= êççíëK= qÜÉ= ~ëëçÅá~íÉÇáåÑä~ãã~íçêó= áåÑáäíê~íÉë= Åçåëáëí= çÑ= äóãéÜçÅóíÉë= ~åÇ= ã~ÅêçéÜ~ÖÉë= ïáíÜ= ~êÉëìäíáåÖ=ÇÉãóÉäáå~íáçåK=j~ÅêçéÜ~ÖÉë=áåÑáäíê~íÉ=íÜÉ=ãóÉäáå=ëÜÉ~íÜ=~åÇ=ëíêáéãóÉäáå= ä~óÉêë= íç= ÇÉãóÉäáå~íÉ= íÜÉ= ìåÇÉêäóáåÖ= ~ñçåK= iÉëáçåë= çÑíÉåéêÉÇçãáå~íÉ= çå= ~åíÉêáçê= êççíëK= jçëí= ~ñçåë= êÉã~áå= áåí~Åí= áå= íÜÉÇÉãóÉäáå~íÉÇ= ~êÉ~K= aìÉ= íç= íÜÉ= áåíÉåëáíó= çÑ= íÜÉ= áåÑä~ãã~íçêó= éêçÅÉëëIÜçïÉîÉêI=çê=íç=ëéÉÅáÑáÅ=~åíáÄçÇáÉë=íç=~ñçå~ä=ÅçãéçåÉåíëI=íÜÉ=~ñçå=Å~å=ÄÉÇ~ã~ÖÉÇ= ïáíÜ= ëìÄëÉèìÉåí= ~ñçå~ä= ÇÉÖÉåÉê~íáçåI= ïÜáÅÜ= éêÉÅäìÇÉë= ê~éáÇêÉãóÉäáå~íáçå= ~åÇ= ~ääÉîá~íáçå= çÑ= íÜÉ= ÅçåÇìÅíáçå= ÄäçÅâ= áåÇìÅÉÇ= Äó= ~ÅìíÉÇÉãóÉäáå~íáçå=EcáÖìêÉë=NJQF=VK


46



47

Figure 1. This schema shows the distribution of

demyelinating lesions in GBS. Demyelination

predominates in proximal parts of the peripheral nervous

system. They are often patchy, especially in nerve trunks.


få= íÜÉ=~ñçå~ä=î~êá~åíë=çÑ=d_pI= íÜÉ= äÉëáçåë=éêÉÇçãáå~íÉ=çå=~ñçåë=~åÇçÅÅ~ëáçå~ääó= çå= åÉìêçåë= EcáÖìêÉ= QF= ïÜáÅÜ= ~ÅÅçìåíë= Ñçê= ÅäáåáÅ~ä= ~åÇÉäÉÅíêçéÜóëáçäçÖáÅ~ä=ÑÉ~íìêÉë=~åÇ=Ä~Çäó=áãé~áêë=êÉÅçîÉêóK

Prognosis and evolution

få= êÉä~íáçå= íç= íÜÉ= ëÉäÑJäáãáíáåÖ=ìåÇÉêäóáåÖ=é~íÜçäçÖáÅ~ä=éêçÅÉëëI= å~ãÉäó~ÅìíÉ= ëÉÖãÉåí~ä= ÇÉãóÉäáå~íáçåI= íÜÉ= çìíÅçãÉ= çÑ= d_p= áë= ëéçåí~åÉçìëäóÖççÇ= áå=ãçëí=Å~ëÉëI=ïáíÜáå=~= ÑÉï=ïÉÉâëI=~ë=ÉãéÜ~ëáëÉÇ= áå= íÜÉ=çêáÖáå~äÇÉëÅêáéíáçå=NK=oÉãóÉäáå~íáçå=çÑ=~ÑÑÉÅíÉÇ=éçêíáçåë=çÑ=êççíë=~åÇ=åÉêîÉë=áë=~ê~éáÇ=éÜÉåçãÉåçåI=ïÜáÅÜ=èìáÅâäó=~ääÉîá~íÉë=ÅçåÇìÅíáçå=ÄäçÅâ=éÉêãáííáåÖ=~êÉíìêå=çÑ=åçêã~ä=ÑìåÅíáçå=~åÇ=ÅäáåáÅ~ä=êÉÅçîÉêó=ïáíÜáå=~=ÑÉï=ïÉÉâë=áå=ãçëíÅ~ëÉëK=^ÑíÉê=íÜÉ=áåáíá~ä=éÜ~ëÉ=çÑ=éêçÖêÉëëáîÉ=é~ê~äóëáëI=íÜÉ=é~íáÉåí=ÉåíÉêë=~


48

Figure 2. Consecutive segments of a nerve fibre isolated from a nerve biopsy specimen

of a patient with Guillain-Barré syndrome to show segmental demyelination. The myelin

sheath is stained in black by post-fixation in osmium tetroxide. The myelin sheath has

normal thickness from top left to the node of Ranvier a and then from b to c and d to e.

Between f and g the fibre is completely demyelinated while the other segments are

thinly remyelinated (arrows).



49

Figure 3. Electron micrograph of a nerve biopsy specimen

from a patient with GBS to show a demyelinated axon (Ax)

surrounded by macrophages (M) which are removing myelin

debris from the demyelinated axon. S = Schwann cell. (Uranyl

acetate and lead citrate staining.)

Figure 4. This schema illustrates the sites of axonal involvement in GBS. The large

arrow points to a demyelinated fibre in the ventral (anterior) root, which contains

motor fibres. Arrows 1 and 2 point to axonal lesions of nerve fibres in the anterior

and posterior roots, respectively. Arrow 3 points to a lesion of the cell body of

sensory neurons in a dorsal root ganglion.

péáå~ä=ÅçêÇ


éä~íÉ~ì= éÜ~ëÉ= çÑ= î~êóáåÖ= Çìê~íáçåI= ïÜáÅÜ= ê~åÖÉë= Ñêçã= Ç~óë= íç= ëÉîÉê~äïÉÉâë=çê=ãçåíÜëK=qÜáë=éÜ~ëÉ=áë=ÑçääçïÉÇ=Äó=~=êÉÅçîÉêó=éÜ~ëÉ=çÑ=î~êóáåÖÇìê~íáçåK=

d_p=áë=ÖÉåÉê~ääó=êÉÖ~êÇÉÇ=~ë=~=ãçåçéÜ~ëáÅ=áääåÉëë=Äìí=~Äçìí=NMB=çÑé~íáÉåíë=ëÜçï=~=êÉJÇÉíÉêáçê~íáçå=çê=êÉä~éëÉK=få=bìêçéÉI=~Äçìí=çåÉ=íÜáêÇ=çÑé~íáÉåíë=ïáíÜ=d_p=êÉã~áå=~ÄäÉ=íç=ï~äâ=EãáäÇäó=~ÑÑÉÅíÉÇ=é~íáÉåíëF=ÇìêáåÖ=íÜÉéä~íÉ~ì=éÜ~ëÉK=få=é~íáÉåíë=ïáíÜ=d_p=ïÜç=~êÉ=~ÇãáííÉÇ=íç=Üçëéáí~ä=~åÇ=~êÉìå~ÄäÉ= íç= ï~äâ= EëÉîÉêÉäó= ~ÑÑÉÅíÉÇ= é~íáÉåíëFI= ~Äçìí= ORB= åÉÉÇ= ~êíáÑáÅá~äîÉåíáä~íáçåI= ã~áåäó= ÄÉÅ~ìëÉ= çÑ= êÉëéáê~íçêó= ãìëÅäÉ= ïÉ~âåÉëëK= qïÉåíóéÉêÅÉåí=çÑ=é~íáÉåíë=êÉèìáêáåÖ=ãÉÅÜ~åáÅ~ä=îÉåíáä~íáçå=ÇáÉI=~åÇ=íÜÉ=êÉÅçîÉêóçÑ=ãçëí=ëìêîáîçêë=áë=~=éêçäçåÖÉÇ=éêçÅÉëëK=líÜÉêïáëÉI=áå=ëéáíÉ=çÑ=íÜÉ=ÉÑÑÉÅíçÑ=áåíê~îÉåçìë=áããìåçÖäçÄìäáå=çê=éä~ëã~=ÉñÅÜ~åÖÉ=íêÉ~íãÉåí=EëÉÉ=ÄÉäçïFI~Äçìí= OMB= çÑ= ëÉîÉêÉäó= ~ÑÑÉÅíÉÇ= é~íáÉåíë= êÉã~áå= ìå~ÄäÉ= íç= ï~äâ= ~ÑíÉê= SãçåíÜë= NMK= jçêÉçîÉêI= ã~åó= é~íáÉåíë= êÉã~áå= Çáë~ÄäÉÇ= çê= Ü~îÉ= ëÉîÉêÉÑ~íáÖìÉK= fí= áë= åçíÉïçêíÜó= íÜ~í= d_p= Ü~ë= ~= ëáÖåáÑáÅ~åí= åÉÖ~íáîÉ= áãé~Åí= çåëçÅá~ä=äáÑÉ=~åÇ=Ç~áäó=~ÅíáîáíáÉëI=ÉîÉå=PJS=óÉ~êë=~ÑíÉê=çåëÉíK=lÑ=íÜÉ=íçí~äáíó=çÑé~íáÉåíë= ïáíÜ= d_pI= PJNMB= ïáää= ÇáÉI= ~åÇ= áå= ëçãÉ= çÑ= íÜÉëÉ= é~íáÉåíë= íÜÉÅ~ìëÉ= áë= äáâÉäó= íç= ÄÉ= EëìÇÇÉåF= ~ìíçåçãáÅ= Ñ~áäìêÉ= ïáíÜ= Å~êÇáçî~ëÅìä~êáåëí~ÄáäáíóK= ^ÖÉ= ÖêÉ~íÉê= íÜ~å= RM= ~åÇ= Çáë~Äáäáíó= ÇìÉ= íç= éêçãáåÉåí= ~ñçå~ääÉëáçåë=~êÉ=íÜÉ=ã~áå=áåÇáÅ~íçêë=Ñçê=~=éççê=éêçÖåçëáë=ïáíÜ=êÉÖ~êÇ=íç=ï~äâáåÖ~í=S=ãçåíÜëK

Treatment

m~íáÉåíë=ïáíÜ=d_p=åÉÉÇ=áåíÉÖê~íÉÇ=ãìäíáÇáëÅáéäáå~êó=Å~êÉ=íç=éêÉîÉåí=~åÇã~å~ÖÉ=éçíÉåíá~ääó=Ñ~í~ä=ÅçãéäáÅ~íáçåëK=m~íáÉåíë=åÉÉÇ=Å~êÉÑìä=ãçåáíçêáåÖ=çÑêÉëéáê~íçêó= ÑìåÅíáçåI= áåÅäìÇáåÖ=îáí~ä=Å~é~ÅáíóI= êÉëéáê~íáçå= ÑêÉèìÉåÅóI=ÄäççÇÖ~ëÉëI= ~åÇ= éçëëáÄäÉ= ~ìíçåçãáÅ= ÇóëÑìåÅíáçåI= ëìÅÜ= ~ë= ÜÉ~êí= ê~íÉI~êêÜóíÜãá~I=~åÇ=ÄäççÇ=éêÉëëìêÉK=fåÑÉÅíáçåë=~åÇ=ÇÉÉé=îÉáå=íÜêçãÄçëáë=åÉÉÇíç=ÄÉ=éêÉîÉåíÉÇK=qÜÉ=êÉÅçÖåáíáçå=çÑ=~ìíçåçãáÅ=ÇóëÑìåÅíáçå=EáäÉìëI=éìéáää~êóäáÖÜí=êÉÑäÉñF=áë=êÉèìáêÉÇ=~äçåÖëáÇÉ=íÜÉ=ã~å~ÖÉãÉåí=çÑ=é~áåI=éÜóëáçíÜÉê~éóIêÉÜ~Äáäáí~íáçå=~åÇ=éëóÅÜçäçÖáÅ~ä=ëìééçêí=Ñçê=ëÉîÉêÉäó=~ÑÑÉÅíÉÇ=é~íáÉåíëK==

qÜÉ= éêÉëìãÉÇ= ~ìíçáããìåÉ= ãÉÅÜ~åáëã= çÑ= d_p= Ü~ë= äÉÇ= íçáããìåçãçÇìä~íáåÖ= íêÉ~íãÉåíëI= ïÜáÅÜ= ëÉÉã= íç= ëÜçêíÉå= êÉÅçîÉêó= íáãÉI


50


ïáíÜçìí= éêÉîÉåíáåÖ= êÉëáÇì~ä= ÇÉÑáÅáíë= áå= ~ää= é~íáÉåíë= NNK= få= ãáäÇäó= ~ÑÑÉÅíÉÇé~íáÉåíë= íÜÉêÉ= Ü~îÉ= åçí= ÄÉÉå= ~åó= éä~ÅÉÄç= ÅçåíêçääÉÇ= íêá~äëI= íç= Ç~íÉI~ëëÉëëáåÖ= íÜÉ= ÉÑÑÉÅí= çÑ= éä~ëã~= ÉñÅÜ~åÖÉ= EmbF= çê= áåíê~îÉåçìëáããìåçÖäçÄìäáå=EfsfdF=áå=íÜÉëÉ=é~íáÉåíë=ïáíÜ=d_pK=

qÜÉ=ä~íÉëí=êÉîáÉï=çå=íÜÉ=ìëÉ=çÑ=mb=áå=d_p=EOMMUF=ëí~íÉÇW=

√ áå=ãáäÇ=dìáää~áåJ_~êê¨=ëóåÇêçãÉI=íïç=ëÉëëáçåë=çÑ=éä~ëã~=ÉñÅÜ~åÖÉ~êÉ=ëáÖåáÑáÅ~åíäó=ëìéÉêáçê=íç=çåÉX

√ áå=ãçÇÉê~íÉ=d_pI=Ñçìê=ëÉëëáçåë=~êÉ=ëáÖåáÑáÅ~åíäó=ëìéÉêáçê=íç=íïçX√ áå=ëÉîÉêÉ=d_pI=ëáñ=ëÉëëáçåë=çÑ=éä~ëã~=ÉñÅÜ~åÖÉ=~êÉ=åçí=ëáÖåáÑáÅ~åíäó

ÄÉííÉê=íÜ~å=ÑçìêX√ éä~ëã~=ÉñÅÜ~åÖÉ= áë=ãçêÉ=ÄÉåÉÑáÅá~ä=ïÜÉå=ëí~êíÉÇ=ïáíÜáå=T=Ç~óë=çÑ

ÇáëÉ~ëÉ= çåëÉí= ê~íÜÉê= íÜ~å= ä~íÉêI= Äìí= ï~ë= ëíáää= ÄÉåÉÑáÅá~ä= áå= é~íáÉåíëíêÉ~íÉÇ=ìé=íç=PM=Ç~óë=~ÑíÉê=ÇáëÉ~ëÉ=çåëÉí=NOK

fåíê~îÉåçìë=áããìåçÖäçÄìäáå=áå=~=êÉÖáãÉå=çÑ=MKQÖLâÖ=ÄçÇó=ïÉáÖÜí=Ç~áäóÑçê=R=ÅçåëÉÅìíáîÉ=Ç~óë=Ü~ë=êÉéä~ÅÉÇ=mb=~ë=íÜÉ=éêÉÑÉêêÉÇ=íêÉ~íãÉåí=áå=ã~åóÅÉåíêÉëI= ã~áåäó= ÄÉÅ~ìëÉ= çÑ= áíë= ÖêÉ~íÉê= ÅçåîÉåáÉåÅÉ= ~åÇ= ~î~áä~ÄáäáíóK= qÜÉä~íÉëí=`çÅÜê~åÉ=êÉîáÉï=ëí~íÉÇ=ëáÖåáÑáÅ~åí=ÄÉåÉÑáíë=çÑ=fsfd=áå=NOW=

√ áãéêçîÉãÉåí=áå=Çáë~Äáäáíó=Öê~ÇÉI=Q=ïÉÉâë=~ÑíÉê=íêÉ~íãÉåíX√ ëÜçêíÉåáåÖ=íÜÉ=íáãÉ=íç=êÉÅçîÉêó=çÑ=ï~äâáåÖ=ìå~áÇÉÇX=~åÇ=√ ëÜçêíÉåáåÖ=íÜÉ=íáãÉ=íç=ÇáëÅçåíáåì~íáçå=çÑ=~êíáÑáÅá~ä=îÉåíáä~íáçåK=

dìáää~áå= _~êê¨= ëóåÇêçãÉ= áë= ~= ëÉäÑJäáãáíáåÖ= áåÑä~ãã~íçêóI= ~ìíçáããìåÉIÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜóK= d_p= Ü~ë= ~= ÖççÇ= çîÉê~ää= éêçÖåçëáëI= ïáíÜëéçåí~åÉçìë= ÅçãéäÉíÉ= êÉÅçîÉêó= áå= ãçëí= Å~ëÉëI= ïáíÜáå= ~= ÑÉï= ïÉÉâë= çêãçåíÜëK=c~í~ä=çìíÅçãÉ=áë=îÉêó=ê~êÉ=Äìí=ëÉèìÉä~É=íÜ~í=áåÅäìÇÉ=ãçíçê=ÇÉÑáÅáíIëÉåëçêó= äçëë= çê= é~áå= êÉã~áå= êÉä~íáîÉäó= ÅçããçåI= áå= ëéáíÉ= çÑ= ãçÇÉêåáããìåçíÜÉê~éóK


51


References

NK dìáää~áå= dI= _~êê¨= gI= píêçÜä= ^K= pìê= ìå= ëóåÇêçãÉ= ÇÉ= ê~ÇáÅìäçå¨îêáíÉ= ~îÉÅ

ÜóéÉê~äÄìãáåçëÉ=Çì=äáèìáÇÉ=Å¨éÜ~äçê~ÅÜáÇáÉå=ë~åë=ê¨~Åíáçå=ÅÉääìä~áêÉK=oÉã~êèìÉë=ëìê

äÉë=Å~ê~Åí≠êÉë=ÅäáåáèìÉë=Éí=Öê~éÜáèìÉë=ÇÉë=ê¨ÑäÉñÉë=íÉåÇáåÉìñK=_ìää=pçÅ=jÉÇ=eçé=m~êáë

NVNSX=OUW=NQSOJTMK

OK jÅhÜ~åå= djI= `çêåÄä~íÜ= aoI= dêáÑÑáå= gtI= Éí= ~äK= ^ÅìíÉ= ãçíçê= ~ñçå~ä= åÉìêçé~íÜóW= ~

ÑêÉèìÉåí=Å~ìëÉ=çÑ=~ÅìíÉ=Ñä~ÅÅáÇ=é~ê~äóëáë=áå=`Üáå~K=^åå=kÉìêçä=NVVPX=PPW=PPPJQOK

PK jÅdêçÖ~å= Î= j~ÇäÉ= dÌ= pÉ~ã~å= ebI= Éí= ~äK= qÜÉ= ÉéáÇÉãáçäçÖó= çÑ= dìáää~áåJ_~êê¨

ëóåÇêçãÉ= ïçêäÇïáÇÉK= ^= ëóëíÉã~íáÅ= äáíÉê~íìêÉ= êÉîáÉïK= kÉìêçÉéáÇÉãáçäçÖó OMMVX= POW

NRMJSPK=

QK cìàáãìê~=eK=qÜÉ=dìáää~áåJ_~êê¨=ëóåÇêçãÉK=e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=PUPJQMOK=

RK jçìäáå=abI=e~ÖÉå=kI=cÉ~ëÄó=qbI=Éí= ~äK=m~áå= áå=dìáää~áåJ_~êê¨= ëóåÇêçãÉK=kÉìêçäçÖó

NVVTX=QUW=POUJPNK

SK hìï~Ä~ê~= pK= dìáää~áåJ_~êê¨= ëóåÇêçãÉW= ÉéáÇÉãáçäçÖóI= é~íÜçéÜóëáçäçÖó= ~åÇ

ã~å~ÖÉãÉåíK=aêìÖë OMMQX=SQW=RVTJSNMK

TK cáëÜÉê= jK= ^å= ìåìëì~ä= î~êá~åí= çÑ= ~ÅìíÉ= áÇáçé~íÜáÅ= éçäóåÉìêáíáë= EëóåÇêçãÉ= çÑ

çéÜíÜ~äãçéäÉÖá~I=~í~ñá~=~åÇ=~êÉÑäÉñá~FK=k=båÖä=g=jÉÇ=NVRSX=ORRW=RTJSRK


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√ Pain and weakness occur at the onset of Guillain-Barré syndrome.

√ Clinical manifestations of Guillain-Barré syndrome are best explained by the

interruption of axonal function by conduction blocks.

√ The Miller-Fisher syndrome is associated with ophthalmoplegia, ataxia and

areflexia. It has a favourable outcome.

√ Axonal involvement delays recovery from GBS.

√ Patient management in the intensive care unit should include physiotherapy

and psychological support.

√ Plasma exchange and intravenous immunoglobulin treatment must be used

early in the course of GBS.

Ü Key points


UK ^ëÄìêó=^hI=^êå~ëçå=_dI=^Ç~ãë=oaK=qÜÉ=áåÑä~ãã~íçêó=äÉëáçå=áå=áÇáçé~íÜáÅ=éçäóåÉìêáíáëK

fíë=êçäÉ=áå=é~íÜçÖÉåÉëáëK=jÉÇáÅáåÉ=E_~äíáãçêÉF NVSVX=QUW=NTPJONRK

VK mêáåÉ~ë=gtK=m~íÜçäçÖó=çÑ=íÜÉ=dìáää~áåJ_~êê¨=ëóåÇêçãÉK=^åå=kÉìêçä=NVUNX=V=EpìééäFW=SJ

NVK

NMK eìÖÜÉë=oÎ=pï~å=^sI=o~éÜ~Éä=gÌ=Éí=~äK=fããìåçíÜÉê~éó=Ñçê=dìáää~áåJ_~êê¨=ëóåÇêçãÉW

~=ëóëíÉã~íáÅ=êÉîáÉïK=_ê~áå OMMTX=NPMW=OOQRJRTK=

NNK iÉÜã~åå= eÌ= e~êíìåÖ= emK= mä~ëã~= ÉñÅÜ~åÖÉ= ~åÇ= áåíê~îÉåçìë= áããìåçÖäçÄìäáåëW

ãÉÅÜ~åáëã=çÑ=~Åíáçå= áå= áããìåÉJãÉÇá~íÉÇ=åÉìêçé~íÜáÉëK=g=kÉìêçáããìåçä=OMNMX=OPNW

SNJVK

NOK= o~éÜ~Øä=gÌ=`ÜÉîêÉí=pI=eìÖÜÉë=oÎ=^åå~åÉ=aK=mä~ëã~=ÉñÅÜ~åÖÉ=Ñçê=dìáää~áåJ_~êê¨

ëóåÇêçãÉK=`çÅÜê~åÉ=a~í~Ä~ëÉ=póëí=oÉî OMNOX=TW=àMMNTVUK


53



54


55

Chronic inflammatory demyelinating

polyneuropathy

Chapter 5

Overview

Chronic inflammatory demylinating polyneuropathy (CIDP) has some similarities to

Guillain-Barré syndrome (GBS) but progresses over a longer period (more than a

month) and requires different treatment. The different patterns of CIDP are outlined,

including the relapsing and progressive forms, as well as a purely sensory neuropathy

at the onset. Other subjects covered are CIDP in diabetic patients, the association of

CIDP with monoclonal gammopathy of unknown significance, and the polyneuropathy,

organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS)

syndrome with plasmacytoma. Treatment with corticosteroids, intravenous

immunoglobulins, plasma exchange and immunosuppressive drugs are also discussed.

Introduction

`ÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜó= E`famF= áë= ~å~ÅèìáêÉÇ= éÉêáéÜÉê~ä= åÉìêçé~íÜóI= éêÉëìã~Ääó= çÑ= áããìåçäçÖáÅ~ä= çêáÖáåK= fíëÅäáåáÅ~ä=éêÉëÉåí~íáçå=~åÇ=ÅçìêëÉ=~êÉ=ÉñíêÉãÉäó=î~êá~ÄäÉK=aá~Öåçëáë=ëÜçìäÇåçí=ÄÉ=ãáëëÉÇ=ÄÉÅ~ìëÉ= áí= áë=çåÉ=çÑ= íÜÉ= ÑÉï=éÉêáéÜÉê~ä=åÉìêçé~íÜáÉë= íÜ~íêÉëéçåÇë=ïÉää=íç=íêÉ~íãÉåíK

`fam= áë= ÅÜ~ê~ÅíÉêáëÉÇ= ãçêéÜçäçÖáÅ~ääó= Äó= äçåÖëí~åÇáåÖ= ãìäíáÑçÅ~äÇÉãóÉäáå~íáçå=íÜ~í=éêÉÇçãáå~åíäó=~ÑÑÉÅíë=ëéáå~ä=êççíëI=ã~àçê=éäÉñìëÉë=~åÇéêçñáã~ä=åÉêîÉ=íêìåâëK=


Epidemiology

qÜÉ= ÅêìÇÉ= éêÉî~äÉåÅÉ= çÑ= `fam= áë= ~ééêçñáã~íÉäó= N= íç= O= éÉê= NMMIMMMéçéìä~íáçåK=qÜÉ=ãÉ~å=~ÖÉ=çÑ=çåëÉí=áë=~êçìåÇ=RM=óÉ~êëI=Äìí=áí=ã~ó=çÅÅìê=~í~åó=~ÖÉK=`fam=áë=íÜÉ=ëÉÅçåÇ=ãçëí=Åçããçå=Å~ìëÉ=çÑ=Çáë~ÄäáåÖ=åÉìêçé~íÜóáå= íÜÉ= ÉäÇÉêäóK= qÜÉ= ëíêáåÖÉåí= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= ÅêáíÉêá~= ÇÉÑáåÉÇ= Äó= íÜÉ^ãÉêáÅ~å=^Å~ÇÉãó=çÑ=kÉìêçäçÖó=^Ç=eçÅ=pìÄÅçããáííÉÉ=êÉëÉ~êÅÜ=ÅêáíÉêá~Ñçê=íÜÉ=Çá~Öåçëáë=çÑ=`fam=ìåÇÉêÉëíáã~íÉë=íÜÉ=êÉ~ä=áåÅáÇÉåÅÉ=çÑ=`fam=NK

Clinical manifestations

qÜÉ=Çá~Öåçëáë=çÑ=`fam=ã~ó=ÄÉ=ã~ÇÉ=ïÜÉå=~=é~íáÉåí=éêÉëÉåíë=ïáíÜ=~åçåJÑáÄêÉJäÉåÖíÜJÇÉéÉåÇÉåí=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜó=íÜ~í=éêçÖêÉëëÉëçîÉê=ãçêÉ= íÜ~å=~=ãçåíÜI=çê= ÉîçäîÉë=ÅÜêçåáÅ~ääó= çîÉê=ã~åó=ãçåíÜëK=qÜÉëìÄëÉèìÉåí=ÅçìêëÉ=Å~å=ÄÉ=éêçÖêÉëëáîÉI=çê=êÉä~éëáåÖ=~åÇ=êÉãáííáåÖI=çÑíÉåïáíÜ= ~= ëÉÅçåÇ~êó= éêçÖêÉëëáîÉ= ÅçìêëÉK= qÜÉ= ìåÇÉêäóáåÖ= ÇÉãóÉäáå~íáåÖéêçÅÉëë= åÉÉÇë= íç= ÄÉ= ÇÉãçåëíê~íÉÇ= Äó= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= çêçÅÅ~ëáçå~ääó=Äó=åÉêîÉ=Äáçéëó=OK=

Precipitating factors

qÜÉêÉ= áë=åç= áÇÉåíáÑáÉÇ=ÖÉåÉíáÅ~ääó=ÇÉíÉêãáåÉÇ=ëìëÅÉéíáÄáäáíó= íç=`famK=^Üáëíçêó= çÑ= ~å= áääåÉëëI= ãçëíäó= åçåJëéÉÅáÑáÅ= ìééÉê= êÉëéáê~íçêó= çêÖ~ëíêçáåíÉëíáå~ä= íê~Åí= áåÑÉÅíáçåI=çê=î~ÅÅáå~íáçå= áå= íÜÉ=éêÉÅÉÇáåÖ=S=ãçåíÜëï~ë=êÉéçêíÉÇ=áå=çåÉ=íÜáêÇ=çÑ=Å~ëÉëK=aáÑÑÉêÉåí=é~ííÉêåë=çÑ=`famI=êÉä~éëáåÖçê=éêçÖêÉëëáîÉI=Ü~îÉ=ÄÉÉå=çÄëÉêîÉÇ=~í=~ää=ëí~ÖÉë=çÑ=efs=áåÑÉÅíáçåK=

Neurological manifestations at onset

qÜÉ=ã~åáÑÉëí~íáçåë=~í=çåëÉí=~êÉ=ÉñíêÉãÉäó=î~êá~ÄäÉK= få= íÜÉ=ÖÉåÉê~äáëÉÇé~ííÉêå= íÜÉ= éêÉëÉåí~íáçå= áåÅäìÇÉë= åìãÄåÉëë= çÑ= íÜÉ= ìééÉê= ~åÇ= äçïÉêÉñíêÉãáíáÉëI=ëéçåí~åÉçìë=é~áå=~åÇ=ïÉ~âåÉëë=íÜ~í=éêçÖêÉëë=Öê~Çì~ääó=çîÉêëÉîÉê~ä=ïÉÉâë=EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çîÉêäÉ~ÑFK=få=ëçãÉ=Å~ëÉë=~=éêçÖêÉëëáîÉëÉåëçêó= ~í~ñá~= áë= íÜÉ= éêÉëÉåíáåÖ= ã~åáÑÉëí~íáçåI= ïÜáäÉ= áå= çíÜÉêë= ~éêÉÇçãáå~åíäó=çê=éìêÉäó=ãçíçê=ÇÉÑáÅáí=áë=çÄëÉêîÉÇ=~í=çåëÉíK=få=ãçëí=Å~ëÉë


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íÜÉ=ÇÉÑáÅáí=áë=êçìÖÜäó=ëóããÉíêáÅ~äI=ÄçíÜ=éêçñáã~ä=~åÇ=Çáëí~äK=få=çíÜÉê=Å~ëÉëÑçÅ~ä= çê= ãìäíáÑçÅ~ä= áåîçäîÉãÉåí= éêçÇìÅÉë= ~= ãìäíáÑçÅ~ä= ÇÉãóÉäáå~íáåÖåÉìêçé~íÜó= ïáíÜ= çê= ïáíÜçìí= ÅçåÇìÅíáçå= ÄäçÅâ= çå= ÉäÉÅíêçéÜóëáçäçÖáÅ~äíÉëíáåÖK=^í=çåëÉíI=íÜÉ=áåÅáÇÉåÅÉ=çÑ=ãçíçê=ÇÉÑáÅáí=î~êáÉë=Ñêçã=TUJVQB=çÑ=íÜÉÅ~ëÉë= ~ë= áääìëíê~íÉÇ= áå= íÜêÉÉ= ä~êÖÉ= ëíìÇáÉë= PJRK= få= jÅ`çãÄÉÛë= ëíìÇó= ~Öê~Çì~ä=çåëÉí=çÑ=ëóãéíçãë=çÅÅìêêÉÇ=áå=UQB=çÑ=é~íáÉåíëI=ïÜáäÉ=áå=NSB=íÜÉçåëÉí= ï~ë= ~ÅìíÉI= ïáíÜ= íÜÉ= éä~íÉ~ì= çÑ= Çáë~Äáäáíó= ÄÉáåÖ= êÉ~ÅÜÉÇ= ïáíÜáå= QïÉÉâëK= få= ã~åó= Å~ëÉëI= íÜÉ= Çá~Öåçëáë= çÑ= `fam= áë= ã~ÇÉ= êÉíêçëéÉÅíáîÉäóÄÉÅ~ìëÉ=çÑ=íÜÉ=ëìÄëÉèìÉåí=êÉä~éëáåÖ=çê=éêçÖêÉëëáîÉ=ÅçìêëÉI=çê=ëÉÅçåÇ~êóáåîçäîÉãÉåí=çÑ=çíÜÉê=åÉêîÉ=íÉêêáíçêáÉëK=

Chapter 5 Chronic inflammatory demyelinating polyneuropathy

57

Chronic inflammatory demyelinating polyneuropathy

Gillian is a 52-year-old diabetic nurse who developed lumbar back pain, which

became severe. Imaging revealed an old crush fracture and she was admitted to an

orthopaedic ward. After a couple of weeks she was transferred to a rehabilitation

facility. She developed a band-like sensation round her lower thoracic region and by

the time she was discharged home from the rehabilitation facility after 3 weeks, she

was bent over and unable to walk without the assistance of a stick. She was seen by

a senior colleague, 2 months after the onset of her symptoms, who found a normal

neurological examination apart from a relative diminution in the knee and ankle

deep tendon reflexes. However, he was significantly worried about her deterioration

and arranged for her admission to the neurology service a couple of days later. She

was re-examined by the admitting attending neurologist who found hip flexion

weakness in the 4/5 range, normal power at the knees, but weakness of dorsiflexion

of the feet in the 4+/5 range and her deep tendon reflexes were absent at her knees

and ankles. The plantar responses were flexor. A clinical diagnosis of chronic

inflammatory demyelinating polyneuropathy was made, which was confirmed by her

cerebrospinal fluid (CSF) showing an elevated protein of 0.95g/L and the peripheral

electrophysiology confirming demyelination. She was started on 0.4g intravenous

immunoglobulin per kg per day for 5 days. Within a week she had significantly

improved and was able to ambulate. Further treatment will be initiated, such as

steroids and azathioprine, if she fails to continue to improve or deteriorates.


Neurological manifestations at steady state

qÜÉ= ÅäáåáÅ~ä= ã~åáÑÉëí~íáçåë= ~í= íÜÉ= ÅÜêçåáÅ= éÜ~ëÉI= ëíÉ~Çó= ëí~íÉI= çê= ~íêÉÑÉêê~äI= êÉÑäÉÅí= íÜÉ= ÉñíêÉãÉ= ëóãéíçã~íáÅ= î~êáÉíó= çÑ= `famK= lå= ~îÉê~ÖÉãçíçê=ÇÉÑáÅáí=çÅÅìêë=áå=UPJVQB=çÑ=é~íáÉåíë=~åÇ=ëÉåëçêó=ÇÉÑáÅáí=áå=TOJUVBKc~Åá~ä=é~äëó=áë=çÄëÉêîÉÇ=áå=QJNRB=~åÇ=äçëë=çÑ=íÉåÇçå=êÉÑäÉñÉë=áå=USJVQBçÑ=é~íáÉåíëK=aóë~ìíçåçãá~=áë=åçí=~=ÑÉ~íìêÉ=çÑ=`famK=

Increased CSF protein content

fåÅêÉ~ëÉÇ= `pc= éêçíÉáå= ÅçåíÉåí= áë= ~= ã~àçê= ÑÉ~íìêÉ= áå= `famK= pçãÉ~ìíÜçêë=Ü~îÉ=ã~ÇÉ=áí=ã~åÇ~íçêó=Ñçê=Çá~Öåçëáë=íÜ~í=íÜÉêÉ=ÄÉ=~=`pc=éêçíÉáåÅçåíÉåí= çÑ= ãçêÉ= íÜ~å= MKQRÖLiI= ïáíÜ= äÉëë= íÜ~å= NM= ÅÉääë= éÉê= ãäK= få= çìêëíìÇáÉëI= íÜÉ= `pc= éêçíÉáå= ÅçåíÉåí= ï~ë= åçêã~ä= áå= NQB= çÑ= é~íáÉåíë= ~åÇÅÉääìä~êáíó= ï~ë= åçêã~ä= áå= ~ää= çÑ= íÜÉãK= tÜÉå= íÜÉ= `pc= áë= åçêã~äI= áí= áëã~åÇ~íçêó= íç= ëìééçêí= íÜÉ= Çá~Öåçëáë= ïáíÜ= ìåÉèìáîçÅ~ä= ÇÉãóÉäáå~íáîÉÑÉ~íìêÉë=çå=ÉäÉÅíêçéÜóëáçäçÖáÅ~ä=íÉëíë=çê=é~íÜçäçÖáÅ~ä=Ç~í~K

Clinical variants of CIDP

`äáåáÅ~ä= ÇáîÉêëáíó= áå= íÜÉ= éêÉëÉåí~íáçå= ~åÇ= ÅçìêëÉ= ~êÉ= íÜÉ= ãçëíêÉã~êâ~ÄäÉ=ÑÉ~íìêÉë=çÑ=`famK

Focal and multifocal neuropathy and CIDP

`ÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜó= éêÉëÉåíë= áå= ê~êÉáåëí~åÅÉë=ïáíÜ=ÑçÅ~ä=çê=ãìäíáÑçÅ~ä=ìééÉê=äáãÄ=áåîçäîÉãÉåíK=póãéíçãë=Å~åÄÉÖáå= áå=çåÉ=~êã=çê=Ü~åÇI=çê= áå=ÄçíÜ=~êãë=çê=Ü~åÇë=ïáíÜ=é~ê~ÉëíÜÉëá~ëIïÉ~âåÉëëI=~åÇ=é~áå=SK

cáåÇáåÖë=Å~å=ÄÉ=áåáíá~ääó=êÉëíêáÅíÉÇ=íç=íÜÉ=çåÉ=åÉêîÉ=ÇáëíêáÄìíáçå=çê=áåîçäîÉãìäíáéäÉ=åÉêîÉëK=^ëáÇÉ=Ñêçã=íÜÉ=ÑçÅ~ä=çåëÉíI=íÜÉêÉ=áë=åç=ÇáÑÑÉêÉåÅÉ=ÄÉíïÉÉåÑçÅ~ä=~åÇ=ÖÉåÉê~äáëÉÇ=`famK


58


Chronic sensory demyelinating polyneuropathy

pçãÉ=é~íáÉåíë=éêÉëÉåí=ïáíÜ= áëçä~íÉÇ=ëÉåëçêó=ã~åáÑÉëí~íáçåëI= áåÅäìÇáåÖ~í~ñá~I=é~áåI=~åÇ=é~ê~ÉëíÜÉëá~ë=çÑ=íÜÉ=äçïÉê=ÉñíêÉãáíáÉëI=ïÜáÅÜ=êÉéêÉëÉåí=~ëìÄëÉí= çÑ= `famK= qïç= Öêçìéë= çÑ= é~íáÉåíë= Å~å= ÄÉ= áÇÉåíáÑáÉÇ= áå= íÜçëÉéêÉëÉåíáåÖ=ïáíÜ=áëçä~íÉÇ=ëÉåëçêó=ã~åáÑÉëí~íáçåëK=få=íÜÉ=Ñáêëí=ÖêçìéI=ëÉåëçêóã~åáÑÉëí~íáçåë=~êÉ=ÑçääçïÉÇ=~ÑíÉê=~=î~êá~ÄäÉ=éÉêáçÇ=çÑ=íáãÉ=Äó=ãçíçê=ÇÉÑáÅáíKfå= íÜÉ=ëÉÅçåÇ=Öêçìé= íÜÉ=ëáÖåë=~åÇ=ëóãéíçãë=êÉã~áå=éìêÉäó=ëÉåëçêó= ÑçêóÉ~êë= çê= ÇÉÅ~ÇÉëK= få= çìê= ëíìÇó= çÑ= OU= é~íáÉåíë= ïáíÜ= áëçä~íÉÇ= ëÉåëçêóã~åáÑÉëí~íáçåë=~í=çåëÉíI=ÑáîÉ=ÇÉîÉäçéÉÇ=ãçíçê=ÇÉÑáÅáí=çå=~îÉê~ÖÉ=QKR=óÉ~êë~ÑíÉê=íÜÉ=çåëÉí=TK=få=íÜÉ=êÉã~áåáåÖ=é~íáÉåíë=íÜÉ=åÉìêçé~íÜó=êÉã~áåÉÇ=éìêÉäóëÉåëçêó= ~ÑíÉê= ~= ãÉ~å= ÑçääçïJìé= çÑ= TKO= óÉ~êëK= i~êÖÉ= ÑáÄêÉ= ÑìåÅíáçå= ï~ëáãé~áêÉÇ= áå= ~ää= é~íáÉåíëX= é~áå= ~åÇ= ~= äçëë= çÑ= íÉãéÉê~íìêÉ= ëÉåë~íáçå= ï~ëÑçìåÇ=áå=VMB=çÑ=Å~ëÉëK=qÜÉ=ãÉ~å=`pc=éêçíÉáå=äÉîÉä=ï~ë=MKTTÖLiI=Äìí=íÜÉ`pc= ï~ë= åçêã~ä= áå= NO= EQQBF= é~íáÉåíëK= ^í= äÉ~ëí= çåÉ= ãçíçê= ÅçåÇìÅíáçåé~ê~ãÉíÉêI= áåÅäìÇáåÖ=ãçíçê=ÅçåÇìÅíáçå=îÉäçÅáíó=Ej`sF=~åÇ=cJï~îÉ=Çáëí~ää~íÉåÅóI=ï~ë= áå= íÜÉ=ÇÉãóÉäáå~íáåÖ= ê~åÖÉ= áå=VSB=çÑ=é~íáÉåíëK=`çåÇìÅíáçåÄäçÅâ=~åÇ=íÉãéçê~ä=ÇáëéÉêëáçå=ïÉêÉ=ÑçìåÇ=áå=PMB=çÑ=é~íáÉåíëK=qÜÉ=ÅçìêëÉï~ë= éêçÖêÉëëáîÉ= Ñêçã= íÜÉ= çåëÉí= áå= OO= é~íáÉåíë= EUNKRBFI= ëÉÅçåÇ~êóéêçÖêÉëëáîÉ= áå= çåÉI= ~åÇ= êÉä~éëáåÖ= áå= Ñçìê= ENRBFK= aáë~ÄáäáíóI= ïÜáÅÜ= ï~ëã~áåäó= ÇìÉ= íç= ~í~ñá~= ~åÇ= äçëë= çÑ= í~ÅíáäÉ= ÇáëÅêáãáå~íáçåI= ï~ë= éêÉëÉåí= ~íêÉÑÉêê~ä= áå= OSB= çÑ= é~íáÉåíëK= fãéêçîÉãÉåí= ï~ë= åçíÉÇ= áå= ULNR= ERPBFé~íáÉåíë=íêÉ~íÉÇ=ïáíÜ=çê~ä=éêÉÇåáëçåÉ=~åÇ=áå=QLNQ=EOVBF=é~íáÉåíë=íêÉ~íÉÇïáíÜ=áåíê~îÉåçìë=áããìåçÖäçÄìäáåë=EfsfdëFK=

Pure motor pattern

mìêÉ=ãçíçê=é~ííÉêåë=~êÉ=çÄëÉêîÉÇ=áå=íÜÉ=ë~ãÉ=éêçéçêíáçå=çÑ=é~íáÉåíë=~ëéìêÉ= ëÉåëçêó= ÑçêãëK= få= ëçãÉ= Å~ëÉë= îÉåíê~ä= êççíë= ÄÉ~ê= íÜÉ= Äêìåí= çÑÇÉãóÉäáå~íáçåI=~ë=áë=çÑíÉå=íÜÉ=Å~ëÉ=áå=d_pK

CIDP in childhood

`fam=áë=ê~êÉê=áå=ÅÜáäÇêÉå=íÜ~å=áå=~Çìäíë=Äìí=íÜÉ=ÅäáåáÅ~ä=~ëéÉÅíëI=ÅçìêëÉ~åÇ= êÉëéçåëÉ= íç= íêÉ~íãÉåí= ~êÉ= ëáãáä~ê= íç= íÜ~í= áå= ~ÇìäíJçåëÉí= `famK= få= ~ëíìÇó= Åçãé~êáåÖ= NO= ÅÜáäÇêÉå= ïáíÜ= áÇáçé~íÜáÅ= `fam= íç= SO= ~Çìäíë= ïáíÜ


59


áÇáçé~íÜáÅ=`famI=páããçåë=~åÇ=ÅçïçêâÉêë= ÑçìåÇ= íÜ~í=ÅÜáäÇêÉå=çÑíÉå=Ü~ÇãçêÉ= ê~éáÇäó= ÑäìÅíì~íáåÖ= ÅçìêëÉë= íÜ~å= ~ÇìäíëX= ~= êÉä~éëáåÖ= ÅçìêëÉ= ï~ëëáÖåáÑáÅ~åíäó= ãçêÉ= Åçããçå= áå= ÅÜáäÇêÉå= íÜ~å= áå= ~Çìäíë= UK= qÜÉ= êÉÅçîÉêó= çÑÅÜáäÇêÉå= Ñêçã= É~ÅÜ= ÉéáëçÇÉ= çÑ= ÇÉíÉêáçê~íáçå= ï~ë= ìëì~ääó= ÉñÅÉääÉåí= ~åÇÄÉííÉêI=çå=~îÉê~ÖÉI=íÜ~å=áå=~ÇìäíëK

CIDP in diabetic patients

m~íáÉåíë= ïáíÜ= Çá~ÄÉíÉë= çÅÅ~ëáçå~ääó= ÇÉîÉäçé= ÅäáåáÅ~ä= ~åÇÉäÉÅíêçÇá~ÖåçëíáÅ= ÑÉ~íìêÉë= ëìÖÖÉëíáîÉ= çÑ= `famK= qÜáë= Çá~Öåçëáë= ãìëí= ÄÉëìëéÉÅíÉÇ=ïÜÉå=~=éêÉÇçãáå~åíäó=ãçíçê=~åÇ=~í~ñáÅ=éçäóåÉìêçé~íÜó=çÅÅìêëáå=~=Çá~ÄÉíáÅ=é~íáÉåíK=få=çåÉ=ëíìÇó=çÑ=Çá~ÄÉíáÅ=é~íáÉåíë=ïáíÜ=`famI=íÜÉ=åÉêîÉÅçåÇìÅíáçå= ëíìÇáÉë= ëÜçïÉÇ= ãçêÉ= ëÉîÉêÉ= ~ñçå~ä= äçëë= ~åÇ= íÜÉ= ÇÉÖêÉÉ= çÑáãéêçîÉãÉåí=ÑçääçïáåÖ=íêÉ~íãÉåí=ï~ë=äÉëë=Ñ~îçìê~ÄäÉ=VK

Postural and action tremor in CIDP

mçëíìê~ä=~åÇ=~Åíáçå=íêÉãçêë=Å~å=ÄÉÅçãÉ=~=îÉêó=Çáë~ÄäáåÖ=ëóãéíçã=áåé~íáÉåíë= ïáíÜ= `famK= pìÅÜ= íêÉãçêë= çÅÅìê= áå= é~íáÉåíë= ïáíÜ= ãáåáã~ä= ãçíçêïÉ~âåÉëë=êÉÖ~êÇäÉëë=çÑ=íÜÉ=áåíÉåëáíó=çÑ=ëÉåëçêó=ã~åáÑÉëí~íáçåëK=fåÅêÉ~ëÉÇéÜóëáçäçÖáÅ~ä= íêÉãçêë= ~êÉ= êÉä~íÉÇ= íç= ïÉ~âåÉëë= ïáíÜ= ~= éçëëáÄäÉ= êçäÉ= çÑÇÉÅêÉ~ëÉÇ=áåéìí=Ñêçã=~ÑÑÉêÉåí=ä~êÖÉ=ãóÉäáå~íÉÇ=ÑáÄêÉëK=

Central nervous system (CNS) involvement in CIDP or CIDPand multiple sclerosis (MS)

`fam=ï~ë=~ëëçÅá~íÉÇ=ïáíÜ=ëóãéíçã~íáÅ= äÉëáçåë=çÑ= íÜÉ=`kp=áå=RB=çÑÅ~ëÉë= áå= çìê= ëÉêáÉë= PK= fã~ÖáåÖ= ÅÜ~ê~ÅíÉêáëíáÅë= çÑ= jp= ïÉêÉ= ÑçìåÇ= áå= íÜÉíÜêÉÉ= é~íáÉåíë= ïÜç= ìåÇÉêïÉåí= ã~ÖåÉíáÅ= êÉëçå~åÅÉ= áã~ÖáåÖ= EjofFK= ^ääé~íáÉåíë=ïáíÜ=`kp=áåîçäîÉãÉåí=ïÉêÉ=ëÉîÉêÉäó=Ü~åÇáÅ~ééÉÇK

Clinical course and prognosis

qÜÉ=äçåÖJíÉêã=çìíÅçãÉ=çÑ=`fam=áë=ìåéêÉÇáÅí~ÄäÉ=áå=íÜÉ=É~êäó=ëí~ÖÉë=çÑíÜÉ=ÇáëÉ~ëÉK=^=î~êá~ÄäÉ=éêçéçêíáçå=çÑ=Å~ëÉë=Ñçääçï=~=êÉä~éëáåÖ=çê=ÅÜêçåáÅ


60


éêçÖêÉëëáîÉ=ÅçìêëÉI=ïáíÜ=ã~åó=é~íáÉåíë= ëí~êíáåÖ=ïáíÜ=~= êÉä~éëáåÖ=ÅçìêëÉÑçääçïÉÇ=Äó=~=ëÉÅçåÇ~êó=éêçÖêÉëëáîÉ=ÅçìêëÉK=få=íÜ~í=êÉëéÉÅí=`fam=Å~å=ÄÉÅçåëáÇÉêÉÇ=~=éÉêáéÜÉê~ä=~å~äçÖìÉ=çÑ=jpK=få=~ÇÇáíáçåI=àìëí=~ë=áå=jpI=äçëë=çÑ~ñçåë=áë=~=ã~àçê=åÉÖ~íáîÉ=éêçÖåçëíáÅ=Ñ~Åíçê=áÇÉåíáÑáÉÇ=áå=`famK

_çìÅÜ~êÇ=Éí=~ä êÉîáÉïÉÇ=íÜÉ=ÑçääçïJìé=Ç~í~=çÑ=UP=é~íáÉåíë=ÅçääÉÅíÉÇ=SóÉ~êë= çå= ~îÉê~ÖÉ= ~ÑíÉê= íÜÉ= Ñáêëí= ã~åáÑÉëí~íáçåë= çÑ= åÉìêçé~íÜó= PK= qÜÉçìíÅçãÉ=ï~ë=ÖççÇ=áå=RSB=çÑ=é~íáÉåíëK=káåÉ=é~íáÉåíë=ÇáÉÇ=~ë=~=êÉëìäí=çÑéêçÖêÉëëáçå=çÑ=íÜÉ=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáí=íç=èì~ÇêáéäÉÖá~I=~åÇ=êÉëéáê~íçêó=~åÇëï~ääçïáåÖ=ÇáÑÑáÅìäíóK=qÜÉ=ãÉ~å=~ÖÉ=çÑ=é~íáÉåíë=ïÜç=ÇáÉÇ=ï~ë=ST=óÉ~êëKoÉä~éëáåÖ= Ñçêãë= Å~êêáÉÇ= ~= ÄÉííÉê= éêçÖåçëáë= íÜ~å= éêçÖêÉëëáîÉ= ÑçêãëI= áåâÉÉéáåÖ=ïáíÜ=~=ÄÉííÉê=êÉëéçåëÉ=çÑ=é~íáÉåíë=ïáíÜ=~ÅìíÉäó=êÉä~éëáåÖ=`fam=íçfsfd= íêÉ~íãÉåíK= ^= êÉä~éëáåÖ= ~åÇ= êÉãáííáåÖ= ÅçìêëÉ= éêÉÇçãáå~íÉÇ= áå= íÜÉàìîÉåáäÉ= ÖêçìéK= cìåÅíáçå~ä= êÉÅçîÉêó= ï~ë= Åçããçå= áå= ~ää= ~ÖÉ= ÖêçìéëI= Äìíï~ë=íÜÉ=äÉ~ëí=~éé~êÉåí=áå=íÜÉ=ÉäÇÉêäó=ÖêçìéK

Electrophysiological data

qÜÉ=ã~áå=éìêéçëÉ=çÑ=ÉäÉÅíêçéÜóëáçäçÖáÅ~ä=ëíìÇáÉë=áå=é~íáÉåíë=ëìëéÉÅíÉÇçÑ= `fam= áë= íç= Éëí~ÄäáëÜ= íÜÉ= éêÉëÉåÅÉ= çÑ= ÑçÅ~äI= ãìäíáÑçÅ~äI= çê= ÇáÑÑìëÉÇÉãóÉäáå~íáçåI=~åÇ= íç=~ëÅÉêí~áå= íÜÉ=~å~íçãáÅ~ä=ÉñíÉåí=~åÇ=ÇáëíêáÄìíáçå=çÑ~Äåçêã~äáíáÉëK= få= éê~ÅíáÅÉI= íÜÉ= Çá~Öåçëáë= çÑ= `fam= êÉëíë= ã~áåäó= çåÇÉãçåëíê~íáçå= çÑ= ~å= ~ëóããÉíêáÅ~ä= ÇÉãóÉäáå~íáåÖ= éêçÅÉëëI= ~åÇ= é~íáÉåíëïáíÜ=~ÅèìáêÉÇ=ÇÉãóÉäáå~íáåÖ=åÉìêçé~íÜó=çÑíÉå=Ü~îÉ=~=ÇáÑÑÉêÉåíá~ä=ëäçïáåÖ=çÑÅçåÇìÅíáçå= îÉäçÅáíó= ïÜÉå= éêçñáã~ä= ~åÇ= Çáëí~ä= ä~íÉåÅáÉë= çÑ= Éèìáî~äÉåíëÉÖãÉåíë=çÑ=íïç=åÉêîÉë=áå=íÜÉ=ë~ãÉ=äáãÄ=~êÉ=Åçãé~êÉÇK=

`êáíÉêá~=Ñçê=`fam=áåÅäìÇÉW=

√ ãçíçê= ÅçåÇìÅíáçå= îÉäçÅáíó= çÑ= äÉëë= íÜ~å= TRB= çÑ= íÜÉ= äçïÉê= äáãáí= çÑåçêã~ä=î~äìÉëX

√ Çáëí~ä=ãçíçê=ä~íÉåÅáÉë=ÖêÉ~íÉê=íÜ~å=NQMB=çÑ=åçêã~ä=î~äìÉëX√ ÅçåÇìÅíáçå= ÄäçÅâ= ~åÇLçê= íÉãéçê~ä= ÇáëéÉêëáçå= çÑ= íÜÉ= ÅçãéçìåÇ

ãìëÅäÉ=~Åíáçå=éçíÉåíá~äX√ áåÅêÉ~ëÉÇ=cJï~îÉ=ä~íÉåÅó=íç=ÖêÉ~íÉê=íÜ~å=NOMB=çÑ=åçêã~äK


61


bäÉÅíêçéÜóëáçäçÖáÅ~ä= íÉëíëI= ~äíÜçìÖÜ= ÅêìÅá~ä= Ñçê= íÜÉ= Çá~Öåçëáë= çÑÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜáÉëI= Çç= åçí= óáÉäÇ= ÅäÉ~êJÅìí= êÉëìäíë= áå= ã~åóÅ~ëÉëI= ÇìÉ= íç= íÜÉ= ãáñíìêÉ= çÑ= ~ñçå~ä= äçëë= ïáíÜ= ÇÉãóÉäáå~íáîÉ= ÑÉ~íìêÉë= áåé~íáÉåíë= ïáíÜ= éêáã~êáäó= ÇÉãóÉäáå~íáåÖ= åÉìêçé~íÜáÉëK= kÉêîÉ= Äáçéëó= ëÜçìäÇÄÉ=ÅçåëáÇÉêÉÇ=ïÜÉå=~=ÅäáåáÅ~ä=ëìëéáÅáçå=çÑ=~å=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖåÉìêçé~íÜó= êÉã~áåë= áå= é~íáÉåíë= ïÜç= Çç= åçí= ãÉÉí= íÜÉ= éêçéçëÉÇÉäÉÅíêçÇá~ÖåçëíáÅ= ÅêáíÉêá~= Ñçê= ÇÉãóÉäáå~íáçåK= kÉêîÉ= Äáçéëó= ~äëç= Ü~ë= áíëéáíÑ~ääë= ÄÉÅ~ìëÉ= íÜÉ= ÇÉãóÉäáå~íáåÖ= éêçÅÉëë= áë= åçí= ÜçãçÖÉåÉçìë= ~åÇ= áëÄ~ëáÅ~ääó= ~ëóããÉíêáÅ~äI= ~åÇ= ÄÉÅ~ìëÉ= ã~êâÉÇ= áåÑä~ãã~íçêó= áåÑáäíê~íÉë= ~êÉåçí=~äï~óë=éêÉëÉåí=~í=íÜÉ=ëáíÉ=çÑ=ÄáçéëóK=kÉêîÉ=Äáçéëó=ã~ó=ëÜçï=çåäó=åçåJëéÉÅáÑáÅ=äÉëáçåë=ïÜÉå=ÇÉãóÉäáå~íáçå=~åÇ=áåÑä~ãã~íáçå=~êÉ=éêçñáã~ä=íç=íÜÉëáíÉ=çÑ=íÜÉ=ÄáçéëóK=qÜìëI=É~ÅÜ=ãÉíÜçÇ=Ü~ë=áíë=äáãáíëK

Neuroimaging

^å= áåÅêÉ~ëÉÇ= ëáÖå~ä= áåíÉåëáíó= çå= qOJïÉáÖÜíÉÇ= áã~ÖÉë= çÑ= íÜÉ= Äê~ÅÜá~äéäÉñìë=Å~å=ÄÉ=ëÉÉå=çå=jof=áå=é~íáÉåíë=ïáíÜ=`famI=~åÇ=~äëç=áå=é~íáÉåíë=ïáíÜãìäíáÑçÅ~ä=ãçíçê=åÉìêçé~íÜó=EjjkFI=ïÜáÅÜ=ã~ó=ÄÉ=ìëÉÑìä=íç=ÇáÑÑÉêÉåíá~íÉíÜÉ= ä~ííÉê= Ñêçã= äçïÉê= ãçíçê= åÉìêçå= ÇáëÉ~ëÉK= jof= ëíìÇó= ã~ó= ëÜçïÜóéÉêíêçéÜó= çÑ= ëéáå~ä= êççíë= ~åÇ= éäÉñìëÉë= ïáíÜ= çê= ïáíÜçìí= Ö~ÇçäáåáìãÉåÜ~åÅÉãÉåíK=fí=áë=ìëÉÑìä=íç=âåçïI=ÜçïÉîÉêI=íÜ~í=áåÅêÉ~ëÉÇ=ëáÖå~ä=áåíÉåëáíóçå=qOJïÉáÖÜíÉÇ=áã~ÖÉë=çÑ=íÜÉ=Äê~ÅÜá~ä=éäÉñìë=~åÇ=ëÅá~íáÅ=åÉêîÉ=Å~å=çÅÅìêáå=çíÜÉê=ÅçåÇáíáçåë= áåÅäìÇáåÖ= áåÑáäíê~íáåÖ=ã~äáÖå~åí= äóãéÜçã~=~åÇ=ëÅá~íáÅ~ëÉÅçåÇ~êó=íç=Çáëâ=ÜÉêåá~íáçåI=~ë=ïÉ=Ü~îÉ=çÄëÉêîÉÇK=

Morphological findings

mÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=EmkpF= äÉëáçåë=Åçåëáëí=çÑ=é~íÅÜó=êÉÖáçåë=çÑÇÉãóÉäáå~íáçå=ïáíÜ= î~êá~ÄäÉ= áåÑä~ãã~íçêó= áåÑáäíê~íÉëI= ÉåÇçåÉìêá~ä= çÉÇÉã~IÇÉãóÉäáå~íÉÇ= ÑáÄêÉëI=ã~ÅêçéÜ~ÖÉJãÉÇá~íÉÇ=ÇÉãóÉäáå~íáçåI= êÉãóÉäáå~íáçåIpÅÜï~åå= ÅÉää= éêçäáÑÉê~íáçå= ïáíÜ= çåáçåJÄìäÄ= Ñçêã~íáçåI= áåÑä~ãã~íçêóáåÑáäíê~íáçå= ïáíÜ= ãçåçåìÅäÉ~ê= ÅÉääëI= ~ñçå~ä= ÇÉÖÉåÉê~íáçåI= ~åÇ= ~ñçå= äçëëEcáÖìêÉë=N=~åÇ=OFK=qÜÉ=éêÉëÉåÅÉ=çÑ=ã~ÅêçéÜ~ÖÉ=ëíêáééáåÖ=çÑ= íÜÉ=ãóÉäáåëÜÉ~íÜ= áë= Çá~ÖåçëíáÅ= çÑ= ~å= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ= åÉìêçé~íÜóKeçïÉîÉêI=íÜÉ=ÇÉãçåëíê~íáçå=çÑ=ã~ÅêçéÜ~ÖÉ=ëíêáééáåÖ=çÑ=íÜÉ=ãóÉäáå=ëÜÉ~íÜ~åÇ= ÉîÉå= çÑ= áåÑä~ãã~íçêó= áåÑáäíê~íÉ= áë= Ñ~ê= Ñêçã= ìåáîÉêë~ä= áå= åÉêîÉ= Äáçéëó


62



63

Figure 1. Longitudinal section of a nerve biopsy specimen

from a patient with CIDP. The arrow points to a demyelinated

axon surrounded by a number of mononuclear cells. (Bar:

10μm.)

Figure 2. Electron micrograph of a cross-section of a nerve

biopsy specimen from a patient with CIDP to show a

demyelinated axon (arrow) and a remyelinating axon

surrounded by a thin myelin sheath (asterisk). (Bar: 1μm.)

*


ëéÉÅáãÉåëK=^å=áãéçêí~åí=ÑáåÇáåÖ=ï~ë=íÜ~í=íÜÉ=ÇÉåëáíó=çÑ=ãóÉäáå~íÉÇ=åÉêîÉÑáÄêÉë=ï~ë=ÄÉäçï=RMB=çÑ=Åçåíêçä=î~äìÉë=áå=QTB=çÑ=é~íáÉåíë=PK=

Immunological factors

_çíÜ=ÅÉääJãÉÇá~íÉÇ=ãÉÅÜ~åáëãë=~åÇ=~å=~åíáÄçÇóJãÉÇá~íÉÇ=êÉëéçåëÉ=íçã~àçê=ÖäóÅçäáéáÇ=çê=ãóÉäáå=éêçíÉáå=~åíáÖÉåë=Ü~îÉ=ÄÉÉå=áåÅêáãáå~íÉÇK=àQH~åÇ=àUH=qJÅÉääë=ã~ó=ÄÉ=ÇÉãçåëíê~íÉÇ= áå=åÉêîÉ=Äáçéëó=ëéÉÅáãÉåëI=Äìíã~ÅêçéÜ~ÖÉë= ÅçåëíáíìíÉ= íÜÉ= ã~àçê= ÅÉää= ÅçãéçåÉåí= çÑ= íÜÉ= áåÑä~ãã~íçêóáåÑáäíê~íÉK=`çåÑäáÅíáåÖ=êÉëìäíë=Ü~îÉ=ÄÉÉå=êÉéçêíÉÇ=êÉÅÉåíäó=ïáíÜ=êÉÖ~êÇ=íç=íÜÉ~åíáÄçÇóJãÉÇá~íÉÇ= ãÉÅÜ~åáëãëK= få= ëìãã~êóI= ~å= áããìåÉ= ãÉÅÜ~åáëã= áëîÉêó=äáâÉäó=áå=`fam=Äìí=åç=êÉäá~ÄäÉ=áããìåçäçÖáÅ~ä=íÉëí=çê=Çá~ÖåçëíáÅ=íççä=áë~î~áä~ÄäÉ=ëç=Ñ~êK=

Differential diagnosis

^= ëÉ~êÅÜ= Ñçê= ãçåçÅäçå~ä= Ö~ããçé~íÜó= EãçåçÅäçå~ä= éêçäáÑÉê~íáçå= çÑäóãéÜçáÇ= ÅÉääë= éêçÇìÅáåÖ= áããìåçÖäçÄìäáåëF= áë= áãéçêí~åí= áå= é~íáÉåíë= ïáíÜ`famK= pÉîÉê~ä= é~ííÉêåë= çÑ= ÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜáÉë= Å~å= ÄÉ~ëëçÅá~íÉÇ=ïáíÜ=ãçåçÅäçå~ä=Ö~ããçé~íÜóK=qÜÉ=ãçëí=Åçããçå=é~ííÉêå=áë=íÜÉ~ëëçÅá~íáçå= çÑ= `fam= ïáíÜ= ÄÉåáÖå= ãçåçÅäçå~ä= Ö~ããçé~íÜó= çÑ= ìåâåçïåëáÖåáÑáÅ~åÅÉ=EjdrpFK

qÜÉ=ã~àçê=ÇáÑÑÉêÉåÅÉ=ÄÉíïÉÉå=é~íáÉåíë=ïáíÜ=jdrp=~åÇ=`fam=áë=íÜÉ=êáëâçÑ= ã~äáÖå~åÅó= áå= íÜÉ= ÑçääçïáåÖ= óÉ~êë= áå= é~íáÉåíë= ïáíÜ= jdrp= ~åÇ= ~å~ÅèìáêÉÇ=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜóI=ëç=é~íáÉåíë=åÉÉÇ=íç=ÄÉ=éÉêáçÇáÅ~ääóíÉëíÉÇ=Ñçê=íÜáë=éçëëáÄáäáíóK

qÜÉ= ~ëëçÅá~íáçå= çÑ= ÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜóI= çêÖ~åçãÉÖ~äó= ïáíÜÉåÇçÅêáåçé~íÜóI=ãçåçÅäçå~ä=Ö~ããçé~íÜó=~åÇ=ëâáå=äÉëáçåë=áë=âåçïå=~ë=íÜÉmlbjp= ëóåÇêçãÉK= få= íÜáë= ëóåÇêçãÉI= éêçÖêÉëëáîÉ= ëÉåëçêóJãçíçêÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜó=áë=~ëëçÅá~íÉÇ=ïáíÜ=ãçåçÅäçå~ä=Ö~ããçé~íÜó~åÇ= çíÜÉê= ìåÅçããçå= ã~åáÑÉëí~íáçåë= áåÅäìÇáåÖ= ëâáå= éáÖãÉåí~íáçåIÜÉé~íçëéäÉåçãÉÖ~äóI= é~éáääçÉÇÉã~I= Éåä~êÖÉÇ= äóãéÜ= åçÇÉëIÉåÇçÅêáåçé~íÜóI= çÉÇÉã~I= íÜêçãÄçÅóíçëáëI= ~åÇ= ÉäÉî~íÉÇ= î~ëÅìä~êÉåÇçíÜÉäá~ä=ÖêçïíÜ=Ñ~Åíçê=EsbdcFK=^=íçí~ä=ÄçÇó=ëÅ~å=ãìëí=ÄÉ=éÉêÑçêãÉÇ=íç


64


ÇÉíÉÅí= ëÅäÉêçíáÅ= ÄçåÉ= äÉëáçåëK= qÜáë= ëóåÇêçãÉ= çÑíÉå= êÉëéçåÇë= ïÉää= íçëéÉÅáÑáÅ=íêÉ~íãÉåí=Ñçê=éä~ëã~Åóíçã~=NMK

^= äáÑÉJíÜêÉ~íÉåáåÖ= ÇáëçêÇÉê= êÉëìäíë= Ñêçã= íÜÉ= éêÉëÉåÅÉ= çÑ= äáÖÜí= ÅÜ~áåJÇÉêáîÉÇ=~ãóäçáÇ=ÇÉéçëáíë= áå=íÜÉ=åÉêîÉ=ÉåÇçåÉìêáìã=~åÇ=çíÜÉê=çêÖ~åëK= fåíÜáë=ëÉííáåÖI=íÜÉ=çÅÅìêêÉåÅÉ=çÑ=~ìíçåçãáÅ=ÇáëíìêÄ~åÅÉëI=áå=~ëëçÅá~íáçå=ïáíÜíÜÉ= ãçåçÅäçå~ä= Ö~ããçé~íÜóI= áå= ~= é~íáÉåí= ïáíÜ= éêçÖêÉëëáîÉ= ~ÅèìáêÉÇÇÉãóÉäáå~íáåÖ= éçäóåÉìêçé~íÜó= ëìÖÖÉëíë= íÜÉ= ÇÉîÉäçéãÉåí= çÑ= äáÖÜí= ÅÜ~áå~ãóäçáÇ= åÉìêçé~íÜóK= qÜáë= Ü~ë= ~= îÉêó= éççê= éêçÖåçëáë= ÇìÉ= íç= éêçÖêÉëëáîÉ~ñçå~ä= ÇÉÖÉåÉê~íáçå= çÑ= íÜÉ= ã~àçêáíó= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîÉ= ÑáÄêÉë= ~åÇãìäíáçêÖ~å=Ñ~áäìêÉK

Treatment

`çêíáÅçëíÉêçáÇëI= fsfdëI= éä~ëã~= ÉñÅÜ~åÖÉ= ~åÇ= áããìåçëìééêÉëëáîÉÇêìÖë=~êÉ=íÜÉ=ã~áå=íêÉ~íãÉåíë=ìëÉÇ=áå=íÜáë=ÅçåÇáíáçå=NNK=kÉ~êäó=~ää=é~íáÉåíëïáíÜ= `fam= ïáää= ëÜçï= ~å= áåáíá~ä= êÉëéçåëÉ= íç= áããìåçãçÇìä~íçêó= íÜÉê~éóKbî~äì~íáçå=çÑ=êÉëéçåëÉ=íç=íêÉ~íãÉåíI=ÜçïÉîÉêI=áë=Ü~ãéÉêÉÇ=Äó=íÜÉ=ä~Åâ=çÑçÄàÉÅíáîÉ= ãÉ~ëìêÉëI= éççê= ÅçêêÉä~íáçå= ïáíÜ= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= Ç~í~Iî~êá~ÄäÉ= áåÅáÇÉåÅÉ= çÑ= ~ñçå~ä= ÇÉÖÉåÉê~íáçå= EïÜáÅÜ= áë= ìåäáâÉäó= íç= êÉëéçåÇèìáÅâäó=íç=íêÉ~íãÉåíF=~åÇ=íÜÉ=î~êá~Äáäáíó=çÑ=íÜÉ=ëéçåí~åÉçìë=ÅçìêëÉ=çÑ=íÜÉÇáëÉ~ëÉK

mêÉÇåáëçåÉ=áë=ìëì~ääó=ëí~êíÉÇ=~åÇ=ã~áåí~áåÉÇ=~í=NãÖLâÖLÇ~ó=Ñçê=~=ÑÉïïÉÉâë= ~åÇ= íÜÉå= í~éÉêÉÇ= ÖÉåíäó= çîÉê= ëÉîÉê~ä= ãçåíÜëK= ^äíÉêå~íáîÉäóIíêÉ~íãÉåí=Å~å=ëí~êí=ïáíÜ=~=ÜáÖÜJÇçëÉ=fsfd=EMKQÖ=éÉê=âÖ=ÄçÇó=ïÉáÖÜíI=çå=RÅçåëÉÅìíáîÉ= Ç~óëFI= íÜÉå= çåÅÉ= ~= ãçåíÜ= Ñçê= P= ãçåíÜëI= ~äíÜçìÖÜ= ~í= äçïÉêÇçëÉëX=~=íçí~ä=ÇçëÉ=çÑ=NÖLâÖ=ÄçÇó=ïÉáÖÜí=çîÉê=P=Ç~óë=ÇáÇ=ïçêâ=áå=ãçëí=çÑçìê=Å~ëÉëK=tÜÉêÉ=íÜáë=Ñ~áäëI=éä~ëã~=ÉñÅÜ~åÖÉ=Å~å=ÄÉ=íêáÉÇK=

líÜÉê= íêÉ~íãÉåí= ïáíÜ= áããìåçëìééêÉëëáîÉ= ÇêìÖë= áåÅäìÇáåÖÅóÅäçéÜçëéÜ~ãáÇÉI= ~ò~íÜáçéêáåÉI= ãóÅçéÜÉåçä~íÉI= ~åÇ= ÅáÅäçëéçêáåI= Ü~ëÄÉÉå=íêáÉÇ=áå=é~íáÉåíë=ïÜç=ÇáÇ=åçí=êÉëéçåÇ=íç=éêÉîáçìë=íêÉ~íãÉåíëI=Äìí=íÜÉêÉëéçåëÉ= áë= îÉêó= î~êá~ÄäÉK= qÜÉëÉ= ÇêìÖë= Å~å= ÄÉ= ìëÉÇ= ~ë= ëíÉêçáÇJëé~êáåÖ~ÖÉåíëK= ^= åìãÄÉê= çÑ= èìÉëíáçåë= êÉã~áå= ìå~åëïÉêÉÇ= êÉÖ~êÇáåÖ= íÜÉé~íÜçéÜóëáçäçÖóI= ã~å~ÖÉãÉåí= ~åÇ= íêÉ~íãÉåí= çÑ= `famI= ïÜáÅÜ= êÉã~áåë= ~Çáë~ÄäáåÖ=~åÇ=ëçãÉíáãÉë=äáÑÉJíÜêÉ~íÉåáåÖ=ÇáëçêÇÉêK


65


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NK= ^Ç= eçÅ= pìÄÅçããáííÉÉ= çÑ= íÜÉ= ^ãÉêáÅ~å= ^Å~ÇÉãó= çÑ= kÉìêçäçÖó= ^fap= q~ëâ= cçêÅÉK

oÉëÉ~êÅÜ= ÅêáíÉêá~= Ñçê= íÜÉ= Çá~Öåçëáë= çÑ= ÅÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ

éçäóê~ÇáÅìäçåÉìêçé~íÜó=E`famFK=kÉìêçäçÖó NVVNX=QNW=SNTJUK

OK p~áÇ= dI= hê~êìé= `K= `ÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáîÉ= éçäóåÉìêçé~íÜóK= e~åÇÄ= `äáå

kÉìêçä=OMNPX=NNRW=QMPJNPK=

PK _çìÅÜ~êÇ=Ì=i~Åêçáñ=Ì=mä~åí¨=sI=Éí=~äK=`äáåáÅçé~íÜçäçÖáÅ~ä=ÑáåÇáåÖë=~åÇ=éêçÖåçëáë=çÑ

ÅÜêçåáÅ=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜóK=kÉìêçäçÖó=NVVVX=ROW=QVUJRMPK

QK aóÅâ=mgI=i~áë=^Ì=lÜí~=jI=Éí=~äK=`ÜêçåáÅ=áåÑä~ãã~íçêó=éçäóê~ÇáÅìäçåÉìêçé~íÜóK=j~óç

`äáåáÅ=mêçÅ NVTRX=RMW=SONJPTK

RK jÅ`çãÄÉ= mÎ= mçää~êÇ= gaI= jÅiÉçÇ= gdK= `ÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ

éçäóê~ÇáÅìäçåÉìêçé~íÜóK=_ê~áå=NVUTX=NNMW=NSNTJPMK

SK dçêëçå=hÌ=oçééÉê=êI=tÉáåÄÉêÖ=aeK=rééÉê= äáãÄ=éêÉÇçãáå~åíI=ãìäíáÑçÅ~ä=ÅÜêçåáÅ

áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜóK=jìëÅäÉ=kÉêîÉ NVVVX=OOW=TRUJSRK

TK= cÉêêÉáê~=mI=içòÉêçå=Ì=i~Åêçáñ=aI=Éí=~äK=pÉåëçêó=ÅÜêçåáÅ= áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ

éçäóåÉìêçé~íÜóW=~=ëíìÇó=çÑ=OU=Å~ëÉë=ïáíÜ=åÉêîÉ=ÄáçéëóK=g=kÉìêçä=OMMPX=ORMW=NQVK

UK páããçåë= wI= ^äÄÉêë= gtI= _êçãÄÉêÖ= jI= cÉäÇã~å= biK= içåÖJíÉêã= ÑçääçïJìé= çÑ= é~íáÉåíë

ïáíÜ= ÅÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖ= éçäóê~ÇáÅìäçåÉìêçé~íÜóI= ïáíÜçìí= ~åÇ= ïáíÜ

ãçåçÅäçå~ä=Ö~ããçé~íÜóK=_ê~áå NVVRX=QUW=PRVJSUK

VK dçêëçå=hÌ=oçééÉê=êI=^ÇÉäã~å=ipI=tÉáåÄÉêÖ=aeK=fåÑäìÉåÅÉ=çÑ=Çá~ÄÉíÉë=ãÉääáíìë=çå

ÅÜêçåáÅ=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜóK=jìëÅäÉ=kÉêîÉ OMMMX=OPW=PTJQPK

NMK aáëéÉåòáÉêá=^K=eçï=f=íêÉ~í=mlbjp=ëóåÇêçãÉK=_äççÇ OMNOX=NNVW=RSRMJUK

NNK iÉÜã~åå= eÌ= eìÖÜÉë= oÎ= e~êíìåÖ= emK= qêÉ~íãÉåí= çÑ= ÅÜêçåáÅ= áåÑä~ãã~íçêó

ÇÉãóÉäáå~íáåÖ=éçäóê~ÇáÅìäçåÉìêçé~íÜóK=e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=QNRJOTK


66

√ The diagnosis of CIDP is based on the association of a proximal and distal

motor deficit of subacute onset, nerve conduction slowing and increased CSF

protein content.

√ Sensory CIDP is difficult to diagnose without a nerve biopsy.

√ The course of CIDP can be subacute progressive or relapsing.

√ CIDP is often associated with monoclonal gammopathy of unknown significance.

√ Corticosteroids, IVIGs and plasma exchange can be tried in CIDP.

Ü Key points


67

Vasculitic neuropathies

Chapter 6

Overview

This chapter describes how an inflammation of blood vessels can cause neuropathies

by affecting the blood vessels that supply nerves (vasa nervorum). These disorders can

occur as part of connective tissue illness such as rheumatoid arthritis or in primary

vasculitides. Occlusion of nerve blood vessels provokes ischaemia of nerve trunks and

axonal degeneration of nerve fibres. Lesions predominate in nerve trunks of the limbs

typically inducing a multifocal sensory and motor neuropathy. Systemic

manifestations are often associated with nerve and skin lesions. Nerve biopsy is

usually required to demonstrate lesions of nerve blood vessels. The mainstay of

treatment is prednisolone, but recovery can be slow and relapses are frequent.

Introduction

s~ëÅìäáíáë= çÅÅìêë= ~ë= ~= éêáã~êó= éÜÉåçãÉåçå= áå= ÅçååÉÅíáîÉ= íáëëìÉÇáëçêÇÉêë=E`qaëF=~åÇ=~ëëçÅá~íÉÇ=áääåÉëëÉëI=áåÅäìÇáåÖ=éçäó~êíÉêáíáë=åçÇçë~Em^kF= ~åÇ= íÜÉ= `ÜìêÖJpíê~ìëë= ëóåÇêçãÉ= E`ppF= î~êá~åíI= êÜÉìã~íçáÇ~êíÜêáíáëI= ëóëíÉãáÅ= äìéìë= ÉêóíÜÉã~íçëìë= EpibFI= ~åÇ= Öê~åìäçã~íçëáë= ïáíÜéçäó~åÖááíáë=EdmF=NK=få=~ää=íÜÉëÉ=ÅçåÇáíáçåë=ÑçÅ~ä=~åÇ=ãìäíáÑçÅ~ä=åÉìêçé~íÜóçÅÅìê=~ë=~=ÅçåëÉèìÉåÅÉ=çÑ=åÉêîÉ=áëÅÜ~Éãá~=êÉä~íÉÇ=íç=ÇÉëíêìÅíáçå=çÑ=íÜÉ~êíÉêá~ä= ï~ää= ~åÇ= çÅÅäìëáçå= çÑ= íÜÉ= äìãÉå= çÑ= ëã~ää= ÉéáåÉìêá~ä= ~êíÉêáÉëKs~ëÅìäáíáë=ã~ó=~äëç=ÅçãéäáÅ~íÉ=íÜÉ=ÅçìêëÉ=çÑ=çíÜÉê=ÅçåÇáíáçåë=ê~åÖáåÖ=ÑêçãáåÑÉÅíáçå=ïáíÜ=íÜÉ=Üìã~å=áããìåçÇÉÑáÅáÉåÅó=îáêìë=EefsF=~åÇ=ïáíÜ=íÜÉ=_=~åÇ`= ÜÉé~íáíáë= îáêìëÉë= íç= Çá~ÄÉíÉë= ~åÇ= ë~êÅçáÇçëáëK= få= ~ää= áåëí~åÅÉëI


ëóãéíçã~íáÅ=î~ëÅìäáíáë=êÉèìáêÉë=ÅçêíáÅçëíÉêçáÇë=íç=Åçåíêçä=íÜÉ=áåÑä~ãã~íçêóéêçÅÉëë=~åÇ=éêÉîÉåí=ÑìêíÜÉê=áëÅÜ~ÉãáÅ=åÉêîÉ=äÉëáçåëK=

Pathophysiology of vasculitis

Primary vasculitis and connective tissue disorders

mêáã~êó=î~ëÅìäáíáÇÉë=~êÉ=çÑíÉå=Åä~ëëáÑáÉÇ=~ÅÅçêÇáåÖ=íç=íÜÉ=ëáòÉ=çÑ=îÉëëÉäëéêÉÇçãáå~åíäó= ~ÑÑÉÅíÉÇK= få= ãÉÇáìãJëáòÉÇ= îÉëëÉäëI= î~ëÅìäáíáÅ= åÉìêçé~íÜóçÅÅìêë= çåäó= áå= é~íáÉåíë= ïáíÜ= m^kK= qÜÉ= Öêçìé= çÑ= ëã~ääJîÉëëÉä= î~ëÅìäáíáÅåÉìêçé~íÜáÉë= áåÅäìÇÉë=dmI= íÜÉ=`pp=OI=~åÇ=ãáÅêçëÅçéáÅ=éçäó~åÖááíáë=ïáíÜÅ~éáää~êó=áåîçäîÉãÉåíK=

qÜÉ= Çá~Öåçëáë= çÑ= m^k= áë= ÜáëíçäçÖáÅ~äK= `êáíÉêá~= áåÅäìÇÉ= íê~åëãìê~äáåÑáäíê~íáçå= çÑ= ëã~ää= ~êíÉêáÉë= ïáíÜ= éçäóãçêéÜçåìÅäÉ~ê= ÅÉääë= EçÑíÉå= ~ÇãáñÉÇïáíÜ= äóãéÜçÅóíÉë= ~åÇ= ÉçëáåçéÜáäëFI= äÉìâçÅóíçÅä~ëá~I= ÑáÄêáåçáÇ= åÉÅêçëáëIÇÉëíêìÅíáçå=çÑ=íÜÉ= áåíÉêå~ä=Éä~ëíáÅ= ä~ãáå~I=çÅÅäìëáçå=çÑ=íÜÉ= äìãÉå=~åÇ=íÜÉìëì~ä=ëé~êáåÖ=çÑ=~Çà~ÅÉåí=îÉåìäÉë=EcáÖìêÉ=NFK=


68

Figure 1. Nerve biopsy from a patient with multifocal

neuropathy due to polyarteritis nodosa. Nerve cross-section of

the paraffin-embedded specimen shows necrotizing arteritis of an

epineurial artery (arrow). (Hematein eosin staining. Bar: 100μm.)


dê~åìäçã~íçëáë= ïáíÜ= éçäó~åÖááíáë= EdmF= áë= ~å= ~åíáÄçÇóJãÉÇá~íÉÇ~ìíçáããìåÉI= Öê~åìäçã~íçìë= î~ëÅìäáíáëI= áå= ïÜáÅÜ= ~åíáÄçÇáÉë= ~Ö~áåëíéêçíÉáå~ëÉ= P= ~åÇ= ãóÉäçéÉêçñáÇ~ëÉ= ~êÉ= ÇÉãçåëíê~í~ÄäÉ= áå= íÜÉ= ëÉêìã= çÑé~íáÉåíëK= pÉêçäçÖáÅ= ÇÉãçåëíê~íáçå= çÑ= íÜÉëÉ= ~åíáJåÉìíêçéÜáä= Åóíçéä~ëãáÅ~åíáÄçÇáÉë= E^k`^ëF= áë= ~= ëÉåëáíáîÉ= ~åÇ= ëéÉÅáÑáÅ= ãÉ~åë= Äó= ïÜáÅÜ= íçÇá~ÖåçëÉ=dmK

Secondary vasculitis

få=î~ëÅìäáíáë=ëÉÅçåÇ~êó=íç=áåÑä~ãã~íçêó=~åÇ=áåÑÉÅíáçìë=ÇáëçêÇÉêëI=íÜÉ=êçäÉçÑ=ÅÉääìä~ê=Ñ~Åíçêë=áë=çÑíÉå=éêçãáåÉåíK=få=ëìÅÜ=ÅçåÇáíáçåëI=ã~ÅêçéÜ~ÖÉë=~åÇÅóíçíçñáÅ=qJäóãéÜçÅóíÉë=ëÉÉã=íç=éä~ó=~=ã~àçê=êçäÉ=áå=îÉëëÉä=ï~ää=Ç~ã~ÖÉK=

The peripheral neuropathy of necrotizing arteritis (NA)

qóéáÅ~ääóI=íÜÉ=ÅäáåáÅ~ä=éáÅíìêÉ=áë=íÜ~í=çÑ=~å=~ÅìíÉ=çê=ëìÄ~ÅìíÉ=ãçåçåÉìêáíáëãìäíáéäÉñK= eçïÉîÉêI= Çáëí~ä= ëóããÉíêáÅ~ä= ëÉåëçêó= çê= ëÉåëçêáãçíçêåÉìêçé~íÜó= çÅÅìêë= áå= QMB= çÑ= Å~ëÉëK= qÜÉ= éÉêçåÉ~ä= åÉêîÉ= áë= íÜÉ= ãçëíÅçããçåäó= ~ÑÑÉÅíÉÇ= åÉêîÉ= ìåáä~íÉê~ääó= çê= Äáä~íÉê~ääóI= ïÜáäëí= áå= íÜÉ= ìééÉêÉñíêÉãáíáÉëI=íÜÉ=ìäå~ê=åÉêîÉ=áë=íÜÉ=ãçëí=Åçããçåäó=~ÑÑÉÅíÉÇK=

få= íóéáÅ~ä=Å~ëÉëI= íÜÉ=çåëÉí=çÑ= íÜÉ=åÉìêçé~íÜó= áë=~Äêìéí=~åÇ=íÜÉ=ÇÉÑáÅáíëÉîÉêÉI=Äìí=~=é~êíá~ä=ÇÉÑáÅáí=áå=~=åÉêîÉ=íÉêêáíçêó=áë=çÑíÉå=çÄëÉêîÉÇK=^=ëäçïäóéêçÖêÉëëáîÉ=ÅçìêëÉ=áë=ÑêÉèìÉåíäó=ëÉÉå=áå=íÜÉ=ÉäÇÉêäóK=

oÉÅçîÉêó=Ñêçã=ãçíçê=ÇÉÑáÅáí=ÇìÉ=íç=áëÅÜ~ÉãáÅ=åÉìêçé~íÜó=í~âÉë=ãçåíÜëIÄÉÅ~ìëÉ=çÑ=íÜÉ=~ñçå~ä=é~ííÉêå=çÑ=åÉêîÉ=Ç~ã~ÖÉK=oÉëáÇì~ä=é~áå=áë=Åçããçå~åÇ= ã~ó= ÄÉ= ÇáÑÑáÅìäí= íç= ÇáÑÑÉêÉåíá~íÉ= Ñêçã= êÉä~éëÉë= çÑ= íÜÉ= åÉìêçé~íÜóK^ìíçåçãáÅ=ÇóëÑìåÅíáçå=áë=ìåÅçããçåK

bäÉÅíêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= EÉäÉÅíêçãóçÖê~éÜó= xbjdz= ~åÇ= åÉêîÉÅçåÇìÅíáçå=íáãÉ=xk`qzF=ëÜçï=ÉîáÇÉåÅÉ=çÑ=~å=~ñçå~ä=åÉìêçé~íÜóK

Chapter 6 Vasculitic neuropathies

69


Demonstration of NA in nerve and muscle biopsy specimens

qÜÉ= Çá~Öåçëáë= çÑ= k^= åÉÉÇë= ÜáëíçäçÖáÅ~ä= ÅçåÑáêã~íáçåI= ïÜáÅÜ= Å~åëçãÉíáãÉë=ÄÉ=~ÅÜáÉîÉÇ=Äó=ÄáçéëóáåÖ=~=ëéÉÅáÑáÅ=ëâáå= äÉëáçåK= fÑ=åçíI=åÉêîÉ~åÇLçê=ãìëÅäÉ=ÄáçéëáÉë=Å~å=ÄÉ=çÄí~áåÉÇ= áå= íÜÉ=ëÉ~êÅÜ= Ñçê=ÅÜ~ê~ÅíÉêáëíáÅäÉëáçåë=çÑ=ãìëÅìä~ê=çê=ÉéáåÉìêá~ä=~êíÉêáÉëK=fí=áë=áãéçëëáÄäÉ=íç=íÉää=Üçï=çÑíÉåíÜÉ= Çá~Öåçëáë= çÑ= k^= Å~ååçí= ÄÉ= ~ÅÜáÉîÉÇ= ÜáëíçäçÖáÅ~ääóK= kçí= ÑáåÇáåÖÉîáÇÉåÅÉ=çÑ=î~ëÅìäáíáë=áå=íÜÉ=åÉêîÉ=Äáçéëó=ëéÉÅáãÉå=ÇçÉë=åçí=êìäÉ=çìí=íÜÉÇá~Öåçëáë=çÑ=k^=~ë=íÜÉêÉ=áë=~=ÖÉåÉê~ä=ÄÉäáÉÑ=íÜ~í=íÜÉ=Ñ~äëÉJåÉÖ~íáîÉ=ê~íÉ=ÑçêÄáçéëó=áë=ëáÖåáÑáÅ~åíK

tÜÉå=ÅÜ~ê~ÅíÉêáëíáÅ= äÉëáçåë=~êÉ=åçí= ÑçìåÇ=çå= íÜÉ= Ñáêëí=ëÉÅíáçåëI=ëÉêá~äëÉÅíáçåë= çÑ= íÜÉ= Äáçéëó= ëéÉÅáãÉåë= ãìëí= ÄÉ= ëíìÇáÉÇ= ÄÉÅ~ìëÉ= k^= áëëÉÖãÉåí~äI= ~åÇ=ÅÜ~ê~ÅíÉêáëíáÅ= äÉëáçåë=ã~ó=ÄÉ=éêÉëÉåí=çîÉê= ëÉÖãÉåíë=çÑíÜÉ=~êíÉêáÉë=~ë=ëÜçêí=~ë=RMμã= áå=çìê=ÉñéÉêáÉåÅÉK=qÜÉ=óáÉäÇ=çÑ=~=éçëáíáîÉÄáçéëó=ëíìÇáÉÇ=çå=ëÉêá~ä=ëÉÅíáçåë=áåÅêÉ~ëÉÇ=Äó=PSB=Ñçê=ãìëÅäÉ=ëéÉÅáãÉåë~åÇ=Äó=RPB=Ñçê=åÉêîÉ=ëéÉÅáãÉåë=PK=

Lesions of nerve fibres — ischaemic neuropathy

kÉêîÉ=áëÅÜ~Éãá~I=~ë=çÄëÉêîÉÇ=áå=î~ëÅìäáíáÅ=åÉìêçé~íÜóI=~äï~óë=áåÇìÅÉë~ÅìíÉ= ~ñçå~ä= ÇÉÖÉåÉê~íáçåK= ^ëóããÉíêó= çÑ= äÉëáçåë= ÄÉíïÉÉå= ~åÇ= ïáíÜáåÑ~ëÅáÅäÉë= áë=~äëç=Åçããçå= áå=î~ëÅìäáíáÅ=åÉìêçé~íÜóK=pçãÉíáãÉë=~ñçå= äçëëéêÉÇçãáå~íÉë= áå= íÜÉ= ÅÉåíêçÑ~ëÅáÅìä~ê= ~êÉ~= EãáÇÇäÉ= çÑ= íÜÉ= åÉêîÉ= ÄìåÇäÉFIïÜáÅÜ=áë=~äëç=ëìÖÖÉëíáîÉ=çÑ=~å=áëÅÜ~ÉãáÅ=çêáÖáåK=

Clinical aspects

Classical polyarteritis nodosa

k^= çÑ= íÜÉ= éçäó~êíÉêáíáë= åçÇçë~= Em^kF= íóéÉ= áë= íÜÉ= ãçëí= ÜçãçÖÉåÉçìëÉåíáíóK=k^=çÅÅìêë=ÇÉ=åçîç áå=m^kI=çê=~ë=~=ëÉÅçåÇ~êó=ÑÉ~íìêÉ=áå=ÇáëÉ~ëÉëëìÅÜ= ~ë= êÜÉìã~íçáÇ= ~êíÜêáíáë= ~åÇI= çÅÅ~ëáçå~ääóI= áå= ëóëíÉãáÅ= äìéìëÉêóíÜÉã~íçëìëK=qÜÉ=ÅçåëÉèìÉåÅÉë=çÑ=î~ëÅìä~ê=áåÑä~ãã~íáçå=~åÇ=çÅÅäìëáçåÇÉéÉåÇ= çå= íÜÉ= ëáòÉ= ~åÇ= åìãÄÉê= çÑ= ÄäççÇ= îÉëëÉäë= ~ÑÑÉÅíÉÇK= `äáåáÅ~ä


70


åÉìêçé~íÜó=çÅÅìêë= áå=RMJTRB=çÑ=é~íáÉåíë=ïáíÜ=ëóëíÉãáÅ=î~ëÅìäáíáë=çÑ= íÜÉm^k=ÖêçìéK

få=çåÉ=çÑ=çìê=ëíìÇáÉë=ïÉ=ÑçìåÇ=íÜ~í=Åä~ëëáÅ=m^k=~ÑÑÉÅíÉÇ=OQB=çÑ=çìêé~íáÉåíë=PK= få=íÜÉëÉ=é~íáÉåíëI=ãìäíáëóëíÉã=áåîçäîÉãÉåí=ï~ë=éêÉëÉåí=ïáíÜÅìí~åÉçìë= î~ëÅìäáíáë= ~ë= íÜÉ= ãçëí= Åçããçå= åçåJåÉìêçäçÖáÅ~äã~åáÑÉëí~íáçåK= péÉÅáÑáÅ= ëâáå= áåîçäîÉãÉåí= áåÅäìÇÉë= äáîÉÇç= êÉíáÅìä~êáëIÅìí~åÉçìë=åÉÅêçëáë=~åÇ=åçÇìäÉëK=kçåJëéÉÅáÑáÅ=çÉÇÉã~I=ìëì~ääó=~ÑÑÉÅíáåÖçåÉ= äáãÄ= ÉñíêÉãáíóI= çÑíÉå= éêÉÅÉÇÉë= íÜÉ= çåëÉí= çÑ= åÉìêçé~íÜóK= oÉå~äáåîçäîÉãÉåí=ï~ë=çÄëÉêîÉÇ=áå=VB=~åÇ=~ëíÜã~=áå=TB=çÑ=çìê=é~íáÉåíëK

Churg and Strauss variant of polyarteritis nodosa

`ÜìêÖJpíê~ìëë= ëóåÇêçãÉ= ~ééäáÉë= íç= ÇáëëÉãáå~íÉÇ= åÉÅêçíáëáåÖî~ëÅìäáíáë=çÅÅìêêáåÖ=~ãçåÖ=~ëíÜã~íáÅ=é~íáÉåíëI=ïáíÜ= ÑÉîÉêI= ÉçëáåçéÜáäá~I~åÇ=~=Ñìäãáå~åí=ãìäíáëóëíÉã=ÇáëÉ~ëÉ=ïáíÜ=~=é~íÜçäçÖó=çÑ=kÎ=ÉçëáåçéÜáäáÅáåÑáäíê~íáçå=~åÇ=Éñíê~î~ëÅìä~ê=Öê~åìäçã~ëK=

qÜÉ= î~ëÅìä~ê= ~åÇ= åÉêîÉ= äÉëáçåë= çÄëÉêîÉÇ= áå= åÉêîÉ= ÄáçéëáÉë= Ñêçãé~íáÉåíë= ïáíÜ= íÜáë= ëóåÇêçãÉ= ~êÉ= ëáãáä~ê= íç= íÜçëÉ= çÄëÉêîÉÇ= áå= m^kKmÉêáåìÅäÉ~ê=~åíáJåÉìíêçéÜáä=Åóíçéä~ëãáÅ=~åíáÄçÇáÉë=Eé^k`^ëF=ëÉÉã=íç=ÄÉÅçããçåäó=ÑçìåÇ=áå=íÜáë=ëóåÇêçãÉK

Necrotising arteritis and neuropathy in patients withrheumatoid arthritis EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çîÉêäÉ~ÑF

qÜÉ= çÅÅìêêÉåÅÉ= çÑ= k^= áå= íÜÉ= ÅçåíÉñí= çÑ= êÜÉìã~íçáÇ= ~êíÜêáíáë= áëÅä~ëëáÅ~ääó=~ëëçÅá~íÉÇ=ïáíÜ=~=éççê=çìíÅçãÉK=få=çìê=ëíìÇó=çÑ=PO=é~íáÉåíëïáíÜ= êÜÉìã~íçáÇ= ~êíÜêáíáë= ~åÇ= åÉìêçé~íÜó= ÇìÉ= íç= ÜáëíçäçÖáÅ~ääó= éêçîÉååÉÅêçíáëáåÖ=î~ëÅìäáíáë=áå=ãìëÅäÉ=~åÇLçê=åÉêîÉ=Äáçéëó=ëéÉÅáãÉåëI=NR=Ü~Ç~=ëÉåëçêó=~åÇ=ãçíçê=åÉìêçé~íÜóI=íÜÉ=çíÜÉêë=~=éìêÉäó=ëÉåëçêó=åÉìêçé~íÜóKqïç=íÜáêÇë=çÑ=íÜÉ=é~íáÉåíë=Ü~Ç=~=ãìäíáÑçÅ~ä=é~ííÉêå=çÑ=åÉìêçäçÖáÅ~ä=ÇÉÑáÅáíIíÜÉ=çíÜÉê=íÜáêÇ=ã~åáÑÉëíÉÇ=Çáëí~ä=ëóããÉíêáÅ~ä=ëÉåëçêó=åÉìêçé~íÜó=QK=


71


Granulomatosis with polyangiitis

dê~åìäçã~íçëáë= ïáíÜ= éçäó~åÖááíáë= EdmF= áë= ÅÜ~ê~ÅíÉêáëÉÇ= ÄóÖê~åìäçã~íçìë=î~ëÅìäáíáë=çÑ=íÜÉ=ìééÉê=~åÇ=äçïÉê=êÉëéáê~íçêó=íê~Åí=ïáíÜ=çêïáíÜçìí=ÖäçãÉêìäçåÉéÜêáíáëK=mÉêáéÜÉê~ä=åÉìêçé~íÜó=Ü~ë=ÄÉÉå=çÄëÉêîÉÇ= áåORB= çÑ= é~íáÉåíë= ïáíÜ= dm= RK= mÉêáéÜÉê~ä= åÉìêçé~íÜó= ëÉäÇçã= áë= íÜÉ= Ñáêëíã~åáÑÉëí~íáçå=çÑ=íÜÉ=ÇáëÉ~ëÉK=få=~=êÉîáÉï=çÑ=POQ=é~íáÉåíë=ïáíÜ=dmI=NMV=Ü~ÇåÉìêçäçÖáÅ~ä= ã~åáÑÉëí~íáçåë= ~í= ëçãÉ= ëí~ÖÉK= cáÑíóJíÜêÉÉ= é~íáÉåíë= Ü~Ç


72

Vasculitic neuropathy associated with rheumatoid arthritis

Cheryl is a 43-year-old right-handed former lunchtime supervisor who presented

with joint problems in her early thirties. She had been followed up in the

rheumatology clinics and started developing painful and numb feet. She had initially

been treated with non-steroidal anti-inflammatories, but for a number of years had

been treated with intermittent courses of prednisolone and had also been treated

with disease- modifying drugs including hydroxychloroquine and sulfasalazine. By

the time she arrived at the neurology clinic she was taking 15mg of prednisolone a

day and cyclophosphamide 1mg a day.

Examination revealed advanced rheumatoid changes in the joints of the hands and

feet. Power was difficult to ascertain due to the joint deformity and pain, but was

probably near normal. The sensory examination revealed a loss of pain perception to

the wrists and 7cm above the ankles, with a similar loss of temperature perception.

Vibration sensation was lost in the toes, but was present in the hands and ankles.

Joint position sense was lost in her toes, but was present at the ankles, and normal

in the hands. The deep tendon reflexes were generally diminished and lost at the

ankles. The plantar responses were difficult to interpret and her gait was wide-based

and unsteady.

She was regularly reviewed in the neurology clinic and the waxing and waning of her

neuropathy, together with her inflammatory markers and rheumatological

examination, informed her rheumatologist, as to the dose of steroid and changes in

her disease-modifying antirheumatic drugs.


éÉêáéÜÉê~ä= åÉìêçé~íÜóI= ïÜáÅÜ= ï~ë= ãìäíáÑçÅ~ä= áå= QOK= `ê~åá~ä= åÉêîÉë= ïÉêÉáåîçäîÉÇ=áå=ONLNMVI=çéÜíÜ~äãçéäÉÖá~=áå=NSLONK=qÜÉ=ãÉ~å=áåíÉêî~ä=ÄÉíïÉÉåíÜÉ=çåëÉí=çÑ=dm=~åÇ=åÉìêçäçÖáÅ~ä=ã~åáÑÉëí~íáçåë=ï~ë=UKQ=ãçåíÜë=RK

Necrotising arteritis and isolated neuropathy

få=~= ä~êÖÉ=éêçéçêíáçå=çÑ=é~íáÉåíë=ïáíÜ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=êÉä~íÉÇ=íçåÉÅêçíáëáåÖ=~êíÉêáíáë=ëÉÉå=áå=åÉìêçäçÖóI=íÜÉêÉ=áë=åç=çêÖ~å=ÅäáåáÅ~ääó=áåîçäîÉÇçíÜÉê=íÜ~å=íÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=EmkpF=SK=qÜáë=Öêçìé=çÑ=é~íáÉåíë~ÅÅçìåíÉÇ= Ñçê= ONB= çÑ= é~íáÉåíë= ëÉÉå= ïáíÜ= ÇÉãçåëíê~íÉÇ= k^= ~åÇåÉìêçé~íÜó= áå=çìê= ëÉêîáÅÉK= få= ëìÅÜ=é~íáÉåíë=éÉêáéÜÉê~ä= åÉìêçé~íÜó= áë= íÜÉéêÉëÉåíáåÖ=~åÇ=çåäó=ã~åáÑÉëí~íáçå=çÑ=åÉÅêçíáëáåÖ=~êíÉêáíáëK=

páäÉåí=áåîçäîÉãÉåí=çÑ=çíÜÉê=çêÖ~åë=áë=Åçããçå=áå=ëìÅÜ=é~íáÉåíëI=ÜçïÉîÉêI~ë=ëÜçïå=Äó= íÜÉ= ÑêÉèìÉåí= ÑáåÇáåÖ=çÑ=åÉÅêçíáëáåÖ=~êíÉêáíáë= áå=ãìëÅäÉ=ÄáçéëóëéÉÅáãÉåëK=qÜìëI=áí=ëÉÉãë=ãçêÉ=~ééêçéêá~íÉ=íç=ÅçåëáÇÉê=íÜ~í=é~íáÉåíë=ïáíÜ~éé~êÉåíäó= áëçä~íÉÇ= î~ëÅìäáíáÅ= åÉìêçé~íÜó= ~êÉ= ~ÑÑÉÅíÉÇ= Äó= ~= äçïJÖê~ÇÉî~ëÅìäáíáëI= ëóãéíçã~íáÅ= áå= åÉêîÉë= çåäóI= ÄÉÅ~ìëÉ= íáëëìÉ= áëÅÜ~Éãá~= áë= ãçêÉäáâÉäó=íç=ÄÉ=ëóãéíçã~íáÅ=áå=åÉêîÉë=íÜ~å=áå=ãçëí=çíÜÉê=çêÖ~åëK=

cêçã= ~= åÉìêçäçÖáÅ~ä= ëí~åÇéçáåíI= ~ééêçñáã~íÉäó= çåÉ= ÑçìêíÜ= çÑ= é~íáÉåíëéêÉëÉåíÉÇ=ïáíÜ=~=Çáëí~ä=ëóããÉíêáÅ~ä=ëÉåëçêó=çê=ëÉåëçêáãçíçê=åÉìêçé~íÜóI~åÇ=íÜÉ=Çá~Öåçëáë=çÑ=k^=Ü~Ç=ëÉäÇçã=ÄÉÉå=ÅçåëáÇÉêÉÇ=ÄÉÑçêÉ=íÜÉ=êÉëìäíëçÑ= íÜÉ= åÉêîÉ= ~åÇ= ãìëÅäÉ= ÄáçéëáÉëK= aìêáåÖ= ÑçääçïJìéI= çåÉ= íÜáêÇ= çÑ= íÜÉé~íáÉåíë=áå=íÜáë=Öêçìé=ÇÉîÉäçéÉÇ=ëóëíÉãáÅ=ã~åáÑÉëí~íáçåëK=^=êÉä~éëÉ=çÑ=íÜÉåÉìêçé~íÜó=çÅÅìêêÉÇ=áå=ORB=çÑ=é~íáÉåíëK

Vasculitic neuropathy in the elderly

kÉìêçé~íÜó=áë=~å=áãéçêí~åí=Ñ~Åíçê=çÑ=Çáë~Äáäáíó=áå=íÜÉ=ÉäÇÉêäóK=få=~=ëÉêáÉëçÑ=NMM=é~íáÉåíë=çîÉê=SR=óÉ~êë=çÑ=~ÖÉ=êÉÑÉêêÉÇ=Ñçê=~=Çáë~ÄäáåÖ=åÉìêçé~íÜóIïÉ= ÑçìåÇ= íÜ~í=OPB=Ü~Ç=î~ëÅìäáíáÅ=åÉìêçé~íÜó=ïáíÜ=åÉÅêçíáëáåÖ=î~ëÅìäáíáëÇÉãçåëíê~íÉÇ= áå= íÜÉ= åÉêîÉ= ~åÇLçê= ãìëÅäÉ= Äáçéëó= ëéÉÅáãÉåEëFK= jçëíé~íáÉåíë= êÉëéçåÇÉÇ= ïÉää= íç= íêÉ~íãÉåíK= få= íÜáë= Öêçìé= çÑ= é~íáÉåíëI= íÜÉåÉìêçé~íÜó=çÅÅìêêÉÇ=áå=íÜÉ=ÅçåíÉñí=çÑ=~=ãìäíáëóëíÉã=ÇáëçêÇÉê=áå=íïç=íÜáêÇëçÑ=é~íáÉåíëI=~åÇ=~ë=~å=áëçä~íÉÇ=åÉìêçé~íÜó=áå=NM=é~íáÉåíë=TK


73


Secondary vasculitic neuropathy

s~ëÅìäáíáë=Å~å=ÅçãéäáÅ~íÉ=íÜÉ=ÅçìêëÉ=çÑ=ÇáÑÑÉêÉåí=ÅçåÇáíáçåë=áåÅäìÇáåÖ=~åáåÑä~ãã~íçêó=áããìåÉ=êÉ~Åíáçå=íêáÖÖÉêÉÇ=Äó=~å=áåÑÉÅíáîÉ=~ÖÉåíI=~=ÇÉä~óÉÇJíóéÉ=ÜóéÉêëÉåëáíáîáíó=êÉ~ÅíáçåI=Çá~ÄÉíÉë=ãÉääáíìë=çê=ã~äáÖå~åÅóK=

Necrotising vasculitis and viral infection

póãéíçã~íáÅ= îáê~ä= áåÑÉÅíáçåI= áåÅäìÇáåÖ= efs= áåÑÉÅíáçåI= çééçêíìåáëíáÅáåÑÉÅíáçå= ïáíÜ= ÅóíçãÉÖ~äçîáêìë= ~í= ~= ä~íÉ= ëí~ÖÉ= çÑ= efs= áåÑÉÅíáçåI= ÅÜêçåáÅÜÉé~íáíáë=_=~åÇ=`=îáêìëÉëI=~åÇ=Üìã~å=qJäóãéÜçíêçéáÅ=îáêìë=íóéÉ=f=EeqisJNFáåÑÉÅíáçå= Å~å= ÄÉ= ~ëëçÅá~íÉÇ= ïáíÜ= åÉìêçé~íÜó= ~åÇ= åÉÅêçíáëáåÖ= î~ëÅìäáíáëKqÜÉ=ÅäáåáÅ~ä=é~ííÉêå=çÑ=î~ëÅìäáíáÅ=åÉìêçé~íÜó=çÄëÉêîÉÇ=áå=~ëëçÅá~íáçå=ïáíÜÜÉé~íáíáë=_=~åÇ=`=îáê~ä= áåÑÉÅíáçå= áë=ëáãáä~ê= íç=íÜ~í=çÑ=éêáã~êó=î~ëÅìäáíáëK= fåíÜáë=ëÉííáåÖ=íÜÉ=åÉìêçé~íÜó=Å~å=~äëç=ÄÉ=~ëëçÅá~íÉÇ=ïáíÜ=ÅêóçÖäçÄìäáå~Éãá~IïÜáÅÜ=çÅÅìêë=áå=é~íáÉåíë=ïáíÜ=ã~äáÖå~åí=Ö~ããçé~íÜó=~ë=ïÉääK=

Sarcoidosis

qÜÉ=çÅÅìêêÉåÅÉ=çÑ=åÉÅêçíáëáåÖ=î~ëÅìäáíáë=áå=~ëëçÅá~íáçå=ïáíÜ=Öê~åìäçã~ëÉÅçåÇ~êó= íç= íÜÉ= ÇÉä~óÉÇ= ÜóéÉêëÉåëáíáîáíó= êÉ~Åíáçå= íÜ~í= ÅÜ~ê~ÅíÉêáëÉëë~êÅçáÇçëáëI=ëíêÉëëÉë=íÜÉ=åÉÉÇ=íç=ÉñÅäìÇÉ=çíÜÉê=Å~ìëÉë=çÑ=Öê~åìäçã~íçìë~åÖááíáëI=ÉëéÉÅá~ääó=dmI=ïÜáÅÜ=ÇáÑÑÉêë= Ñêçã=ë~êÅçáÇçëáë=ÄçíÜ=ÅäáåáÅ~ääó=~åÇÄáçäçÖáÅ~ääóK

Vasculitis in diabetic neuropathy

fåÑä~ãã~íçêó= äÉëáçåë= çÑ= åÉêîÉ= ÄäççÇ= îÉëëÉäë= éä~ó= ~= êçäÉ= áå= ÑçÅ~ä= ~åÇãìäíáÑçÅ~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜóK=EmäÉ~ëÉ=êÉÑÉê=íç=`Ü~éíÉê=U=Ô=aá~ÄÉíáÅ=~åÇìê~ÉãáÅ=åÉìêçé~íÜáÉë=Ô=Ñçê=ãçêÉ=ÇÉí~áäKF

Vasculitis in malignancy

m~ê~åÉçéä~ëíáÅ=åÉìêçé~íÜáÉë=~êÉ=ëçãÉíáãÉë=~ëëçÅá~íÉÇ=ïáíÜ=î~ëÅìäáíáëK


74


EmäÉ~ëÉ= êÉÑÉê= íç=`Ü~éíÉê=V=Ô=kÉìêçé~íÜáÉë= áå=é~íáÉåíë=ïáíÜ=ãçåçÅäçå~äÖ~ããçé~íÜó=~åÇ=ã~äáÖå~åÅó=Ô=Ñçê=ãçêÉ=ÇÉí~áäKF

Treatment

qêÉ~íãÉåí= áë= Ä~ëÉÇ= çå= ~Çãáåáëíê~íáçå= çÑ= ÅçêíáÅçëíÉêçáÇë= ~åÇáããìåçëìééêÉëëáîÉ=ÇêìÖëK=mêÉÇåáëçåÉ=ëÜçìäÇ=ÄÉ=ëí~êíÉÇ=~í=NãÖLâÖLÇ~óKpáãìäí~åÉçìë=íêÉ~íãÉåí=ïáíÜ=ÅóÅäçéÜçëéÜ~ãáÇÉI=OãÖLâÖLÇ~óI=ã~ó=ÜÉäé=íçêÉÇìÅÉ= íÜÉ= ÇçëÉ= çÑ= ÅçêíáÅçëíÉêçáÇëK= få= Å~ëÉë= ïáíÜ= ëÉîÉêÉ= ÖÉåÉê~äã~åáÑÉëí~íáçåëI=éìäëÉë=çÑ=áåíê~îÉåçìë=éêÉÇåáëçäçåÉ=ã~ó=ÜÉäéK=fí=áë=ÇáÑÑáÅìäííç=ëíáéìä~íÉ=Üçï=äçåÖ=íÜáë=íêÉ~íãÉåí=ëÜçìäÇ=ÄÉ=ã~áåí~áåÉÇ=~í=Ñìää=ÇçëÉK=qÜáëÇÉéÉåÇë= çå= íÜÉ= êÉëéçåëÉ= íç= íêÉ~íãÉåíI= çå= íÜÉ= ÅçìêëÉ= ~åÇ= Ñçêã= çÑ= íÜÉÇáëÉ~ëÉI= ~åÇ= çå= íÜÉ= íçäÉê~åÅÉ= çÑ= íÜÉ= íêÉ~íãÉåíK= ^= Ñìää= ëíÉêçáÇ= ÇçëÉ= áëìëì~ääó= ÖáîÉå= Ñçê= ~ééêçñáã~íÉäó= SJU= ïÉÉâë= ~åÇ= áë= ëìÄëÉèìÉåíäó= í~éÉêÉÇçîÉê= ~= éÉêáçÇ= çÑ= SJNM= ãçåíÜë= çê= ãçêÉK= fí= áë= åÉÅÉëë~êó= íç= Åçåíêçä= íÜÉÉêóíÜêçÅóíÉ=ëÉÇáãÉåí~íáçå=ê~íÉ=EbpoF=~åÇ=çíÜÉê=ã~êâÉêë=çÑ=ÇáëÉ~ëÉ=~ÅíáîáíóáåÅäìÇáåÖ= `JêÉ~ÅíáîÉ= éêçíÉáå= EòmF= ~åÇ= ÉçëáåçéÜáäáÅ= éçäóãçêéÜçåìÅäÉ~êÅÉää=äÉîÉäëI=ïÜáÅÜ=ã~ó=î~êó=Ñêçã=çåÉ=é~íáÉåí=íç=~åçíÜÉêI=~åÇ=íç=~Çàìëí=íÜÉÇçëÉë= çÑ= éêÉÇåáëçåÉ= ~ÅÅçêÇáåÖäóK= j~åó= é~íáÉåíë= êÉä~éëÉ= ÉáíÜÉê= ÇìêáåÖêÉÇìÅíáçå=çÑ=ëíÉêçáÇ=íÜÉê~éó=çê=~ÑíÉê= íêÉ~íãÉåí=Ü~ë=ÄÉÉå=ëíçééÉÇK= få=çìêÉñéÉêáÉåÅÉI=ìé=íç=Ü~äÑ=çÑ=é~íáÉåíë=êÉä~éëÉ=ÇìêáåÖ=í~éÉêáåÖ=çÑ=éêÉÇåáëçåÉ=çê~ÑíÉê= íêÉ~íãÉåí= Ü~ë= ÄÉÉå= ëíçééÉÇK= `~êÉÑìä= ÑçääçïJìé= çÑ= é~íáÉåíë= áëã~åÇ~íçêóK

få=Å~ëÉë=ïáíÜçìí=ÅäáåáÅ~ä=ÉîáÇÉåÅÉ=çÑ=éçäóëóëíÉãáÅ=áåîçäîÉãÉåíI=çê=ïáíÜ~=åçêã~ä=bpoI=~åÇ=áå=ÉäÇÉêäó=é~íáÉåíëI=íÜÉ=ÄÉåÉÑáí=çÑ=éêçäçåÖÉÇ=íêÉ~íãÉåíïáíÜ=ÜáÖÜ=ÇçëÉë=çÑ=ëíÉêçáÇë=áë=äÉëë=ÅÉêí~áåK=fåÇçãÉíÜ~Åáå=ENMMãÖLÇ~óF=ã~óÄÉ= ~ÇÇÉÇ= íç= ëíÉêçáÇë= ïÜÉå= ÑÉîÉê= éÉêëáëíë= çê= ïÜÉå= íÜÉ= bpo= êÉã~áåë~Äåçêã~ä=áå=ëéáíÉ=çÑ=ä~êÖÉ=ÇçëÉë=çÑ=ëíÉêçáÇëK

tÜáäëí= Éî~äì~íáåÖ= íÜÉ= ÉÑÑáÅ~Åó= çÑ= íÜÉ= íêÉ~íãÉåí= çÑ= î~ëÅìäáíáÅåÉìêçé~íÜáÉëI=áí=ãìëí=ÄÉ=êÉãÉãÄÉêÉÇ=íÜ~í=íÜÉêÉ=áë=~=ïáÇÉ=ê~åÖÉ=çÑ=ÉîçäîáåÖãçÇ~äáíáÉë= áå= kÎ= ~åÇ= íÜ~í= ëéçåí~åÉçìë= êÉãáëëáçå= çÑ= ëÉîÉê~ä= óÉ~êëÛÇìê~íáçå= Å~å= çÅÅìêK= pÉåëçêáãçíçê= ÇÉÑáÅáí= êÉëìäíáåÖ= Ñêçã= åÉêîÉ= áëÅÜ~Éãá~ïáää= í~âÉ= ãçåíÜë= íç= êÉÅçîÉêI= ÄÉÅ~ìëÉ= çÑ= íÜÉ= ìåÇÉêäóáåÖ= ~ñçå~ä= äÉëáçåëKjçíçê= êÉÅçîÉêó= ïáää= ÄÉ= ÜÉäéÉÇ= Äó= éÜóëáçíÜÉê~éóI= Äìí= êÉëáÇì~ä= é~áå= áëÅçããçåK


75


References

NK c~äâ=ogI=dêçëë=tiI=dìáääÉîáå=iI=Éí=~äK=oÜÉìã~íáëãK=dê~åìäçã~íçëáë=ïáíÜ=éçäó~åÖááíáë

EtÉÖÉåÉêÛëFW=~å=~äíÉêå~íáîÉ=å~ãÉ=Ñçê=tÉÖÉåÉêÛë=Öê~åìäçã~íçëáëK=^êíÜêáíáë=oÜÉìã OMNNX

SPW=USPJQK

OK `ÜìêÖ=gI=píê~ìëë=iK=^ääÉêÖáÅ=Öê~åìäçã~íçëáëI=~ääÉêÖáÅ=~åÖááíáëI=~åÇ=éÉêá~êíÉêáíáë=åçÇçë~K

^ã=g=m~íÜçä NVRNX=OTW=OTTJPMNK=

PK p~áÇ=dI=i~Åêçáñ=Ì=cìàáãìê~=eI=Éí=~äK=qÜÉ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=çÑ=åÉÅêçíáòáåÖ=~êíÉêáíáëW

~=ÅäáåáÅçé~íÜçäçÖáÅ=ëíìÇóK=^åå=kÉìêçä NVUUX=OPW=QSNJRK

QK sê~åÅâÉå=^cI=p~áÇ=dK=s~ëÅìäáíáÅ=åÉìêçé~íÜóK=e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=QSPJUPK

RK káëÜáåç= eI= oìÄáåç= cÎ= aÉoÉãÉÉ= oÎ= Éí= ~äK= kÉìêçäçÖáÅ~ä= áåîçäîÉãÉåí= áå= tÉÖÉåÉêÛë

Öê~åìäçã~íçëáëW=~å=~å~äóëáë=çÑ=POQ=ÅçåëÉÅìíáîÉ=é~íáÉåíë=~í=íÜÉ=j~óç=`äáåáÅK=^åå=kÉìêçä

NVVPX=PPW=QJVK

SK aóÅâ=mgI=_ÉåëíÉ~Ç=qgI=`çåå=aiI=Éí=~äK=kçåëóëíÉãáÅ=î~ëÅìäáíáÅ=åÉìêçé~íÜóK=_ê~áå=NVUTX

NNMW=UQPJRQK=

TK `Üá~= iI= cÉêå~åÇÉò= Î= i~Åêçáñ= Ì= Éí= ~äK= `çåíêáÄìíáçå= çÑ= åÉêîÉ= Äáçéëó= ÑáåÇáåÖë= íç= íÜÉ

Çá~Öåçëáë= çÑ= Çáë~ÄäáåÖ= åÉìêçé~íÜó= áå= íÜÉ= ÉäÇÉêäóW= ~= êÉíêçëéÉÅíáîÉ= êÉîáÉï= çÑ= NMM

ÅçåëÉÅìíáîÉ=é~íáÉåíëK=_ê~áå=NVVSX=NNVW=NMVNJUK


76

√ Vasculitic neuropathy is typically a multifocal painful, motor and sensory

neuropathy of subacute onset.

√ Inflammation and necrosis of nerve blood vessels cause ischaemic nerve

lesions.

√ Vasculitis can be primary as in polyarteritis nodosa or secondary to systemic

disorders.

√ Nerve and muscle biopsies are needed for diagnosis in most cases.

√ In a large proportion of patients neuropathy is the only manifestation of

vasculitis.

√ Treatment rests on corticosteroid and immunosuppressive drugs.

Ü Key points


77

Infectious neuropathies

Chapter 7

Overview

This chapter deals with the different infective processes that may affect the

peripheral nervous system. Leprous neuropathy, with its two patterns —

lepromatous and tuberculoid — still affects several million people, mainly in

intertropical developing countries. Mycobacterium leprae is predominantly found

in nerve trunks and skin. Lyme disease due to infection by Borrelia burgdorferi is

transmitted by a tick bite. It induces a benign meningoradiculitis lasting a few

weeks. HIV infection can induce a variety of peripheral neuropathies plus

cytomegalovirus neuropathy that occurs at the end stage of HIV infection.

Introduction

fåÑä~ãã~íçêó= åÉìêçé~íÜáÉë= Å~å= ÄÉ= ÇÉÑáåÉÇ= ~ë= åÉìêçé~íÜáÉë= áå= ïÜáÅÜäÉëáçåë= çÑ= éÉêáéÜÉê~ä= åÉêîÉ= ÑáÄêÉë= ~êÉ= ~ëëçÅá~íÉÇ= ïáíÜ= áåÑä~ãã~íçêóáåÑáäíê~íáçåK=få=ãçëí=áåëí~åÅÉëI=áåÑä~ãã~íçêó=åÉìêçé~íÜáÉë=Ñçääçï=áåÑÉÅíáçå=çÑíÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=Äó=îáêìëÉëI=Ä~ÅíÉêá~=çê=é~ê~ëáíÉëK=qÜáë=Öêçìéáë= íÜÉ= ä~êÖÉëí= Öêçìé= çÑ= åÉìêçé~íÜáÉë= áå= íÜÉ= ïçêäÇK= få= íÜáë= ÅçåíÉñí= åÉêîÉäÉëáçåë=Å~å=êÉëìäí=Ñêçã=íÜÉ=áåÑä~ãã~íçêó=êÉ~Åíáçå=áåÇìÅÉÇ=Äó=íÜÉ=áåÑÉÅíáîÉ~ÖÉåí=çê=Ñêçã=íÜÉ=áããìåÉ=êÉ~Åíáçå=çÑ=íÜÉ=é~íáÉåíK


Leprosy

iÉéêçìë= åÉìêçé~íÜóI= ïÜáÅÜ= áë= ÇìÉ= íç= áåÑÉÅíáçå= çÑ= åÉêîÉ= ÅÉääë= ÄójóÅçÄ~ÅíÉêáìã=äÉéê~ÉI=ëíáää=~ÑÑÉÅíë=ãáääáçåë=çÑ=éÉçéäÉ=áå=ã~åó=ÇÉîÉäçéáåÖÅçìåíêáÉëK=qÜÉ=ÅäáåáÅ~ä=~åÇ=é~íÜçäçÖáÅ~ä=ã~åáÑÉëí~íáçåë=~êÉ=ÇÉíÉêãáåÉÇ=ÄóíÜÉ= å~íìê~ä= êÉëáëí~åÅÉ= çÑ= íÜÉ= Üçëí= íç= áåî~ëáçå= çÑ= jóÅçÄ~ÅíÉêáìã= äÉéê~ÉKc~áäìêÉ=çÑ=É~êäó=ÇÉíÉÅíáçå=çÑ=äÉéêçëó=çÑíÉå=äÉ~Çë=íç=ëÉîÉêÉ=Çáë~Äáäáíó=áå=ëéáíÉçÑ= Éê~ÇáÅ~íáçå= çÑ= ãóÅçÄ~ÅíÉêá~= ~í= ~= ä~íÉê= Ç~íÉK= få= íÜÉ= äÉéêçã~íçìë= íóéÉIÄ~Åáääá=~êÉ=É~ëáäó= ÑçìåÇ=áå=íÜÉ=ëâáåI=~åÇ= áå=åÉêîÉ=ÅÉääë= áåÅäìÇáåÖ=pÅÜï~ååÅÉääëI= ÉåÇçíÜÉäá~ä= ÅÉääë= ~åÇ= ã~ÅêçéÜ~ÖÉë= EcáÖìêÉ= NFK= få= íÜÉ= íìÄÉêÅìäçáÇíóéÉI=~=ëíêçåÖ=ÅÉääJãÉÇá~íÉÇ=áããìåÉ=êÉ~Åíáçå=äÉ~Çë=íç=Öê~åìäçã~=Ñçêã~íáçå~åÇ=ÇÉëíêìÅíáçå=çÑ=ÅÉääë=Ü~êÄçìêáåÖ=Ä~Åáääá=~åÇ=åÉáÖÜÄçìêáåÖ=åÉêîÉ=ÑáÄêÉëKfå= ã~åó= Å~ëÉë= íêÉ~íãÉåí= çÑ= é~íáÉåíë= ïáíÜ= ãìäíáÄ~Åáää~êó= äÉéêçëó= áëÅçãéäáÅ~íÉÇ=Äó=êÉîÉêë~ä=êÉ~Åíáçå=~åÇ=ÑìêíÜÉê=åÉêîÉ=Ç~ã~ÖÉ=EcáÖìêÉë=O=~åÇPF=NI=OK


78

Figure 1. Nerve biopsy from a patient with lepromatous

leprous neuropathy to show partial involvement of a nerve

trunk. Four fascicles (arrows) are affected while the other

three appear normal at this magnification. (Bar: 1mm.)


Chapter 7 Infectious neuropathies

79

Figure 2. Nerve biopsy from a patient with a late upgradereversal reaction that occurred more than 20 years aftertreatment of a multibacillary leprous neuropathy. Note thegranulomas close to an epineurial blood vessel (arrow). Theendoneurium is filled with connective tissue with a fewregenerating nerve fibres. (One-micron-thick plastic section.Toluidine blue staining. Bar: 50μm.)

Figure 3. Electron micrograph of a nerve biopsy specimen

from a patient with lepromatous (multibacillary) leprous

neuropathy at an early stage. Note the preservation of many

myelinated and unmyelinated nerve fibres. Bacilli can be seen

in vacuoles in the cytoplasm of some Schwann cells (arrows).

(Uranyl acetate and lead citrate staining. Bar: 1μm.)


Historical and epidemiological aspects

iÉéêçëó=Ü~ë=ÄÉÉå=êÉÅçÖåáëÉÇ=ëáåÅÉ=íÜÉ=~åÅáÉåí=Åáîáäáë~íáçåë=çÑ=`Üáå~IbÖóéí=~åÇ=fåÇá~K=qÜÉ=çäÇÉëí=ÇÉëÅêáéíáçå=çÑ=äÉéêçëó=éêçÄ~Ääó=Ç~íÉë=Ä~Åâ=íçSMM=_K`K=^í= íÜ~í= íáãÉ= áí=ìëÉÇ= íç=ÄÉ=ÅçåëáÇÉêÉÇ=~=ÇáîáåÉ=éìåáëÜãÉåí= ÑçêëáåëI= ~ÅÅçêÇáåÖ= íç= íÜÉ= läÇ= qÉëí~ãÉåíI= ~åÇ= íç= â~êã~= áå= íÜÉ= _ìÇÇÜáëíêÉäáÖáçåK=få=NUTQI=e~åëÉå=áÇÉåíáÑáÉÇ=íÜÉ=Å~ìë~ä=~ÖÉåí=çÑ=íÜáë=áääåÉëë=~í=~=íáãÉïÜÉå=äÉéêçëó=ï~ë=ÉåÇÉãáÅ=áå=kçêï~ó=PK=

pìÄëÉèìÉåí= áãéêçîÉãÉåíë= áå= íÜÉ= íêÉ~íãÉåíI= ã~å~ÖÉãÉåí= ~åÇ= éìÄäáÅÜÉ~äíÜ=~ééêç~ÅÜ=Ü~îÉ=ÅçåíêáÄìíÉÇ=íç=~=åÉ~ê=Éê~ÇáÅ~íáçå=çÑ=íÜÉ=ÇáëÉ~ëÉ=áåáåÇìëíêá~äáëÉÇ=ÅçìåíêáÉëK=vÉí=äÉéêçëó=ëíáää=êÉã~áåë=~ãçåÖ=íÜÉ=Ñáêëí=Å~ìëÉë=ÑçêåÉìêçé~íÜó=áå=íÜÉ=ïçêäÇ=ÉîÉå=íÜçìÖÜ=íÜÉ=ä~íÉëí=Éëíáã~íÉ=çÑ=íÜÉ=åìãÄÉê=çÑäÉéêçëó= Å~ëÉë= ïçêäÇïáÇÉ= áë= RKR= ãáääáçå= EïÜáÅÜ= áë= ~Äçìí= Ü~äÑ= íÜÉ= åìãÄÉêÉëíáã~íÉÇ= áå= íÜÉ= É~êäó= NVUMëFI= ïáíÜ= OJP= ãáääáçå= é~íáÉåíë= Ü~îáåÖ= êÉëáÇì~äÇÉÑçêãáíóK=

iÉéêçëó= áë= éêáã~êáäó= ÑçìåÇ= áå= íêçéáÅ~ä= ~åÇ= ëìÄJíêçéáÅ~ä= ÇÉîÉäçéáåÖÅçìåíêáÉëI= ïáíÜ= ëçãÉ= é~êíë= çÑ= ^ëá~= ~åÇ= ^ÑêáÅ~= Ü~îáåÖ= ~= éêÉî~äÉåÅÉÉñÅÉÉÇáåÖ=NM=éÉê=NMMM=éçéìä~íáçå=~åÇ=ãçêÉ=íÜ~å=MKR=ãáääáçå=åÉï=Å~ëÉëÇÉíÉÅíÉÇ=É~ÅÜ=óÉ~êK=aìêáåÖ=OMMUI=NT=ÅçìåíêáÉë=êÉéçêíÉÇ=ãçêÉ=íÜ~å=NMMMåÉï= Å~ëÉëK= iÉéêçëó= ÅçåíáåìÉë= íç= ÄÉ= ~å= áãéçêí~åí= ÜÉ~äíÜ= éêçÄäÉãïçêäÇïáÇÉ= Äìí= áë= ãçëí= éêÉî~äÉåí= áå= fåÇá~I= _ê~òáäI= fåÇçåÉëá~= ~åÇ= káÖÉêá~KfåÇá~=~ÅÅçìåíë=Ñçê=SQB=çÑ=ïçêäÇïáÇÉ=Å~ëÉë=QK

Pathophysiology

pÉîÉê~ä=ÑÉ~íìêÉë=çÑ=äÉéêçëó=~êÉ=ìåáèìÉ=~åÇ=ëíêáâáåÖäó=ÇáÑÑÉêÉåí=Ñêçã=çíÜÉêáåÑÉÅíáçìë=ÇáëÉ~ëÉë=çÑ=ã~åI=áå=êÉä~íáçå=íç=íÜÉ=å~íìêÉ=çÑ=íÜÉ=áåÑÉÅíáîÉ=~ÖÉåí~åÇ= íç= íÜÉ= áããìåçäçÖáÅ~ä= ëí~íìë= çÑ= íÜÉ= ÜçëíK= jìÅÜ= çÑ= íÜÉ= åÉìêçäçÖáÅ~ä~ëéÉÅíë=çÑ=äÉéêçìë=åÉìêçé~íÜáÉë=Ü~ë=ÄÉÉå=âåçïå=Ñçê=ÇÉÅ~ÇÉëI=óÉí=äÉéêçëóêÉã~áåë=~=ëìÄàÉÅí=çÑ=áåíÉêÉëí=ÄÉÅ~ìëÉ=íÜÉ=Ñçêã=çÑ=äÉéêçëó=ã~áåäó=ÇÉéÉåÇëçå= íÜÉ= áããìåÉ= êÉ~Åíáçå= çÑ= íÜÉ= Üçëí= íç= jóÅçÄ~ÅíÉêáìã= äÉéê~É ~åíáÖÉåëIê~åÖáåÖ= áå= ÉñíêÉãÉë= Ñêçã= íÜÉ= äçïÉëí= ÅÉääJãÉÇá~íÉÇ= áããìåáíó= íçjóÅçÄ~ÅíÉêáìã= äÉéê~ÉI= íÜÉ= äÉéêçã~íçìë= éçäÉI= íç= íÜ~í= çÑ= íÜÉ= ÜáÖÜÉëíáããìåÉ=êÉëéçåëÉI=íÜÉ=íìÄÉêÅìäçáÇ=éçäÉK


80


Mycobacterium leprae

iÉéêçëó= áë= Å~ìëÉÇ= Äó= jóÅçÄ~ÅíÉêáìã= äÉéê~ÉI= ~å= áåíê~ÅÉääìä~ê= Öê~ãJéçëáíáîÉ= ~äÅçÜçäJ~ÅáÇJÑ~ëí= Ä~ÅáääìëK= fí= áë= ãçêéÜçäçÖáÅ~ääó= áåÇáëíáåÖìáëÜ~ÄäÉÑêçã=jóÅçÄ~ÅíÉêáìã=íìÄÉêÅìäçëáëK fí=ÇçÉëåÛí=Öêçï=áå=Üìã~å=íÉãéÉê~íìêÉë~í= PTø`X= íÜÉ= çéíáã~ä= íÉãéÉê~íìêÉ= Ñçê= ÖêçïíÜ= áë= ÄÉíïÉÉå= OTJPMø`K= qÜáë~ÅÅçìåíë=Ñçê=íÜÉ=éêÉÇçãáå~åÅÉ=çÑ=äÉéêçìë=äÉëáçåë=áå=íÜÉ=ÅçäÇÉëí=~êÉ~ë=çÑíÜÉ=ÄçÇóK

jóÅçÄ~ÅíÉêáìã=äÉéê~É áë=~å=çÄäáÖ~íçêó=áåíê~ÅÉääìä~ê=é~ê~ëáíÉ=ïáíÜ=íêçéáëãÑçê=ã~ÅêçéÜ~ÖÉë=~åÇ=pÅÜï~åå=ÅÉääëK=qÜáë=éêÉÑÉêÉåÅÉ=áë=ÇÉíÉêãáåÉÇ=Äó=íÜÉ~íí~ÅÜãÉåí= çÑ= íÜÉ= Ä~ÅíÉêáìã= çåíç= íÜÉ= ~äÑ~JO= ÅÜ~áå= ÚdÛ= ìåáí= ÑçìåÇ= áå= íÜÉÄ~ë~ä= ä~ãáå~= çÑ= pÅÜï~åå= ÅÉääëK= qÜáë= ä~ãáå~= áë= çåäó= ÑçìåÇ= áå= éÉêáéÜÉê~äåÉêîÉëK=^í=~åó=ÖáîÉå=ãçãÉåíI=qJÅÉääë=ïáää=êÉÅçÖåáëÉ=íÜÉ=Ä~ÅíÉêáìã=áåëáÇÉ~åÇ= ÜÉåÅÉ= íêáÖÖÉê= ~= ëìÄ~ÅìíÉ= ~åÇ= ä~íÉê= ÅÜêçåáÅ= áåÑä~ãã~íçêó= êÉëéçåëÉKqÜáë=êÉëìäíë= áå=éêçÖêÉëëáîÉ=Ç~ã~ÖÉ=íç=ÄçíÜ=ãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇåÉêîÉ= ÑáÄêÉë= ÑçääçïÉÇ=Äó= êÉéä~ÅÉãÉåí=çÑ= ÑìåÅíáçå~ä= íáëëìÉ=Äó=ÅçåàìåÅíáîÉíáëëìÉK= qÜÉ= éÜÉåçäáÅ= ÖäóÅçäáéáÇJN= EmdiJNF= áë= ~= ÅÉääJï~ää= ~åíáÖÉå= çå= íÜÉëìêÑ~ÅÉ=çÑ=jóÅçÄ~ÅíÉêáìã= äÉéê~É íÜêçìÖÜ=ïÜáÅÜ= áí=~íí~ÅÜÉë= íç=pÅÜï~ååÅÉääëK=kÉêîÉ=ÑáÄêçëáë=áë=êÉëéçåëáÄäÉ=Ñçê=áêêÉîÉêëáÄäÉ=åÉêîÉ=Ç~ã~ÖÉ=áå=äÉéêçìëåÉìêçé~íÜóK

Transmission

_çíÜ=å~ë~ä=ëÉÅêÉíáçåë=~åÇ=ëâáå= Ñêçã=ìåíêÉ~íÉÇ=ãìäíáÄ~Åáää~êó=Å~ëÉë=çÑäÉéêçëó= ~êÉ= Å~é~ÄäÉ= çÑ= ëÜÉÇÇáåÖ= jóÅçÄ~ÅíÉêáìã= äÉéê~É íç= íÜÉÉåîáêçåãÉåíI=ïÜáÅÜ=ã~ó=ÄÉ=ÇÉéçëáíÉÇ=çå=ÉáíÜÉê=çê=ÄçíÜ=íÜÉ=å~ë~ä=~åÇ=ëâáåÉéáíÜÉäá~=ïÜÉå=áå=Åçåí~Åí=ïáíÜ=çíÜÉêë=~åÇ=ëç=éçíÉåíá~ääó=áåáíá~íáåÖ=áåÑÉÅíáçåKe~Éã~íçÖÉåçìë=ÇáëëÉãáå~íáçå=áë=ïáÇÉäó=~ÅÅÉéíÉÇ=~ë=íÜÉ=ãçëí=äáâÉäó=êçìíÉçÑ= áåÑÉÅíáçå= áå= íÜÉ= äÉéêçã~íçìë= ÑçêãK=qÜÉ= áåÅìÄ~íáçå=éÉêáçÇ= áë=ÉñíêÉãÉäóî~êá~ÄäÉK=fí=ã~ó=ÄÉ=~ë=äçåÖ=~ë=NMJOM=óÉ~êëK=bñéçëìêÉ=íç=íÜÉ=Ä~Åáääìë=áë=åçíëìÑÑáÅáÉåí= Ñçê= íÜÉ= ÇáëÉ~ëÉ= íç= ÇÉîÉäçéK= c~Åíçêë= ëìÅÜ= ~ë= åìíêáíáçåI= ÜóÖáÉåÉ~åÇ= ÜçìëÉ= ÅêçïÇáåÖ= éä~ó= ~å= áãéçêí~åí= êçäÉK= qÜÉêÉ= áë= ~= ã~äÉéêÉéçåÇÉê~åÅÉ= áå= íÜÉ= äÉéêçã~íçìë= Ñçêã= ~åÇ= ~= ÑÉã~äÉ= ÉñÅÉëë= áå= íÜÉíìÄÉêÅìäçáÇ=íóéÉK=


81



Specific cutaneous lesionspéÉÅáÑáÅ=Åìí~åÉçìë=äÉëáçåëI=áåÅäìÇáåÖ=ã~Åìä~É=~åÇ=äÉéêçã~ÉI=êÉîÉ~ä=íÜÉ

ÇáëÉ~ëÉ= áå=Ü~äÑ=çê=ãçêÉ=çÑ=é~íáÉåíëI=ÇÉéÉåÇáåÖ=çå=íÜÉ=íóéÉ=çÑ= äÉéêçëóK= fåçíÜÉêëI=ëã~ää=~êÉ~ë=çÑ=ëÉåëçêó=äçëëI=äáãáíÉÇ=~åÜóÇêçëáë=~åÇ=~äçéÉÅá~=òçåÉëIé~êÉëáë= çÑ= Ñ~Åá~ä= ãìëÅäÉëI= ÜóéçÅÜêçãáÅ= çê= ~íêçéÜáÅ= Åìí~åÉçìë= òçåÉë= çêé~áåÑìä= Éåä~êÖÉãÉåí= çÑ= ~= åÉêîÉ= íêìåâ= ~êÉ= íÜÉ= éêÉëÉåíáåÖ= ã~åáÑÉëí~íáçåëKmä~åí~ê=ìäÅÉêë=~åÇ=çíÜÉê=íêçéÜáÅ=ÅÜ~åÖÉë=çÅÅìê=ä~íÉê=áå=íÜÉ=ÅçìêëÉ=çÑ=íÜÉÇáëÉ~ëÉI=~ë=~=ÅçåëÉèìÉåÅÉ=çÑ=ëÉåëçêó=äçëëK

Sensory losspÉåëçêó= äçëë= áë= íÜÉ= ãçëí= Åçåëí~åí= ÑáåÇáåÖ= çÑ= äÉéêçìë= åÉìêçé~íÜóK

pÉåëçêó= äçëëI= ïÜáÅÜ= áë= ÇìÉ= íç= ãáñÉÇ= ÇÉêã~ä= åÉêîÉ= ~åÇ= åÉêîÉ= íêìåâÇ~ã~ÖÉI= áë= ÉñíêÉãÉäó= î~êá~ÄäÉ= áå= ÇáëíêáÄìíáçåI= ê~åÖáåÖ= Ñêçã= ~= ëã~ää= ëâáåé~íÅÜ= ïáíÜ= áãé~áêÉÇ= ëÉåë~íáçå= íç= ëÉîÉêÉ= ëÉåëçêó= äçëë= çîÉê= ãçëí= çÑ= íÜÉÄçÇó=ëìêÑ~ÅÉI=Äìí=~îçáÇáåÖ=íÜÉ=ÄçÇó=ÑçäÇëK=b~êäó=Åìí~åÉçìë=äÉëáçåë=ëÜçïëçãÉ=éêÉëÉêî~íáçå=çÑ=ëÉåë~íáçåI=ïáíÜ=áãé~áêãÉåí=çÑ=äáÖÜí=íçìÅÜI=~åÇ=äçëë=çÑíÜÉêã~ä= ~åÇ= é~áå= ëÉåëÉI= ïÜáÅÜ= äÉ~Çë= íç= é~áåäÉëë= íê~ìã~= ~åÇ= íêçéÜáÅÅÜ~åÖÉëK=mêçéêáçÅÉéíáçå=áë=éêÉëÉêîÉÇI=ëç=é~íáÉåíë=Å~å=ëíáää=ìëÉ=íÜÉáê=ä~êÖÉäó~å~ÉëíÜÉíáÅ= äáãÄë= ÉÑÑÉÅíáîÉäóK= içëë= çÑ= ÇÉêã~ä= éáÖãÉåí= áå= íÜÉ= íÉêêáíçêó= çÑ~ÑÑÉÅíÉÇ=Åìí~åÉçìë=åÉêîÉë=äÉ~Çë=íç=íÜÉ=ÇÉîÉäçéãÉåí=çÑ=ä~êÖÉ=~å~ÉëíÜÉíáÅé~íÅÜÉë= áå=Ç~êâJëâáååÉÇ=éÉçéäÉI=ïáíÜ= äçëë=çÑ= ëïÉ~íáåÖ= áå=ÅçêêÉëéçåÇáåÖ~êÉ~ëK= `çäÇÉê= ~êÉ~ë= çÑ= íÜÉ= ÄçÇó= ëÉÉã= ãçêÉ= ~ÑÑÉÅíÉÇI= Äìí= íÉãéÉê~íìêÉJäáåâÉÇ=ëÉåëçêó=äçëëI=ïÜáÅÜ=áë=åçí=çÄëÉêîÉÇ=áå=íìÄÉêÅìäçáÇ=äÉéêçëóI=Å~ååçí~ÅÅçìåí= Ñçê= ~ää= é~ííÉêåë= çÑ= åÉêîÉ= äÉëáçåë= áå= äÉéêçëóK= få= ëçãÉ= Å~ëÉëIÅçãéäÉíÉ= äçëë= çÑ= é~áå= ~åÇ= íÉãéÉê~íìêÉ= ëÉåë~íáçå= áå= ~= ÅÉêí~áå= ~êÉ~Åçåíê~ëíë=ïáíÜ=éêÉëÉêî~íáçå=çÑ=í~ÅíáäÉ=ëÉåë~íáçåK=qÜáë=Åä~ëëáÅ~ä=ÇáëëçÅá~íáçåçÑ=ëÉåëçêó=äçëë=áë=ëÉäÇçã=ÅçãéäÉíÉ=áå=äÉéêçëóK=få=ãçëí=Å~ëÉë=~ää=ãçÇ~äáíáÉëçÑ=ëìéÉêÑáÅá~ä=ëÉåë~íáçå=~êÉ=~ÑÑÉÅíÉÇK=pÉåëçêó=äçëë=~äëç=çÅÅìêë=áå=íÜÉ=~êÉ~ëÅçêêÉëéçåÇáåÖ= íç= ã~Åìä~ÉI= ÇÉãçåëíê~íáåÖ= É~êäó= áåîçäîÉãÉåí= çÑ= ëÉåëçêóåÉêîÉ=íÉêãáå~äëK=

qÜÉ= íçéçÖê~éÜáÅ~ä= ÇáëíêáÄìíáçå= çÑ= ëÉåëçêó= ÇáëíìêÄ~åÅÉ= áë= ÉñíêÉãÉäóî~êá~ÄäÉK=pÉåëçêó=äçëë=ã~ó=Ñçêã=~å=Úáåëìä~êÛ=é~ííÉêåI=áå=ïÜáÅÜ=~å~ÉëíÜÉíáÅ~êÉ~ë=çÑ=î~êá~ÄäÉ=ÑçêãëI=ëáòÉ=~åÇ=åìãÄÉê=~êÉ=ÑçìåÇI=~åÇ=íÜÉëÉ=~êÉ~ë=ã~óçê=ã~ó=åçí=ÅçêêÉä~íÉ=ïáíÜ=íÜÉ=ã~Åìä~êJíóéÉ=Åìí~åÉçìë=äÉëáçåëK=qÜáë=ëÉåëçêóäçëëI= ïÜáÅÜ= ã~ó= ä~ëí= Ñçê= óÉ~êëI= áë= ìëì~ääó= ~ëëçÅá~íÉÇ= ïáíÜ= çíÜÉê


82


ÇáëíìêÄ~åÅÉë=ëìÅÜ=~ë=~åÜóÇêçëáëI=~äçéÉÅá~=~åÇ=î~ëçãçíçê=~êÉÑäÉñá~K=qÜÉëÉã~åáÑÉëí~íáçåë=~êÉ=êÉä~íÉÇ=íç=äÉëáçåë=çÑ=ëÉåëçêó=åÉêîÉ=ÉåÇáåÖë=çê=íç=íÜ~í=çÑ~=äáãáíÉÇ=åìãÄÉê=çÑ=åÉêîÉ=Ñ~ëÅáÅäÉë=çÑ=~=åÉêîÉ=íêìåâK=pÉåëçêó=äçëë=ã~ó=~äëçÇáëéä~ó=~=åÉêîÉ=íêìåâ=é~ííÉêåK=få=Å~ëÉë=çÑ=äçåÖëí~åÇáåÖ=ÉîçäìíáçåI=íÜÉ=Çáëí~äé~êí=çÑ=íÜÉ=äáãÄë=ëÜçï=íÜÉ=ÖêÉ~íÉëí=ëÉåëçêó=äçëëK=qÜáë=ÉñíÉåÇë=éêçñáã~ääóíç=~=ÖêÉ~íÉê=çê=äÉëëÉê=ÉñíÉåíI=ê~êÉäó=íç=íÜÉ=íêìåâK=tÜÉå=íÜÉ=íêìåâ=áë=áåîçäîÉÇIëÉåëçêó=äçëë=~ÑÑÉÅíë=~å=áåëìä~ê=é~ííÉêåK=qÜáë=é~ííÉêå=çÑ=ëÉåëçêó=äçëë=ÇçÉëåçí=~ÑÑÉÅí=íÜÉ=~åíÉêáçê=~ëéÉÅí=çÑ=íÜÉ=íêìåâ=áå=~=äÉåÖíÜJÇÉéÉåÇÉåí=é~ííÉêåI=~ëáå= ëÉîÉêÉ= Çá~ÄÉíáÅI= ~ãóäçáÇ= çê= ~äÅçÜçäáÅ= éçäóåÉìêçé~íÜóK= få= áåÇáîáÇì~äé~íáÉåíëI= ÇáëëçÅá~íáçå= ÄÉíïÉÉå= ëÉåë~íáçåë= ã~ó= ÄÉ= ÑçìåÇ= áå= ëçãÉ= ~êÉ~ëçåäóK=qÜÉ=ä~êÖÉ=åÉêîÉ=íêìåâë=ãçëí=Åçããçåäó=~ÑÑÉÅíÉÇ=~êÉ=íÜÉ=ìäå~ê=~åÇ=íÜÉä~íÉê~ä=éçéäáíÉ~ä=åÉêîÉëI=ÑçääçïÉÇ=Äó=íÜÉ=ãÉÇá~åI=éçëíÉêáçê=íáÄá~äI=ëìéÉêÑáÅá~äê~Çá~äI=éÉêçåÉ~ä=åÉêîÉëI=~åÇ=íÜÉ=ÖêÉ~íÉê=~ìêáÅìä~ê=~åÇ=Ñ~Åá~ä=åÉêîÉëK

Nerve hypertrophykÉêîÉ=íêìåâë=~êÉ=é~äé~Ääó=Éåä~êÖÉÇ=áå=çåÉ=íÜáêÇ=çÑ=é~íáÉåíë=ïáíÜ=äÉéêçëóI

ëçãÉíáãÉë= ÄÉÑçêÉ= íÜÉ= çÅÅìêêÉåÅÉ= çÑ= ëÉåëçêó= äçëë= áå= íÜÉ= ÅçêêÉëéçåÇáåÖíÉêêáíçêó=RK=pìéÉêÑáÅá~ä=åÉêîÉë=äáâÉ=íÜÉ=ÖêÉ~íÉê=~ìêáÅìä~ê=åÉêîÉ=áå=íÜÉ=åÉÅâIíÜÉ= ëìéê~çêÄáí~êó= Äê~åÅÜ= çÑ= íÜÉ= íêáÖÉãáå~ä= åÉêîÉ= çê= ä~êÖÉê= åÉêîÉ= íêìåâëIÉëéÉÅá~ääó= íÜÉ=ìäå~ê=åÉêîÉ=~ÄçîÉ= íÜÉ=ÉäÄçïI= íÜÉ=éÉêçåÉ~ä=åÉêîÉI=~åÇ= íÜÉê~Çá~ä=Åìí~åÉçìë=åÉêîÉ=~í=íÜÉ=ä~íÉê~ä=ÄçêÇÉê=çÑ=íÜÉ=ïêáëíI=~êÉ=çÑíÉå=Éåä~êÖÉÇK=

Motor disturbance and amyotrophyjçíçê=áåîçäîÉãÉåí=áë=ìëì~ääó=~=ä~íÉ=ÉîÉåí=áå=íÜÉ=ÅçìêëÉ=çÑ=íÜÉ=ÇáëÉ~ëÉK

^ãóçíêçéÜó=~åÇ=ãçíçê=ïÉ~âåÉëë=ìëì~ääó=éêçÖêÉëë=Éèì~ääóX=áå=ëçãÉ=Å~ëÉëIÜçïÉîÉêI= ~ãóçíêçéÜó= áë= ãçêÉ= ã~êâÉÇ= íÜ~å= ïÉ~âåÉëëI= ïÜáÅÜ= ÄçíÜéêÉÇçãáå~íÉ=áå=íÜÉ=ìäå~ê=~åÇ=ãÉÇá~å=åÉêîÉ=íÉêêáíçêáÉëI=ïáíÜ=ÅÜ~ê~ÅíÉêáëíáÅÅä~ï= Ü~åÇëK= mêÉëÉêî~íáçå= çÑ= ÇÉÉé= íÉåÇçå= êÉÑäÉñÉë= áå= ã~åó= Å~ëÉë= çÑäÉéêçìë=åÉìêçé~íÜó=áë=ÅÜ~ê~ÅíÉêáëíáÅ=çÑ=íÜÉ=éêÉÇçãáå~åí=áåîçäîÉãÉåí=çÑ=íÜÉãçëí=Çáëí~ä=é~êí=çÑ=íÜÉ=åÉêîÉëK=

Facial palsyc~Åá~ä=é~äëó=ïáíÜ=ä~ÖçéÜíÜ~äãçë=çÑ=çåÉ=çê=ÄçíÜ=ÉóÉëI=ïáíÜ=ëé~êáåÖ=çÑ

íÜÉ=çíÜÉê=ãìëÅäÉë=ëìééäáÉÇ=Äó=íÜÉ=Ñ~Åá~ä=åÉêîÉI= áë=~=Åä~ëëáÅ~ä=ÑÉ~íìêÉ=çÑäÉéêçëóK=


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Trophic disturbancesqêçéÜáÅ=éä~åí~ê=ìäÅÉêë=~êÉ=~=ÅçããçåI=åçåJëéÉÅáÑáÅ=ÅçãéäáÅ~íáçå=çÑ=äçëë

çÑ=é~áå=ëÉåë~íáçå=çîÉê=íÜÉ=éä~åí~ê=ëçäÉK=pÉîÉêÉ=ëÉåëçêó=ÇáëíìêÄ~åÅÉë=~êÉ~äï~óë= ÑçìåÇ= áå= íÜçëÉ= ~êÉ~ë= ïÜÉêÉ= ìäÅÉêë= çÅÅìêK= mä~åí~ê= ìäÅÉêë= çÅÅìêëìÄëÉèìÉåí= íç= ãáÅêçíê~ìã~= çå= ëâáå= íÜ~í= Ü~ë= äçëí= é~áåÑìä= ëÉåë~íáçåK= qÜÉ~ÄëÉåÅÉ= çÑ= éêçíÉÅíáîÉ= ëÉåë~íáçå= áå= äáãÄ= ÉñíêÉãáíáÉë= äÉ~Çë= íç= çîÉêìëÉI~ÅÅáÇÉåí~ä=ëÉäÑJáåàìêóI=êÉÅìêêÉåí=áåÑÉÅíáçåëI=~åÇ=íç=~=Öê~Çì~ä=ÇÉîÉäçéãÉåí=çÑÑìêíÜÉê=ÇÉÑçêãáíáÉë=~ë=çÄëÉêîÉÇ=áå=ëÉåëçêó=åÉìêçé~íÜó=çÑ=~=ÇáÑÑÉêÉåí=çêáÖáåK=

qÜÉ=ëéÉÅíêìã=çÑ=ÅäáåáÅ~ä=ã~åáÑÉëí~íáçåë=ÅçêêÉä~íÉë=ïÉää=ïáíÜ=íÜÉ=ÅÉääìä~êáããìåÉ=êÉëéçåëáîÉåÉëë=çÑ=íÜÉ=é~íáÉåí=íç=jóÅçÄ~ÅíÉêáìã=äÉéê~É ~åíáÖÉåëIïÜáÅÜ= ê~åÖÉ= Ñêçã= íÜÉ= äçïÉëí= ÅÉääJãÉÇá~íÉÇ= áããìåáíó= íç= jóÅçÄ~ÅíÉêáìãäÉéê~ÉI=íÜÉ=äÉéêçã~íçìë=éçäÉI=íç=íÜ~í=çÑ=íÜÉ=ÜáÖÜÉëí=ÅÉääJãÉÇá~íÉÇ=áããìåáíóIíÜÉ=íìÄÉêÅìäçáÇ=éçäÉ=SK

Different patterns of leprous neuropathy

qïç= ã~áå= é~ííÉêåë= çÑ= äÉéêçìë= åÉìêçé~íÜó= Ü~îÉ= ÄÉÉå= áÇÉåíáÑáÉÇW= íÜÉäÉéêçã~íçìë=íóéÉ=çê=ãìäíáÄ~Åáää~êó=äÉéêçìë=åÉìêçé~íÜóI=~åÇ=íÜÉ=íìÄÉêÅìäçáÇäÉéêçìë=åÉìêçé~íÜó=çê=é~ìÅáÄ~Åáää~êó=äÉéêçìë=åÉìêçé~íÜó=TJVK

Lepromatous and borderline lepromatous leprosy — multibacillaryleprosyqÜÉëÉ=êÉéêÉëÉåí=íÜÉ=ãçëí=Åçããçå=íóéÉë=çÑ= äÉéêçëó= áå=ã~åó=ÉåÇÉãáÅ

~êÉ~ë=çÑ=^ÑêáÅ~K=få=åÉ~êäó=~ää=Å~ëÉë=íÜÉó=~êÉ=~ëëçÅá~íÉÇ=ïáíÜ=ÅÜ~ê~ÅíÉêáëíáÅëâáå=äÉëáçåëK=lÅÅ~ëáçå~ääóI=íÜÉêÉ=áë=åç=ÇÉíÉÅí~ÄäÉ=ëâáå=äÉëáçåK=pâáå=äÉëáçåëIÜçïÉîÉêI= ~êÉ= ìëì~ääó= åìãÉêçìë= ÅçåëáëíáåÖ= çÑ= ã~ÅìäÉëI= é~éìäÉë= ~åÇåçÇìäÉë=ïáíÜ=áåÑáäíê~íáçå=~åÇ=íÜáÅâÉåáåÖ=çÑ=íÜÉ=ëâáåI=~ÑÑÉÅíáåÖ=éêÉÇçãáå~åíäóíÜÉ=ÅççäÉê=~êÉ~ë=çÑ=íÜÉ=ÄçÇóK=^í=íÜáë=ëí~ÖÉI=ÇáÑÑìëÉI=Äáä~íÉê~ä=~åÇ=ÖÉåÉê~ääóëóããÉíêáÅ~ä=åÉêîÉ=Ç~ã~ÖÉ=çÅÅìêëK=få=ëìÅÜ=é~íáÉåíë=Ä~ÅíÉêá~=Å~å=ÄÉ=ÑçìåÇáå=ëâáå=äÉëáçåëI=å~ë~ä=ëãÉ~êëI=çê=ÉîÉå=áå=ÄäççÇ=ïáíÜ=~=ã~àçêáíó=çÑ=ÅáêÅìä~íáåÖÄ~Åáääá= ÑçìåÇ= áåíê~ÅÉääìä~êäóI= áå= éçäóãçêéÜçåìÅäÉ~ê= äÉìâçÅóíÉëI= ãçåçÅóíÉë~åÇ=ä~êÖÉ=ÅáêÅìä~íáåÖ=ÜáëíáçÅóíÉëK=

få=íÜáë=ÑçêãI=íÜÉ=ëéÉÅáÑáÅ=ìåêÉëéçåëáîÉåÉëë=çÑ=íÜÉ=Üçëí=íç=~åíáÖÉåë=çÑ=íÜÉäÉéêçëó=Ä~Åáääìë=éÉêãáíë=ìåÅÜÉÅâÉÇ=éêçäáÑÉê~íáçå=çÑ=Ä~ÅáääáK=


84


iáÖÜí= ãáÅêçëÅçéáÅ= Éñ~ãáå~íáçå= çÑ= åÉêîÉ= ÅêçëëJëÉÅíáçåë= çÑ= ~ÑÑÉÅíÉÇåÉêîÉë=ëÜçï=~å=Éåçêãçìë=áåÑä~ãã~íçêó=êÉ~Åíáçå=~ÑÑÉÅíáåÖ=íÜÉ=ÉéáåÉìêáìãçÑ= ~ää= åÉêîÉ= ëéÉÅáãÉåë= ~åÇ= íÜÉ= éÉêáåÉìêáìã= çÑ= ãçëí= Ñ~ëÅáÅäÉë= NMK= qÜáëáåÑä~ãã~íçêó= êÉ~Åíáçå= áë= êÉëéçåëáÄäÉ= Ñçê= íÜÉ= åÉêîÉ= Éåä~êÖÉãÉåíKjóÅçÄ~ÅíÉêáìã=äÉéê~É ~êÉ=ÉñíêÉãÉäó=åìãÉêçìëI=áå=~ää=Ñçêãë=çÑ=äÉéêçã~íçìëåÉìêçé~íÜóX= íÜÉó= ~êÉ= çÑíÉå= áå= ~= ÖäçÄìë= ~êê~åÖÉãÉåíI= çå= wáÉÜäJëí~áåÉÇé~ê~ÑÑáåJÉãÄÉÇÇÉÇ=ëéÉÅáãÉåëK=jóÅçÄ~ÅíÉêáìã=äÉéê~É=~êÉ=É~ëáäó=áÇÉåíáÑáÉÇçå=ÉäÉÅíêçå=ãáÅêçëÅçéáÅ=Éñ~ãáå~íáçå=~ë=Ç~êâI=çëãáçéÜáäáÅ=ëéÜÉêÉë=ìëì~ääóäçÅ~íÉÇ= áå=~=Åóíçéä~ëãáÅ=î~ÅìçäÉ=Åçåí~áåáåÖ=éÜÉåçäáÅ=ÖäóÅçäáéáÇJf= EmdiJfF~åÇ= äáéç~ê~Äáåçã~åå~åI= ÄçíÜ= éêçÇìÅÉÇ= áå= ä~êÖÉ= ~ãçìåíë= ÄójóÅçÄ~ÅíÉêáìã=äÉéê~ÉK

^åçíÜÉê=ë~äáÉåí=ÑÉ~íìêÉ=çÄëÉêîÉÇ=áå=åÉêîÉë=çÑ=é~íáÉåíë=ïáíÜ=äÉéêçã~íçìëäÉéêçëó=áë=íÜÉ=áåíÉåëÉ=éêçäáÑÉê~íáçå=çÑ=ÑáÄêçÄä~ëíë=ïáíÜ=áåÅêÉ~ëÉÇ=ëóåíÜÉëáëçÑ=Åçää~ÖÉåI= äÉ~ÇáåÖ=íç=ÉåÇçJ=~åÇ=éÉêáJåÉìêá~ä= ÑáÄêçëáë=ïÜáÅÜ=Å~å=Ü~ãéÉêÖêçïíÜ=çÑ=êÉÖÉåÉê~íáåÖ=ÑáÄêÉëK=`çåíêçä=çÑ=ìåï~åíÉÇ=ëÅäÉêçëáë=Å~å=ÅÉêí~áåäóáãéêçîÉ=íÜÉ=çìíÅçãÉ=çÑ=íÜÉ=åÉìêçé~íÜóK

Tuberculoid leprosy ^í= íÜÉ=çíÜÉê=ÉåÇ=çÑ= íÜÉ=ëéÉÅíêìãI= íìÄÉêÅìäçáÇ= äÉéêçëó= áë= áÇÉåíáÑáÉÇ=Äó

ÅçãéäÉíÉ= åÉêîÉ= ÇÉëíêìÅíáçåK= få= íÜáë= Ñçêã= é~íáÉåíë= ÇÉîÉäçé= ÜáÖÜ= äÉîÉäë= çÑëéÉÅáÑáÅ=ÅÉääJãÉÇá~íÉÇ=áããìåáíó=íÜ~í=ìäíáã~íÉäó=âáääë=~åÇ=ÅäÉ~êë=íÜÉ=Ä~Åáääá=áåíÜÉ=íáëëìÉëI=áåÇìÅáåÖ=ëáãìäí~åÉçìë=Ç~ã~ÖÉ=íç=íÜÉ=åÉêîÉë=íÜ~í=Ü~êÄçìê=íÜÉÄ~ÅáääáK= `äáåáÅ~ääóI= íìÄÉêÅìäçáÇ= äÉëáçåë= ã~ó= ÄÉ= ëáåÖäÉ= çê= ÑÉïI= ~åÇ= ~êÉÇáëíêáÄìíÉÇ= ~ëóããÉíêáÅ~ääó= áå= íÜÉ= îáÅáåáíó= çÑ= íóéáÅ~ä= Üóéç~ÉëíÜÉíáÅ= çê~å~ÉëíÜÉíáÅ=ÜóéçéáÖãÉåíÉÇ=ëâáå= äÉëáçåëK=qÜÉêÉ= áë=ÅçåëáÇÉê~ÄäÉ=ÉîáÇÉåÅÉëìÖÖÉëíáåÖ= íÜ~í= é~íáÉåíë= ïáíÜ= íìÄÉêÅìäçáÇ= äÉéêçëó= Ü~îÉ= åÉêîÉ= Ç~ã~ÖÉÅ~ìëÉÇ= åçí= Äó= íÜÉ= Ä~Åáääá= Äìí= Äó= íÜÉ= ÅÉääJãÉÇá~íÉÇ= áããìåÉ= êÉëéçåëÉ= íçjóÅçÄ~ÅíÉêáìã=äÉéê~É ~åíáÖÉåëK

eáëíçé~íÜçäçÖáÅ~ääóI= íÜÉ= äÉëáçå= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ÉéáíÜÉäáçáÇJÅÉääÖê~åìäçã~í~=ïáíÜ= áåíÉåëÉ= äóãéÜçÅóíáÅ= áåÑáäíê~íáçåK=kçêã~ä=åÉêîÉ=ëíêìÅíìêÉã~ó=åç=äçåÖÉê=ÄÉ=áÇÉåíáÑáÉÇ=áå=ã~åó=Å~ëÉë=~åÇ=Ä~Åáääá=~êÉ=åçí=ÑçìåÇ=áå=íÜÉäÉëáçåëI=Äìí=jóÅçÄ~ÅíÉêáìã=äÉéê~É ~åíáÖÉåë=Ü~îÉ=ÄÉÉå=ÇÉíÉÅíÉÇ=áå=åÉêîÉëìëáåÖ= ~åíáJ_`d= EÄ~Åáääìë= `~äãÉííÉJdì¨êáåF= ~åíáJëÉê~= ïÜáÅÜ= ÅêçëëJêÉ~ÅíïáíÜ= jóÅçÄ~ÅíÉêáìã= äÉéê~É ~åíáÖÉåëK= få= ëâáå= äÉëáçåë= íÜÉ= íìÄÉêÅìäçáÇáåÑáäíê~íÉë= éêÉÇçãáå~åíäó= Åçåí~áå= ÜÉäéÉê= qJÅÉääëK= qÜÉ= Ä~ëáë= Ñçê= íÜÉÅçåëéáÅìçìë=ÇÉëíêìÅíáçå=çÑ=åÉêîÉ=ëíêìÅíìêÉ=áë=íÜçìÖÜí=íç=ÄÉ=~=ÇÉä~óÉÇJíóéÉ


85


ÜóéÉêëÉåëáíáîáíó= êÉ~Åíáçå= ïáíÜ= ëéÉÅáÑáÅ= ÜÉäéÉê= qJÅÉääë= êÉ~ÅíáåÖ= ïáíÜjóÅçÄ~ÅíÉêáìã= äÉéê~É= ~åíáÖÉåë= éêÉëÉåíÉÇ= áå= íÜÉ= ÉåÇçåÉìêáìã= Äóã~ÅêçéÜ~ÖÉë= ~åÇ= éçëëáÄäó= Äó= pÅÜï~åå= ÅÉääë= ÉñéêÉëëáåÖ= íÜÉ= ei^Jao~åíáÖÉå= áåÇìÅÉÇ= Äó= áåíÉêÑÉêçå= êÉäÉ~ëÉÇ= Äó= ÜÉäéÉê= qJÅÉääëK= ^Åíáî~íáçå= çÑã~ÅêçéÜ~ÖÉë=áå=íÜáë=ÅçåíÉñí=äÉ~Çë=íç=íÜÉ=êÉäÉ~ëÉ=çÑ=~=åìãÄÉê=çÑ=ëÉÅêÉíçêóéêçÇìÅíë=åçñáçìë=íç=ëìêêçìåÇáåÖ=ÅÉääëK

Reactional states

låÉ= çÑ= íÜÉ= ã~åó= ÅçåÅÉêåë= áå= é~íáÉåíë= ìåÇÉê= íêÉ~íãÉåí= Ñçê= äÉéêçìëåÉìêçé~íÜó= áë= íÜÉ= çÅÅìêêÉåÅÉ= çÑ= ~= ëìÇÇÉå= ~äíÉê~íáçå= áå= áããìåçäçÖáÅ~äëí~íìë=~åÇ=íÜÉ=ÇÉîÉäçéãÉåí=çÑ=~=êÉ~Åíáçå~ä=ëí~íÉK

The reversal or upgrade form or type 1 reactionqÜáë= ~ééÉ~êë= Åçããçåäó= ÇìêáåÖ= íÜÉ= Ñáêëí= óÉ~ê= çÑ= íÜÉê~éóI= çê= ìé= íç

ÇÉÅ~ÇÉë=ä~íÉêK=fí=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=~=ÜÉáÖÜíÉåÉÇ=ÅÉääJãÉÇá~íÉÇ=êÉëéçåëÉçÅÅìêêáåÖ= ã~áåäó= áå= é~íáÉåíë= ïáíÜ= íÜÉ= ÄçêÇÉêäáåÉJäÉéêçã~íçìë= Ñçêã= çÑäÉéêçëó=EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çîÉêäÉ~ÑFK=qÜáë=êÉ~Åíáçå=áë=áÇÉåíáÑáÉÇ=Äó=ëïÉääáåÖ~åÇ= Éñ~ÅÉêÄ~íáçå= çÑ= ÉñáëíáåÖ= ëâáå= ~åÇ= åÉêîÉ= äÉëáçåë= áå= ~ëëçÅá~íáçå= ïáíÜÖÉåÉê~ä=ã~ä~áëÉ=~åÇ=ÑÉîÉêK=m~áåÑìä=ëïÉääáåÖ=çÑ=åÉêîÉ=íêìåâë=áë=~ÅÅçãé~åáÉÇÄó=ëÉåëçêó=~åÇ=ãçíçê=ÇÉÑáÅáí=áå=íÜÉ=ÅçêêÉëéçåÇáåÖ=íÉêêáíçêóK=få=ëçãÉ=Å~ëÉëIåÉêîÉë= ïÜáÅÜ= ïÉêÉ= ~éé~êÉåíäó= ìå~ÑÑÉÅíÉÇ= ~êÉ= ÜÉ~îáäó= Ç~ã~ÖÉÇKbåÇçåÉìêá~ä= Öê~åìäçã~ëI= ãìäíáåìÅäÉ~íÉÇ= Öá~åí= ÅÉääëI= äóãéÜçÅóíáÅáåÑáäíê~íáçåI= î~ëÅìäáíáë= ~åÇ= éÉêáåÉìêáíáë= ~êÉ= éêÉëÉåí= çå= ãçêéÜçäçÖáÅ~äÉñ~ãáå~íáçåK= kÉÅêçëáë= çÑ= íÜÉ= ÉåÇçåÉìêá~ä= ÅçåíÉåí= ã~ó= äÉ~Ç= íç= åÉêîÉ~ÄëÅÉëëÉëK=kç=jóÅçÄ~ÅíÉêáìã=äÉéê~É ~êÉ=çÄëÉêîÉÇ=áå=íÜáë=êÉ~ÅíáçåK=

Erythema nodosum leprosum (ENL)-type 2 reactionbki=ÅçêêÉëéçåÇë=íç=~=ÇçïåÖê~ÇÉ=êÉ~ÅíáçåK=fí=áë=~äãçëí=ÉñÅäìëáîÉäó=ëÉÉå

áå=íÜÉ=äÉéêçã~íçìë=éçäÉ=áå=ÉåÇÉãáÅ=~êÉ~ëK=fí=áë=~=ÅçãéäÉñ=ëóëíÉãáÅ=ÇáëçêÇÉê~ÑÑÉÅíáåÖ= ãìäíáéäÉ= çêÖ~åëK= qÜÉ= ~ééÉ~ê~åÅÉ= çÑ= íóéáÅ~ä= ÉêóíÜÉã~íçìë= ëâáåäÉëáçåë=áë=íÜÉ=Çá~ÖåçëíáÅ=ÅêáíÉêáçå=Ñçê=bkiK=kçÇìäÉë=~åÇ=é~áåÑìäI=ê~áëÉÇI=êÉÇé~éìäÉë= ~êÉ= ÅÜ~ê~ÅíÉêáëíáÅK= ^ÅÅçãé~åóáåÖ= íÜÉëÉ= åçÇìäÉë= ~êÉ= ìîÉáíáëIáêáÇçÅóÅäáíáëI= ÉéáëÅäÉêáíáëI= åÉìêáíáëI= ~êíÜêáíáëI= Ç~ÅíáäáíáëI= äóãéÜç~ÇÉåáíáëI= ~åÇçêÅÜáíáëK= cÉîÉêI= éêçëíê~íáçåI= ~åçêÉñá~I= ~ë= ïÉää= ~ë= çíÜÉê= Åçåëíáíìíáçå~äëóãéíçãëI= ~êÉ= ÑêÉèìÉåíI= ïáíÜ= íÜÉ= ÑáåÇáåÖ= çÑ= ÅÜêçåáÅ= çê= ëìÄJ~ÅìíÉáåÑä~ãã~íçêó=áåÑáäíê~íÉë=~åÇ=~êÉ~ë=çÑ=ÅçåàìåÅíáîÉ=íáëëìÉ=ïáíÜ=çê=ïáíÜçìí=Ä~ÅáääáK


86


Diagnosis

qÜÉ=íÜêÉÉ=Çá~ÖåçëíáÅ=ëáÖåë=çÑ= äÉéêçëó=~êÉ=ÜóéçéáÖãÉåíÉÇ=ëâáå= äÉëáçåëïáíÜ= äçëë= çÑ= ëÉåë~íáçåI= íÜáÅâÉåáåÖ= çÑ= éÉêáéÜÉê~ä= åÉêîÉë= ~åÇ= ëâáåJëãÉ~êéçëáíáîáíó= Ñçê= íÜÉ= ~ÅáÇJÑ~ëí= Ä~ÅáääáK= få= éìêÉäó= åÉìêçé~íÜáÅ= Ñçêãë= ïÜáÅÜ= ~êÉëÉÉå=áå=íÜÉ=íìÄÉêÅìäçáÇ=Ñçêã=~åÇI=äÉëë=çÑíÉåI=áå=äÉéêçã~íçìë=äÉéêçëóI=åÉêîÉ


87

Late reversal reaction in a patient with lepromatous leprousneuropathy

Peter was first seen at the age of 38. He was a school teacher living in French

Polynesia. He was on a summer vacation in Europe and decided to consult for

numbness in the hands. He was in good general condition. Hand numbness had

started gradually on one side and subsequently affected both sides approximately 3

years before referral. Upon examination the patient had not noticed a loss of

temperature, pain and light touch sensation over the lower extremities, up to the

mid-leg. In the upper limbs sensory loss extended up to the elbows. Proprioception

was preserved. Muscle strength and tendon reflexes were normal. The superficial

radial nerve was enlarged and firm upon palpation. There were no skin lesions. A

biopsy of the superficial radial nerve revealed multibacillar leprosy. The patient was

put on a treatment regimen of rifampicin and dapsone for more than 2 years. His

neurological condition improved although he retained some sensory loss 4 years

later.

Six years after the diagnosis and start of the treatment, the patient experienced rapid

worsening of his neurological condition with pains in all four limbs and walking

difficulty. He had some fever and arthralgia. He had bilateral weakness of foot

dorsiflexion and loss of sensation over both legs. A biopsy of the superficial peroneal

nerve was performed. It showed loss of axons associated with endoneurial

granulomas without any detectable Mycobacterium leprae. The patient’s condition

improved quickly after treatment with corticosteroids.

This patient had a late reversal reaction after treatment of lepromatous leprous

neuropathy.


Äáçéëó= áë= íÜÉ= çåäó= ï~ó= íç= êÉ~ÅÜ= ~= Çá~ÖåçëáëK= fí= áë= ÉëéÉÅá~ääó= ìëÉÑìä= áåÅçìåíêáÉë= ïÜÉêÉ= äÉéêçëó= áë= åçí= ÅçããçåK= få= ëìÅÜ= ÅçìåíêáÉëI= áí= ãìëí= ÄÉåçíÉÇ= íÜ~í= äÉéêçìë= åÉìêçé~íÜó= ã~ó= ÄÉÅçãÉ= ëóãéíçã~íáÅ= óÉ~êë= çêÇÉÅ~ÇÉë= ~ÑíÉê= íÜÉ= é~íáÉåí= Ü~ë= ãçîÉÇ= Ñêçã= ÉåÇÉãáÅ= ~êÉ~ëK= få= ÅçìåíêáÉëïÜÉêÉ= äÉéêçëó= áë= ÉåÇÉãáÅI= åÉêîÉ= Äáçéëó= ã~ó= ÄÉ= ìëÉÑìä= áå= ÇáÑÑÉêÉåíá~íáåÖäÉéêçìë=åÉìêçé~íÜó=Ñêçã=~=åÉìêçé~íÜó=çÑ=çíÜÉê=çêáÖáåëI=áåÅäìÇáåÖ=Çá~ÄÉíáÅåÉìêçé~íÜóI=ÜÉêÉÇáí~êó=ëÉåëçêó=åÉìêçé~íÜáÉë=çê=~ãóäçáÇ=åÉìêçé~íÜó=ïÜáÅÜÅ~å=äÉ~Ç=íç=ëÉåëçêó=~åÇ=íêçéÜáÅ=ã~åáÑÉëí~íáçåë=íÜ~í=ã~ó=ÄÉ=ãáëí~âÉå=ÑçêäÉéêçìë=åÉìêçé~íÜóK=wáÉÜäÛë=ëí~áåáåÖ=çÑ=é~ê~ÑÑáåJÉãÄÉÇÇÉÇ=ëÉÅíáçåë=éÉêãáíëîáëì~äáë~íáçå=çÑ=Ä~Åáääá= áå= íÜÉ=éäìêáÄ~Åáää~ê= Ñçêãë=çÑ= íÜÉ=ÇáëÉ~ëÉK=_~Åáääá=~êÉëÅ~êÅÉ= çê= ~ÄëÉåí= Ñêçã= åÉêîÉë= ïáíÜ= íìÄÉêÅìäçáÇ= äÉéêçëó= ~åÇ= áå= êÉîÉêë~äêÉ~ÅíáçåK=

Treatment

få= ãìäíáÄ~Åáää~êó= äÉéêçëóI= íÜÉ= ëí~åÇ~êÇ= íêÉ~íãÉåí= êÉÖáãÉå= áë= êáÑ~ãéáÅáåSMMãÖ=çåÅÉ=~=ãçåíÜI=Ç~éëçåÉ=NMMãÖ=Ç~áäóI=~åÇ=ÅäçÑ~òáãáåÉ=PMMãÖ=çåÅÉ~= ãçåíÜ= ~åÇ= RMãÖ= Ç~áäó= Ñçê= NO= ãçåíÜëI= ~äíÜçìÖÜ= áå= ëçãÉ= é~íáÉåíëíêÉ~íãÉåí=ïáíÜ=íÜÉëÉ=ÇêìÖë=ã~ó=ÄÉÅçãÉ=åÉÅÉëë~êó=Ñçê=ìé=OQ=ãçåíÜëK=fåé~ìÅáÄ~Åáää~êó= äÉéêçëóI= íÜÉ=ëí~åÇ~êÇ=êÉÖáãÉå= áë=êáÑ~ãéáÅáå=SMMãÖ=çåÅÉ=~ãçåíÜ=~åÇ=Ç~éëçåÉ=NMMãÖ=Ç~áäó=Ñçê=S=ãçåíÜëK=jçêÉçîÉêI=íÜÉëÉ=êÉÖáãÉåëëÜçï= ~= ÜáÖÜ= ÑêÉèìÉåÅó= çÑ= êÉ~Åíáçå~ä= ëí~íÉë= ÄçíÜ= ÇìêáåÖ= ~åÇ= ~ÑíÉêíêÉ~íãÉåíK=

m~íáÉåíë=ïÜç=ÇÉîÉäçé=ÇÉä~óÉÇ=åÉêîÉ=áãé~áêãÉåí=ãçåíÜë=çê=óÉ~êë=~ÑíÉêíêÉ~íãÉåíI=ïÜáÅÜ=Å~ååçí=ÄÉ=Éñéä~áåÉÇ=Äó=êÉä~éëÉëI=ãìëí=ÄÉ=íêÉ~íÉÇ=ïáíÜ=~ãçêÉ= ~ÖÖêÉëëáîÉ= ëÅÜÉÇìäÉ= çÑ= ÅçêíáÅçëíÉêçáÇë= EÜáÖÜÉê= ÇçëÉ= ~åÇ= äçåÖÇìê~íáçåFK

Lyme disease

ióãÉ=ÇáëÉ~ëÉ=áë=~=ãìäíáëóëíÉã=áääåÉëë=íÜ~í=~ÑÑÉÅíë=íÜÉ=ëâáåI=àçáåíëI=ÜÉ~êí~åÇ= åÉêîçìë= ëóëíÉãI= Å~ìëÉÇ= Äó= ~= íáÅâJíê~åëãáííÉÇ= ëéáêçÅÜ~ÉíÉI= _çêêÉäá~ÄìêÖÇçêÑÉêáK= qÜÉ= Ñáêëí= ÇÉëÅêáéíáçå= çÑ= íáÅâJÄáíÉJ~ëëçÅá~íÉÇ= é~ê~äóëáë= ~åÇãÉåáåÖáíáë=~ééÉ~êÉÇ=áå=bìêçéÉ=NNJNPI=Äìí=íÜÉ=êÉÅçÖåáíáçå=çÑ=ióãÉ=ÇáëÉ~ëÉ~ë= ~= ëÉé~ê~íÉ= Éåíáíó= ï~ë= ã~ÇÉ= Äó= píÉÉêÉ= Éí= ~ä áå= NVTT= áå= ióãÉI


88


`çååÉÅíáÅìí= Erp^F= NQK= `Éêí~áå= ÇáÑÑÉêÉåÅÉë= Ü~îÉ= ÄÉÉå= åçíÉÇ= ÄÉíïÉÉå^ãÉêáÅ~å= ~åÇ= bìêçéÉ~å= áëçä~íÉë= çÑ= _çêêÉäá~= ÄìêÖÇçêÑÉêá áå= ãçêéÜçäçÖóIçìíÉê=ëìêÑ~ÅÉ=éêçíÉáåëI=éä~ëãáÇë=~åÇ=ak^=ÜçãçäçÖóI=ïÜáÅÜ=ã~ó=~ÅÅçìåí~äëç=Ñçê=ëçãÉ=ÅäáåáÅ~ä=ÇáÑÑÉêÉåÅÉë=NRK=

fñçÇáÅ= íáÅâë=~êÉ= íÜÉ=ìëì~ä= îÉÅíçêëK=qáÅâë= ÑÉÉÇ=çåÅÉ=ÇìêáåÖ= íÜÉ= íÜêÉÉëí~ÖÉë=çÑ=íÜÉáê=ìëì~ä=íïçJóÉ~ê=äáÑÉK=i~êî~ä=íáÅâë=í~âÉ=çåÉ=ÄäççÇ=ãÉ~ä=áå=ä~íÉëìããÉêI=åóãéÜë=ÑÉÉÇ=ÇìêáåÖ=íÜÉ=ÑçääçïáåÖ=ëéêáåÖ=~åÇ=É~êäó=ëìããÉêI=~åÇ~Çìäíë=ÇìêáåÖ= íÜ~í=~ìíìãåK= få= íÜÉ=råáíÉÇ=pí~íÉëI= íÜÉ=éêÉÑÉêêÉÇ=Üçëí= ÑçêÄçíÜ= íÜÉ= ä~êî~ä= ~åÇ= åóãéÜ~ä= ëí~ÖÉë= áë= íÜÉ= ïÜáíÉJÑççíÉÇ= ãçìëÉI= ïÜáäÉïÜáíÉJí~áäÉÇ= ÇÉÉê= ~êÉ= íÜÉ= éêÉÑÉêêÉÇ= Üçëí= çÑ= ~Çìäí= áñçÇÉëK= få= bìêçéÉIíÜçìë~åÇë=çÑ=åÉï=Å~ëÉë=çÅÅìê=É~ÅÜ=ëìããÉêI=é~êíáÅìä~êäó=áå=ÅÉåíê~ä=~åÇåçêíÜÉêå= bìêçéÉK= få= íÜÉ= råáíÉÇ= pí~íÉëI= ióãÉ= ÄçêêÉäáçëáë= áë= ã~áåäóêÉéçêíÉÇ=Ñêçã=íÜêÉÉ=~êÉ~ëW=íÜÉ=kçêíÜÉ~ëíI=jáÇïÉëíI=~åÇ=áå=`~äáÑçêåá~=~åÇlêÉÖçå=áå=íÜÉ=tÉëíK


qÜÉ=ÅçìêëÉ=çÑ=íÜÉ=ÇáëÉ~ëÉ=Ñçääçïë=íÜêÉÉ=ëí~ÖÉë=~ë=çìíäáåÉÇ=ÄÉäçïK

Stage 1qÜÉ= íóéáÅ~ä= é~íáÉåí= Ñáêëí= Ü~ë= ÉêóíÜÉã~= ãáÖê~åëI= ëçãÉíáãÉë= ÑçääçïÉÇ

ëÉîÉê~ä= ïÉÉâë= çê= ãçåíÜë= ä~íÉê= Äó= ãÉåáåÖáíáë= çê= Ñ~Åá~ä= é~äëóI= ~åÇ= çÑíÉåIãçåíÜë= ä~íÉê= Äó= ~êíÜêáíáëK= içÅ~äáëÉÇ= ÉêóíÜÉã~= ãáÖê~åë= êÉëìäíë= Ñêçã= äçÅ~äëéêÉ~ÇáåÖ=çÑ=_çêêÉäá~=ÄìêÖÇçêÑÉêá áå=íÜÉ=ëâáåK=fí=ëí~êíë=~ë=~=êÉÇ=ã~ÅìäÉ=çêé~éìäÉ=~í= íÜÉ=ëáíÉ=çÑ= íÜÉ=ÄáíÉ=~åÇ=Éñé~åÇë= íç= Ñçêã=~= ä~êÖÉ= êÉÇ= êáåÖ=ïáíÜÅÉåíê~ä= ÅäÉ~êáåÖK= fí= áë= ~ÅÅçãé~åáÉÇ= Äó= ÑÉîÉêI= ãáåçê= Åçåëíáíìíáçå~äëóãéíçãëI=çê=êÉÖáçå~ä=äóãéÜ~ÇÉåçé~íÜóK=

Stage 2táíÜáå=Ç~óë=çê=ïÉÉâë=~ÑíÉê=áåçÅìä~íáçåI=íÜÉ=ëéáêçÅÜ~ÉíÉ=ã~ó=ëéêÉ~Ç=áå

íÜÉ=é~íáÉåíÛë=ÄäççÇ=íç=ã~åó=ëáíÉëX=ëéáêçÅÜ~ÉíÉë=Ü~îÉ=ÄÉÉå=êÉÅçîÉêÉÇ=ÑêçãÄäççÇ=ÇìêáåÖ=íÜáë=ëí~ÖÉ=~åÇ=Ñêçã=ã~åó=çêÖ~åëK=pÉÅçåÇ~êó=~ååìä~ê=äÉëáçåëIïÜáÅÜ= êÉëÉãÄäÉ= íÜÉ= éêáã~êó= ÉêóíÜÉã~= ãáÖê~åë= çÅÅìê= áå= ~Äçìí= Ü~äÑ= çÑé~íáÉåíë= áå= ~ëëçÅá~íáçå= ïáíÜ= ãáÖê~íçêó= ãìëÅìäçëâÉäÉí~ä= ~åÇ= àçáåí= é~áå= NSKtáÇÉäó=ÇáëëÉãáå~íÉÇ=ëóãéíçãë=ëÉÉã=íç=ÄÉ=ãçêÉ=Åçããçå=áå=íÜÉ=råáíÉÇpí~íÉë= íÜ~å= áå=bìêçéÉK=_ó= íÜáë= íáãÉI= íÜÉ=Üçëí=ëí~êíë= íç=ÇÉîÉäçé=~=ëíêçåÖ


89


áããìåÉ=êÉëéçåëÉ=íç=_çêêÉäá~=ÄìêÖÇçêÑÉêá ~åíáÖÉåë=íÜ~í=êÉëìäí=áå=ÇÉëíêìÅíáçåçÑ=ëéáêçÅÜ~ÉíÉë=Äó=ÅçãéäÉãÉåí=~Åíáî~íáçå=íÜêçìÖÜ=áããìåÉ=ÅçãéäÉñÉëK=

^ÑíÉê=ëÉîÉê~ä=ïÉÉâë=çê=ãçåíÜëI=NRJOMB=çÑ=é~íáÉåíë=ÇÉîÉäçé=åÉìêçäçÖáÅëáÖåëI= çÑíÉå= ê~ÇáÅìä~ê= é~áå= çÑ= ~= ÄìêåáåÖ= íóéÉI= ïÜáÅÜ= ã~ó= çê= ã~ó= åçí= ÄÉ~ëëçÅá~íÉÇ=ïáíÜ=ïÉ~âåÉëëI=~åÇ=ïáíÜ=äáííäÉ=çê=åç=ÅäáåáÅ~ä=ëáÖåë=çÑ=ãÉåáåÖáíáëEÜ ëÉÉ=Å~ëÉ=ëíìÇó=ÄÉäçïFK=m~áåÑìä=ãÉåáåÖçê~ÇáÅìäáíáë=áë=çÑíÉå=Å~ääÉÇ=íÜÉ


90

Lyme disease

John, a 59-year-old patient, had been treated with insulin for 10 years for type 1

diabetes, which had remained uncomplicated so far. At the end of May 2002 he

started to complain of spontaneous burning pains in the right leg and inner and

plantar aspects of the foot. Pain was present day and night, preventing sleep. Within

a few days, pain extended over the dorsal aspect of the foot and the patient noticed

some walking difficulty due to weakness of the right foot. Examination showed a

sensory loss of pain, light touch, temperature and pinprick sensation over the dorsal

and plantar aspects of the right foot and minimal weakness of foot dorsiflexion on

the same side. The ankle jerk was abolished. There was no other clinical abnormality.

The patient was in a good general condition. Electrophysiological examination

showed that the sural nerve action potential was abolished on the right side and

normal on the left side (10.93μV) with a normal nerve conduction velocity (40m/s).

A biopsy of the right superficial peroneal nerve revealed inflammatory infiltrates

made up of mononuclear cells associated with axonal degeneration (Figure 4).

Cerebrospinal fluid (CSF) examination showed pleocytosis at 15 cells/ml with a

mixture of lymphocytes, polymorphonuclear cells, monocytes and macrophages.

The protein level was 0.56g/L with oligoclonal bands.

After these findings of inflammatory nerve lesions and pleocytosis of the CSF, Lyme

disease was queried and the patient was then questioned about tick bites. He

revealed that he had had several tick bites months before without secondary

erythema. Serological tests for Borrelia burgdorferi were positive in the blood and in

the CSF. The patient recovered completely after treatment with ceftriaxone.


_~ååï~êíÜÛë= ëóåÇêçãÉK= jÉåáåÖáíáë= áë= íÜÉ= ãçëí= Åçããçå= åÉìêçäçÖáÅ~ä~Äåçêã~äáíó=áå=ióãÉ=ÇáëÉ~ëÉK=fí=Å~å=ÄÉ=íÜÉ=Ñáêëí=ëóãéíçã=çÑ=ióãÉ=ÇáëÉ~ëÉIÄìí=áë=éêÉÅÉÇÉÇ=áå=ãçëí=Å~ëÉë=Äó=ÉêóíÜÉã~=ãáÖê~åëI=íÜÉå=ìëì~ääó=ÄÉÖáåë~ÑíÉê=íÜÉ=ëâáå=äÉëáçåë=êÉëçäîÉK=m~éáääçÉÇÉã~=~åÇ=áåÅêÉ~ëÉÇ=ÅÉêÉÄêçëéáå~äÑäìáÇ= E`pcF=éêÉëëìêÉ=Å~å=çÅÅìêK=`pc=Éñ~ãáå~íáçå=êÉîÉ~äë=~= äóãéÜçÅóíáÅéäÉçÅóíçëáëI= ìëì~ääó= ~= ÑÉï= íÉåë=çê=ÜìåÇêÉÇë=çÑ= ÅÉääë=éÉê=ãäI=ïáíÜ=~=ãáäÇÉäÉî~íáçå= çÑ= éêçíÉáå= ïáíÜ= ~= ÜáÖÜ= éêçéçêíáçå= çÑ= áããìåçÖäçÄìäáåë= ~åÇçäáÖçÅäçå~ä= Ä~åÇëK= qÜÉ= `pc= ÖäìÅçëÉ= áë= ìëì~ääó= åçêã~ä= Äìí= Å~å= ÄÉ= äçïKpéáêçÅÜ~ÉíÉë=Ü~îÉ=ÄÉÉå=ÅìäíìêÉÇ=Ñêçã=`pc=çå=ëÉîÉê~ä=çÅÅ~ëáçåëK=

jìäíáÑçÅ~ä=ëéáå~ä=êççí=çê=Åê~åá~ä=åÉêîÉ=áåîçäîÉãÉåí=çÑíÉå=ÇÉîÉäçéë=ïáíÜáå~= ÑÉï= Ç~óë= çê= ïÉÉâëI= ïáíÜ= ìåáJ= çê= Äáä~íÉê~ä= Ñ~Åá~ä= é~äëó= ~åÇ= ~ëóããÉíêáÅëÉåëçêáãçíçê=ê~ÇáÅìäçåÉìêçé~íÜóK=`ê~åá~ä=åÉìêçé~íÜó=áë=éêÉëÉåí=áå=RMB=çÑé~íáÉåíë= ïáíÜ= åÉìêçäçÖáÅ~ä= ~Äåçêã~äáíáÉëK= c~Åá~ä= é~äëó= áë= çÑíÉå= Äáä~íÉê~äKlíÜÉê= Åê~åá~ä= åÉêîÉë= Å~å= ÄÉ= ~ÑÑÉÅíÉÇI= áå= ~ëëçÅá~íáçå= ïáíÜ= ãÉåáåÖáíáëK`ê~åá~ä= åÉêîÉ= é~äëáÉë= êÉëçäîÉ= ïáíÜáå= ïÉÉâë= çê= ãçåíÜëI= ëçãÉíáãÉëáåÅçãéäÉíÉäóK=rêáå~êó=~åÇ=~å~ä=ëéÜáåÅíÉê=ÇáëíìêÄ~åÅÉ=ã~ó=~äëç=çÅÅìêK=


91

Figure 4. One-micron-thick plastic section of a biopsy

specimen of the superficial peroneal nerve from a patient who

presented with mononeuritis related to Lyme disease. Note

the massive inflammatory infiltration (asterisks) and axonal

degeneration of nerve fibres. (Toluidine blue staining. Bar:

20μm.)

*

*


mÉêáéÜÉê~ä= åÉìêçé~íÜó= çÅÅìêë= áå= ~ééêçñáã~íÉäó= Ü~äÑ= çÑ= é~íáÉåíë= ïáíÜãÉåáåÖáíáëK= cçÅ~ä= çê= ãìäíáÑçÅ~ä= áåîçäîÉãÉåí= áë= íÜÉ= ãçëí= ÅçããçåéêÉëÉåí~íáçåK= `çããçå= é~ííÉêåë= áåÅäìÇÉ= é~áåÑìä= íÜçê~Åç~ÄÇçãáå~äëÉåëçêó= ê~ÇáÅìäáíáëI= ~ëëçÅá~íÉÇ= ïáíÜ= Çáëí~ä= áåîçäîÉãÉåíK= _çíÜ= ïÉ~âåÉëë~åÇ=ëÉåëçêó=äçëë=áãéêçîÉ=ïáíÜáå=~=ÑÉï=ïÉÉâëI=Äìí=êÉÅçîÉêó=ã~ó=í~âÉ=ìéíç=ëÉîÉê~ä=ãçåíÜë=~åÇ=çÑíÉå=êÉã~áåë=áåÅçãéäÉíÉ=NTK

bäÉÅíêçéÜóëáçäçÖáÅ~ä=íÉëíáåÖ=çÑ=é~íáÉåíë=ïáíÜ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=Ü~ëëÜçïå=ÉîáÇÉåÅÉ=ÄçíÜ=çÑ=ÇÉãóÉäáå~íáçå=~åÇ=çÑ=~ñçå~ä=ÇÉÖÉåÉê~íáçåI=Äìíìëì~ääó=~ñçå~ä=äÉëáçåë=éêÉÇçãáå~íÉK=eáëíçäçÖáÅ~ääóI=íÜÉ=åÉêîÉ=äÉëáçåë=~êÉ~ëëçÅá~íÉÇ= ïáíÜ= äóãéÜçéä~ëãçÅóíáÅ= áåÑä~ãã~íçêó= áåÑáäíê~íÉë= íÜ~íéêÉÇçãáå~íÉ=áå=íÜÉ=åÉêîÉ=êççíëK=qÜÉ=éêÉëÉåÅÉ=çÑ=_çêêÉäá~=ÄìêÖÇçêÑÉêá Ü~ëåçí=ÄÉÉå=ÅçåîáåÅáåÖäó=ÇçÅìãÉåíÉÇ=áå=íÜÉ=åÉêîÉëK=

`~êÇá~Å= áåîçäîÉãÉåí= çÅÅìêë= áå= QJUB= çÑ= é~íáÉåíëK= qÜÉó= áåÅäìÇÉÑäìÅíì~íáåÖ=~íêáçîÉåíêáÅìä~ê=åçÇÉ=ÄäçÅâI=ãáäÇ= äÉÑí=îÉåíêáÅìä~ê=ÇóëÑìåÅíáçåIçêI= ê~êÉäóI= Å~êÇáçãÉÖ~äó= çê= Ñ~í~ä= é~åÅ~êÇáíáëK= qÜÉ= Çìê~íáçå= çÑ= Å~êÇá~Å~Äåçêã~äáíó= áë= ìëì~ääó= ÄêáÉÑ= ~åÇ= ÇçÉë= åçí= åÉÅÉëëáí~íÉ= íÜÉ= éÉêã~åÉåíáåëÉêíáçå=çÑ=~=é~ÅÉã~âÉê=NUK

Stage 3^êíÜêáíáë=çÅÅìêë= áå= íê~åëáÉåí=ÉéáëçÇÉëI=~=ãÉ~å=çÑ=S=ãçåíÜë=~ÑíÉê= íÜÉ

çåëÉí=çÑ= íÜÉ=ÇáëÉ~ëÉK=pìÅÜ=ÉéáëçÇÉë=~ÑÑÉÅí=~êçìåÇ=SMB=çÑ=é~íáÉåíë= áåíÜÉ= råáíÉÇ= pí~íÉë= ~åÇ= ~êÉ= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~ëóããÉíêáÅ= çäáÖç~êíáÅìä~ê~êíÜêáíáë= ÉëéÉÅá~ääó= áå= íÜÉ= âåÉÉX= çåÉ= çê= ~= ÑÉï= àçáåíë= ã~ó= ÄÉ= ~ÑÑÉÅíÉÇKpéáêçÅÜ~ÉíÉë=Ü~îÉ=ÄÉÉå=çÅÅ~ëáçå~ääó= ÅìäíìêÉÇ= Ñêçã= àçáåí= ÑäìáÇK=^êíÜêáíáëëÉÉãë=äÉëë=Åçããçå=áå=bìêçéÉK

^=î~êáÉíó=çÑ=ä~íÉ=ëóåÇêçãÉë=~ÑÑÉÅíáåÖ=íÜÉ=ÅÉåíê~ä=åÉêîçìë=ëóëíÉã=Ü~îÉÄÉÉå=ÇÉëÅêáÄÉÇI=áåÅäìÇáåÖ=ëé~ëíáÅ=é~ê~é~êÉëáëI=~í~ñá~I=êÉä~éëáåÖ=ãìäíáéäÉëÅäÉêçëáëJäáâÉ= áääåÉëëI= Ää~ÇÇÉê= ÇóëÑìåÅíáçåI= ÅçÖåáíáîÉ= áãé~áêãÉåíIÇÉãÉåíá~I= ~åÇ= ëìÄ~ÅìíÉ= ÉåÅÉéÜ~äáíáëK= ^äíÜçìÖÜ= íÜÉëÉ= é~íáÉåíë= Ü~ÇëÉêçäçÖáÅ~ä= ÉîáÇÉåÅÉ= çÑ= ióãÉ= ÇáëÉ~ëÉI= íÜÉó= ÇáÇ= åçí= Ü~îÉ= áåíê~íÜÉÅ~äëóåíÜÉëáë= çÑ= ~åíáÄçÇó= íç= _çêêÉäá~= ÄìêÖÇçêÑÉêáK= qÜìëI= íÜÉêÉ= áë= åçÅçåîáåÅáåÖ=ÉîáÇÉåÅÉ=Ñçê=ÅÉåíê~ä=åÉêîçìë=ëóëíÉã=ÅçãéäáÅ~íáçåë=çÑ=ióãÉÇáëÉ~ëÉK


92


Diagnosis

cêçã= ~= åÉìêçäçÖáÅ~ä= éçáåí= çÑ= îáÉïI= íÜÉ= éêÉëÉåÅÉ= çÑ= ~= ëìÄ~ÅìíÉãÉåáåÖçê~ÇáÅìäçåÉìêáíáë= ïáíÜ= Ñ~Åá~ä= é~äëó= ~åÇ= ëáÖåë= ~åÇ= ëóãéíçãëëìÖÖÉëíáåÖ= ~= ãìäíáÑçÅ~ä= áåîçäîÉãÉåí= çÑ= íÜÉ= éÉêáéÜÉê~ä= åÉêîçìë= ëóëíÉãI= áëÜáÖÜäó= ëìÖÖÉëíáîÉ= çÑ= ióãÉ= ÄçêêÉäáçëáëK= pÉêçäçÖó= áë= íÜÉ= çåäó= éê~ÅíáÅ~ää~Äçê~íçêó=~áÇ=áå=Çá~ÖåçëáëI=Äìí=ëÉêçäçÖáÅ=íÉëíáåÖ=áë=åçí=óÉí=ëí~åÇ~êÇáëÉÇ=~åÇíÜÉ=êÉëìäíë=Ñêçã=ÇáÑÑÉêÉåí=ä~Äçê~íçêáÉë=ã~ó=î~êóK=qÜÉ=éÜóëáÅá~å=ãìëí=ÄÉ=~ï~êÉçÑ=Ñ~äëÉJåÉÖ~íáîÉ=~åÇI=ãçêÉ=ÅçããçåäóI=Ñ~äëÉJéçëáíáîÉ=êÉëìäíë=NSK=qáíêÉë=ëÜçìäÇáåÅêÉ~ëÉ= ÑçìêJÑçäÇ= çê= ãçêÉ= ÄÉíïÉÉå= íÜÉ= ÉêóíÜÉã~= ãáÖê~åë= éÜ~ëÉ= ~åÇëìÄëÉèìÉåí= åÉìêçäçÖáÅ~ä= áåîçäîÉãÉåíK= j~åó= é~íáÉåíë= Ü~îÉ= ~ëóãéíçã~íáÅ_çêêÉäá~=ÄìêÖÇçêÑÉêá=áåÑÉÅíáçåI=~åÇ=Éèì~ääóI=~=Ñ~äëÉJéçëáíáîÉ=êÉëìäí=Å~å=çÅÅìêIé~êíáÅìä~êäó= ïáíÜ= áããìåçÖäçÄìäáå= j= EfÖjFI= ÄçíÜ= áå= ÜÉ~äíÜó= ëìÄàÉÅíë= ~åÇ= áåé~íáÉåíë=ïáíÜ=~=î~êáÉíó=çÑ=çíÜÉê=ÇáëÉ~ëÉëK=oÉÑáåÉãÉåíë=çÑ=ëÉêçäçÖáÅ=ãÉíÜçÇëã~ó=ÄÉ=ÜÉäéÑìä= áå= íÜÉ=ÑìíìêÉ= íç=ÇáÑÑÉêÉåíá~íÉ=é~íáÉåíë=ïáíÜ=êÉëáÇì~ä=éçëáíáîáíó~åÇ=~=Ñ~äëÉJéçëáíáîÉ=êÉëìäí=Ñêçã=íÜçëÉ=ëìÑÑÉêáåÖ=ïáíÜ=ióãÉ=ÇáëÉ~ëÉK

Treatment

fí=ãìëí=ÄÉ=âÉéí= áå=ãáåÇ=íÜ~í=ióãÉ=ÇáëÉ~ëÉ= áë=~=ÄÉåáÖå=ÅçåÇáíáçåK= få=~ÑçääçïJìé= ëíìÇó= çÑ= TO= é~íáÉåíë= ìé= íç= OT= óÉ~êë= ~ÑíÉê= ìåíêÉ~íÉÇ= ióãÉåÉìêçÄçêêÉäáçëáëI=åçåÉ=çÑ=íÜÉ=é~íáÉåíë=ëÜçïÉÇ=ëáÖåë=çÑ=~ÅíáîÉ=ÇáëÉ~ëÉ=çêÇáëÉ~ëÉ=éêçÖêÉëëáçå=íç=ÅÜêçåáÅ=ióãÉ=åÉìêçÄçêêÉäáçëáë=NVK=qêÉ~íãÉåí=ïáíÜÜáÖÜ=ÇçëÉë=çÑ=éÉåáÅáääáå=ÖáîÉë=ÖççÇ=êÉëìäíë=~í=ëí~ÖÉ=NI=Äìí=íÜÉ=êÉëìäíë=~êÉåçí= ~ë= ÖççÇ= áå= é~íáÉåíë= ïáíÜ= ëí~ÖÉ= O= åÉìêçäçÖáÅ= ~Äåçêã~äáíáÉëI= ~åÇ= áåé~íáÉåíë= ïáíÜ= ~êíÜêáíáëK= _çêêÉäá~= ÄìêÖÇçêÑÉêá ëÉÉãë= ÜáÖÜäó= ëÉåëáíáîÉ= íçíÉíê~ÅóÅäáåÉI=~ãéáÅáääáåI=ÅÉÑíêá~ñçåÉI=Äìí=çåäó=ãçÇÉê~íÉäó= íç=éÉåáÅáääáåK=cçêÉ~êäó=ióãÉ=ÇáëÉ~ëÉI= äçÅ~äáëÉÇ=ëí~ÖÉ=N=çê=ÇáëëÉãáå~íÉÇ=ëí~ÖÉ=O=áåÑÉÅíáçåIçê~ä=íÉíê~ÅóÅäáåÉ=áë=ÖÉåÉê~ääó=~å=ÉÑÑÉÅíáîÉ=~åíáÄáçíáÅ=OMK=açñóÅóÅäáåÉI=~=äçåÖJ~ÅíáåÖ= íÉíê~ÅóÅäáåÉ= íÜ~í=~ÅÜáÉîÉë=ÄÉííÉê= íáëëìÉ= äÉîÉäëI=ã~ó=ÄÉ=éêÉÑÉê~ÄäÉKqÜÉ=íêÉ~íãÉåí=ëÜçìäÇ=ÄÉ=~ÇãáåáëíÉêÉÇ=Ñçê=NMJPM=Ç~óëK=qÜÉ=êÉÅçããÉåÇÉÇÇçë~ÖÉ= Ñçê= NQ= Ç~óë= áë= áKîK= éÉåáÅáääáå= OM= ãáääáçå= r= Ç~áäó= áå= ÇáîáÇÉÇ= ÇçëÉëIÅÉéÜ~äçëéçêáåI= ÉKÖK= áKîK= ÅÉÑíêá~ñçåÉ= OÖ= çåÅÉ= ~= Ç~óI= çê= çê~ä= ÇçñóÅóÅäáåÉNMMãÖ=íïáÅÉ=Ç~áäóK=açëÉ=ÑáåÇáåÖ=íêá~äë=êÉÖ~êÇáåÖ=íÜÉ=Çìê~íáçå=çÑ=íêÉ~íãÉåíÜ~îÉ=åÉîÉê=ÄÉÉå=ÅçåÇìÅíÉÇK=få=îáÉï=çÑ=íÜÉ=äçï=êáëâ=çÑ=ióãÉ=ÇáëÉ~ëÉ=~ÑíÉê~=êÉÅçÖåáëÉÇ=ÇÉÉêJíáÅâ=ÄáíÉ=~åÇ=íÜÉ=ìåÅÉêí~áå=ÉÑÑÉÅíáîÉåÉëë=çÑ=éêçéÜóä~ÅíáÅ~åíáãáÅêçÄá~ä=~ÖÉåíëI=êçìíáåÉ=~åíáãáÅêçÄá~ä=éêçéÜóä~ñáë= Ñçê=éÉêëçåë=ïáíÜ=~êÉÅçÖåáëÉÇ=ÇÉÉêJíáÅâ=ÄáíÉ=áë=åçí=áåÇáÅ~íÉÇK


93


Infection with retroviruses

mÉêáéÜÉê~ä=åÉêîÉ=äÉëáçåë=~êÉ=Åçããçåäó=~ëëçÅá~íÉÇ=ïáíÜ=Üìã~å=êÉíêçîáê~äáåÑÉÅíáçåI=ïÜáÅÜ=áåÅäìÇÉë=áåÑÉÅíáçå=ïáíÜ=íÜÉ=Üìã~å=áããìåçÇÉÑáÅáÉåÅó=îáêìëEefsFI= íÜÉ= Å~ìë~ä= ~ÖÉåí= çÑ= ^fapI= ~åÇI= äÉëë= ÅçããçåäóI= íÜÉ= Üìã~å= qJäóãéÜçíêçéáÅ= îáêìë= íóéÉ= f= EeqisJNFI= íÜÉ= Å~ìë~ä= ~ÖÉåí= çÑ= íêçéáÅ~äãóÉäçåÉìêçé~íÜóK

Neuropathies in HIV infection

^äíÜçìÖÜ= íÜÉ= ~ååì~ä= åìãÄÉê= çÑ= åÉï= efs= áåÑÉÅíáçåë= Ü~ë= ÄÉÉå= ëíÉ~ÇáäóÇÉÅäáåáåÖ=ëáåÅÉ=íÜÉ=ä~íÉ=NVVMëI=íÜáë=ÇÉÅêÉ~ëÉ=áë=çÑÑëÉí=Äó=íÜÉ=êÉÇìÅíáçå=áå^fapJêÉä~íÉÇ= ÇÉ~íÜë= ÇìÉ= íç= íÜÉ= ëáÖåáÑáÅ~åí= áãéêçîÉãÉåíë= áå= ~åíáêÉíêçîáê~äíÜÉê~éó=EôqF=çîÉê=íÜÉ=é~ëí=ÑÉï=óÉ~êëK=eáÖÜäó=~ÅíáîÉ=~åíáêÉíêçîáê~ä=íÜÉê~éóEe^ôqF= ~åÇ= ÅçãÄáåÉÇ= ~åíáêÉíêçîáê~ä= íÜÉê~éó= EÅôqF= Ü~îÉ= Éå~ÄäÉÇëìëí~áåÉÇ= ëìééêÉëëáçå= çÑ= efs= êÉéäáÅ~íáçå= ~åÇ= êÉÅçîÉêó= çÑ= àQH= qJÅÉääÅçìåíëX=ÜçïÉîÉêI=íÜÉêÉ=áë=ëíáää=åç=ÅìêÉ=Ñçê=efs=áåÑÉÅíáçå=çå=íÜÉ=Üçêáòçå=ONI=OOK

póãéíçã~íáÅ= åÉìêçé~íÜó= ~ÑÑÉÅíë= ~å= Éëíáã~íÉÇ= RJNMB= çÑ= efsJáåÑÉÅíÉÇé~íáÉåíëK=^=ïáÇÉ=î~êáÉíó=çÑ=åÉìêçé~íÜáÉë=Ü~îÉ=ÄÉÉå=çÄëÉêîÉÇ=áå=íÜÉ=ÅçìêëÉçÑ=efs=áåÑÉÅíáçåI=~åÇ=áå=ëçãÉ=Å~ëÉëI=åÉìêçé~íÜó=Å~å=ÄÉ=íÜÉ=Ñáêëí=~åÇ=çåäóã~åáÑÉëí~íáçå=çÑ=efs=áåÑÉÅíáçåK=qÜÉ=åÉìêçé~íÜáÉë=çÄëÉêîÉÇ=áå=efs=é~íáÉåíëáåÅäìÇÉ=áåÑä~ãã~íçêó=éçäóåÉìêçé~íÜó=çÑ=íÜÉ=dìáää~áåJ_~êê¨=íóéÉI=ãìäíáÑçÅ~äåÉìêçé~íÜóI=ãÉåáåÖçê~ÇáÅìäçåÉìêáíáëI=~ÅìíÉ=ìåáJ=çê=Äáä~íÉê~ä=Ñ~Åá~ä=é~äëó=~åÇé~åÇóë~ìíçåçãá~K=^ää=íÜÉëÉ=ã~åáÑÉëí~íáçåë=Å~å=ÄÉ=~ëëçÅá~íÉÇ=ïáíÜ=ÅÉåíê~äåÉêîçìë=ëóëíÉã=áåîçäîÉãÉåí=çê=ïáíÜ=áåÑä~ãã~íçêó=ãóçé~íÜó=OPK

Inflammatory polyneuritis of the GuillainBarré type

dìáää~áåJ_~êê¨= ëóåÇêçãÉ= Ed_pF= Å~å= ÄÉ= çÄëÉêîÉÇ= ~í= íÜÉ= íáãÉ= çÑëÉêçÅçåîÉêëáçå=íç=efsK=jáäÇ=íç=ëÉîÉêÉ=ãçíçê=ÇÉÑáÅáí=áë=~ëëçÅá~íÉÇ=ïáíÜ=ÜáÖÜÑÉîÉêI= Çá~êêÜçÉ~I= ê~ëÜI= ~ÇÉåçé~íÜó= ~åÇ= ãçåçåìÅäÉçëáÅ= ëóåÇêçãÉK= qÜÉÖÉåÉê~ä=ã~åáÑÉëí~íáçåë=~êÉ=çÄëÉêîÉÇ=çåäó=~í= íÜÉ= íáãÉ=çÑ= ëÉêçÅçåîÉêëáçåKjçÇáÑáÅ~íáçåë=çÑ=íÜÉ=`pc=ÅçåíÉåí=~êÉ=ëáãáä~ê=íç=íÜçëÉ=çÄëÉêîÉÇ=áå=Åä~ëëáÅ~äd_pK=


94


Subacute multifocal neuropathy

qÜáë=áë=íÜÉ=ãçëí=çêáÖáå~ä=é~ííÉêå=çÑ=åÉìêçé~íÜó=çÄëÉêîÉÇ=áå=efs=é~íáÉåíëIÄÉÑçêÉ=íÜÉ=çåëÉí=çÑ=ÅÉääìä~ê=áããìåçÇÉéêÉëëáçåK=^=ëÉåëçêó=çê=ëÉåëçêáãçíçêÇÉÑáÅáí= çÑíÉå= éêÉÇçãáå~íÉë= áå= íÜÉ= äçïÉê= äáãÄëK= m~ê~ÉëíÜÉëá~ë= ~åÇëéçåí~åÉçìë=é~áåë=~êÉ=ÅçããçåK=qÜÉó=~êÉ=Äáä~íÉê~ä=Äìí=çÑíÉå=éêÉÇçãáå~íÉçå=çåÉ=ëáÇÉI=çê=Å~å=~ÑÑÉÅí=íÜÉ=íÉêêáíçêó=çÑ=~=åÉêîÉ=íêìåâ=çê=çÑ=~=ëéáå~ä=êççíKqÜÉó=çÑíÉå=éêçÖêÉëë=çîÉê=~= ÑÉï=ïÉÉâëI=~åÇ=~ÑÑÉÅí= íÜÉ=ìééÉê= äáãÄëK=qÜÉ`pc= ìëì~ääó= ëÜçïë= ~å= áåÅêÉ~ëÉ= áå= éêçíÉáå= ÅçåíÉåí= ~åÇ= ãáäÇ= éäÉçÅóíçëáëïáíÜ=åçêã~ä=ÖäìÅçëÉ=äÉîÉäëK=qÜÉ=çìíÅçãÉ=çÑ=íÜÉëÉ=åÉìêçé~íÜáÉë=áë=ìëì~ääóÖççÇK= m~íáÉåíë= áãéêçîÉ= ëéçåí~åÉçìëäó= çê= ~ÑíÉê= íêÉ~íãÉåí= ïáíÜÅçêíáÅçëíÉêçáÇëK=

få=åÉêîÉ=ÄáçéëáÉë=çÑ=é~íáÉåíë=ïáíÜ=ëìÄ~ÅìíÉ=ãìäíáÑçÅ~ä=åÉìêçé~íÜóI=ãáñÉÇ~ñçå~ä=~åÇ=ÇÉãóÉäáå~íáîÉ=äÉëáçåë=çÑ=åÉêîÉ=ÑáÄêÉë=~êÉ=~ëëçÅá~íÉÇ=ïáíÜ=ãáäÇáåÑä~ãã~íçêó= áåÑáäíê~íÉëK= få=ãçëí=Å~ëÉëI=éÉêáî~ëÅìä~ê=ÅìÑÑáåÖ= áë=~ëëçÅá~íÉÇïáíÜ= ÉåÇçåÉìêá~ä= áåÑä~ãã~íçêó= áåÑáäíê~íÉëI= ã~áåäó= ã~ÇÉ= ìé= çÑ= àUH= qJäóãéÜçÅóíÉë= ~åÇ= ã~ÅêçéÜ~ÖÉëK= få= ~= ÑÉï= é~íáÉåíë= ïÉ= ÑçìåÇ= åÉÅêçíáëáåÖ~êíÉêáíáë=çÑ=íÜÉ=íóéÉ=çÄëÉêîÉÇ=áå=éçäó~êíÉêáíáë=åçÇçë~I=ÄçíÜ=áå=åÉêîÉ=~åÇ=áåãìëÅäÉ=ëéÉÅáãÉåëK=

Distal symmetrical axonal polyneuropathy

aáëí~ä=ëóããÉíêáÅ~ä=åÉìêçé~íÜáÉë=çêáÖáå~ääó=êÉéêÉëÉåí=íÜÉ=ãçëí=ÅçããçåíóéÉ=çÑ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=áå=efs=é~íáÉåíëI=ÉëéÉÅá~ääó=~í=íÜÉ=ä~íÉ=ëí~ÖÉçÑ= íÜÉ= efs= áåÑÉÅíáçåK= _çíÜ= ÑÉÉí= ~êÉ= ~ÑÑÉÅíÉÇ= ëáãìäí~åÉçìëäó= Äó= é~áåÑìäëÉåë~íáçåëI= çÑíÉå= çÑ= íÜÉ= ÄìêåáåÖ= íóéÉI= ~ëëçÅá~íÉÇ= ïáíÜ= é~áåÑìä= Åçåí~ÅíÇóë~ÉëíÜÉëá~ë=ïÜáÅÜ=êÉåÇÉê=Éñ~ãáå~íáçå=ÇáÑÑáÅìäíK=m~áåÑìä=êÉíê~Åíáçå=çÑ=íÜÉÅ~äÑ= ãìëÅäÉë= çÅÅìêëK= jçíçê= áåîçäîÉãÉåí= áë= ìëì~ääó= ~ÄëÉåí= çê= ãçÇÉê~íÉKpäáÖÜí= éóê~ãáÇ~ä= íê~Åí= áåîçäîÉãÉåí= áë= ÅçããçåK= qÜÉ= ~åâäÉ= êÉÑäÉñÉë= ~êÉ~ÄëÉåí=çê=ÇÉÅêÉ~ëÉÇX=íÜÉ=çíÜÉê=íÉåÇçå=êÉÑäÉñÉë=~êÉ=çÑíÉå=ÄêáëâK=

Cytomegalovirus (CMV) neuropathy

`js=åÉìêçé~íÜó=áë=~=íêÉ~í~ÄäÉ=åÉìêçé~íÜó=íÜ~í=çÅÅìêë=~í=íÜÉ=ä~íÉ=ëí~ÖÉçÑ=áããìåçÇÉéêÉëëáçå=OQK=`js=áåÑÉÅíáçå=êÉéêÉëÉåíë=íÜÉ=ãçëí=Åçããçå=îáê~ä


95


çééçêíìåáëíáÅ= áåÑÉÅíáçå= áå= ^fapI= ~ÑÑÉÅíáåÖ= NRJPRB= çÑ= ^fap= é~íáÉåíëK= fíëãçëí= Åçããçå= ÅäáåáÅ~ä= ã~åáÑÉëí~íáçå= áë= êÉíáåáíáëI= ïáíÜ= îáëáçå= äçëëI= íÜ~í= áëçÑíÉå=Äáä~íÉê~äK=qÜÉ=Çá~Öåçëáë=çÑ=`js=åÉìêçé~íÜó=ëÜçìäÇ=åçí=ÄÉ=ãáëëÉÇëáåÅÉ=áí=áë=~ÅÅÉëëáÄäÉ=íç=ëéÉÅáÑáÅ=íêÉ~íãÉåí=Äó=Ö~åÅáÅäçîáê=çê=ÑçëÅ~êåÉíK=fåãçëí= Å~ëÉëI= é~íáÉåíë= ïáíÜ= éêçîÉå= `js= åÉìêçé~íÜó= Ü~îÉ= ^fap= ïáíÜçééçêíìåáëíáÅ= áåÑÉÅíáçåëI= éêçÑçìåÇ= áããìåçÇÉéêÉëëáçåI= ÑÉîÉêI= Å~ÅÜÉñá~I= ~àQH= qJÅÉää= Åçìåí= ÄÉäçï= RM= éÉê= ãäI= ~åÇ= `js= êÉíáåáíáëI= Äìí= áå= ëçãÉé~íáÉåíëI=`js=åÉìêçé~íÜó=áë=íÜÉ=Ñáêëí=~åÇ=çåäó=çééçêíìåáëíáÅ=áåÑÉÅíáçå=~åÇçÅÅìêë=áå=é~íáÉåíë=áå=~=êÉä~íáîÉäó=ÖççÇ=ÖÉåÉê~ä=ÅçåÇáíáçåK=

qÜÉ=ÇáÑÑÉêÉåí=é~ííÉêåë=çÑ=`js=åÉìêçé~íÜó=áåÅäìÇÉW

√ íÜÉ= éçäóê~ÇáÅìäçåÉìêçé~íÜáÅ= é~ííÉêå= áå= ïÜáÅÜ= é~íáÉåíë= ÇÉîÉäçéIïáíÜáå= ~= ÑÉï= Ç~óë= çê= ïÉÉâëI= ~= ëÉåëçêáãçíçê= ÇÉÑáÅáí= çÑ= íÜÉ= äçïÉêëéáå~ä=êççíëI=çê=~=ÅçãéäÉíÉ=Å~ìÇ~=Éèìáå~=ëóåÇêçãÉI=ïáíÜ=ëéÜáåÅíÉêÇáëíìêÄ~åÅÉëX=

√ íÜÉ=ãìäíáÑçÅ~ä=é~ííÉêå=ïáíÜ=äÉëáçåë=çÑ=ëéáå~ä=êççíëI=åÉêîÉ=íêìåâëI=~åÇëçãÉíáãÉë=Åê~åá~ä=åÉêîÉ=áåîçäîÉãÉåíX=

√ ëÉîÉêÉ= `kp= ã~åáÑÉëí~íáçåë= áåÅäìÇáåÖ= åÉÅêçíáÅ= ãóÉäáíáë= ~åÇÉåÅÉéÜ~äáíáëX=

√ `pc=~Äåçêã~äáíáÉë= íÜ~í=Å~å=ÄÉ=çÄëÉêîÉÇ= áå= íÜáë=ëÉííáåÖ=ëìÅÜ=~ë=~ÜáÖÜ= éêçíÉáå= ÅçåíÉåí= EãçêÉ= íÜ~å= NMÖLi= áå= çåÉ= çÑ= çìê= é~íáÉåíëFIéäÉçÅóíçëáë= ïáíÜ= ~= éçäóãçêéÜçåìÅäÉ~ê= äÉìÅçÅóíÉ= êÉ~Åíáçå= ~åÇ= ~ÇÉÅêÉ~ëÉ=áå=`pc=ÖäìÅçëÉK=qÜÉ=`pc=Å~åI=ÜçïÉîÉêI=êÉã~áå=åçêã~äK

qÜÉ= ãìäíáÑçÅ~äI= åÉÅêçíáÅI= ÉåÇçåÉìêá~ä= äÉëáçåë= ïáíÜ= ~= åÉìíêçéÜáäáÅ= ÅÉääêÉëéçåëÉI=ïÜáÅÜ=ã~ó=äççâ=äáâÉ=ãìäíáéäÉ=ÉåÇçåÉìêá~ä=ãáÅêç~ÄÅÉëëÉëI=ëÉÉãìåáèìÉ= íç= íÜáë= îáê~ä= ~ÖÉåíK= qÜÉëÉ= äÉëáçåë= ã~êâÉÇäó= ÇáÑÑÉê= Ñêçã= íÜçëÉçÄëÉêîÉÇ= áå= çíÜÉê= íóéÉë= çÑ= efsJ~ëëçÅá~íÉÇ= åÉìêçé~íÜáÉë= EcáÖìêÉ= RFKpéÉÅáÑáÅ= äÉëáçåë= çÑ= íÜÉ= `kpI= ïáíÜ= éêÉÇçãáå~åí= áåÑÉÅíáçå= çÑ= Öäá~ä= ~åÇÉåÇçíÜÉäá~ä= ÅÉääëI= ~êÉ= çÑíÉå= ÑçìåÇ= çå= éçëíãçêíÉã= Éñ~ãáå~íáçåK= ^äíÜçìÖÜáåÑêÉèìÉåíI=ÇáëíáåÅí=åÉìêçäçÖáÅ~ä=ëóåÇêçãÉë=Å~ìëÉÇ=Äó=`js=ÅçåíáåìÉ= íçÅ~ìëÉ=ÜáÖÜ=ãçêí~äáíó=~ãçåÖ=^fap=é~íáÉåíëK=pìêîáî~ä=ÇÉéÉåÇë=ìéçå=íÜÉ=ìëÉçÑ= ÉÑÑÉÅíáîÉ= ~åíáîáê~ä= íÜÉê~éó= ~Ö~áåëí= `js= ~åÇ= íÜÉ= É~êäó= áåíêçÇìÅíáçå= çÑe^ôqK


96


Malignant lymphomas

j~äáÖå~åí= äóãéÜçã~ëI=ïÜáÅÜ=ã~ó=ÅçãéäáÅ~íÉ= íÜÉ= áããìåçëìééêÉëëáçåçÑ= ^fap= Å~å= áåÇìÅÉ= ÑçÅ~ä= çê= ãìäíáÑçÅ~ä= åÉêîÉ= äÉëáçåë= Äó= áåî~ÇáåÖ= ëéáå~äêççíë=çê=åÉêîÉ=íêìåâëK

Toxic neuropathy in AIDS

qÜÉ= ~åíáîáê~ä= ÇêìÖ= OÛIPÛJÇáÇÉçñóáåçëáåÉ= EaafFI= ~å= ~åí~Öçåáëí= çÑ= ak^îáê~ä=ëóåíÜÉëáëI= áåÇìÅÉë=~=Çáëí~ä=ëóããÉíêáÅ~ä=ëÉåëçêó=éçäóåÉìêçé~íÜó= áåTB= çÑ= é~íáÉåíë= êÉÅÉáîáåÖ= äÉëë= íÜ~å= NOãÖLâÖLÇ~óK= qÜÉ= ëóãéíçãëÇáë~ééÉ~ê=~ÑíÉê=ïáíÜÇê~ï~ä=çÑ=íÜÉ=ÇêìÖI=ïÜáÅÜ=Å~å=ÄÉ=ëí~êíÉÇ=~Ö~áå=ä~íÉê~í=äçïÉê=ÇçëÉëK=líÜÉêë=Ü~îÉ=ÑçìåÇ=åÉìêçé~íÜó=áå=OB=çÑ=é~íáÉåíë=çåäóK


97

Figure 5. Electron micrograph of a nerve biopsy specimenfrom a patient with AIDS and multifocal neuropathy due toopportunistic infection with cytomegalovirus. The arrowspoint to CMV virions present in an endoneurial cell. (Uranylacetate and lead citrate staining. Bar: 1μm.)


References

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tel NVTQX=RNW=QRNJSRK=

OK= _äççã=_oI=dçÇ~ä=qK=pÉäÉÅíáîÉ=éêáã~êó=ÜÉ~äíÜ=Å~êÉW=ëíê~íÉÖáÉë=Ñçê=Åçåíêçä=çÑ=ÇáëÉ~ëÉ=áå

íÜÉ=ÇÉîÉäçéáåÖ=ïçêäÇK=sK=iÉéêçëóK=oÉîáÉïë=çÑ=fåÑÉÅí=aáëÉ~ëÉë NVUPX=RW=TSRJUMK=

PK= e~åëÉå=d^K=råÇÉê=ëÖÉáëÉê=~åÖ~ÉåÇÉ=ëéÉÇ~äëâÜÉÇÉåë~ê~ëÖÉê=íáÉÇÉäë=ìÑ=ÑçêíÉ=ë~ããÉã

ãÉÇ=Ñçêëí~åÇÉê=e~êíïáÖK=kçêëâÉ=j~Ö=i~ÉÖÉîáÇÉåëà=NUTQX=QW=pNK

QK= telK= mêçÖêÉëë= áå= iÉéêçëó= `çåíêçäI= fåÇçåÉëá~= NVVNJOMMUK= tÉÉâäó= béáÇÉãáçäçÖáÅ~ä

oÉÅçêÇ=OMNMX=URW=OQVJSOK

RK= qòçìêáç=Ì=p~áÇ=dI=jáä~å=gK=^ëóãéíçã~íáÅ=åÉêîÉ=ÜóéÉêíêçéÜó=áå=äÉéêçã~íçìë=äÉéêçëóW=~

ÅäáåáÅ~äI=ÉäÉÅíêçéÜóëáçäçÖáÅ~ä=~åÇ=ãçêéÜçäçÖáÅ~ä=ëíìÇóK=g=kÉìêçä NVVOX=OPVW=PSTJTQK

SK= ÇÉ=cêÉáí~ë=joI=p~áÇ=dK=iÉéêçìë=åÉìêçé~íÜóK=e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=QVVJRNQK

TK= oáÇäÉó=apI=gçéäáåÖ=teK=`ä~ëëáÑáÅ~íáçå=çÑ= äÉéêçëó=~ÅÅçêÇáåÖ=íç=áããìåáíóK=^=ÑáîÉJÖêçìé

ëóëíÉãK=fåí=g=iÉéê=líÜÉê=jóÅçÄ~Åí=aáë NVSSX=PQW=ORRJTPK

UK= içÅâïççÇ=akI=p~êåç=bI=pãáíÜ=t`K=`ä~ëëáÑáÅ~íáçå=çÑ= äÉéêçëó=é~íáÉåíëW=ëÉ~êÅÜáåÖ=Ñçê

íÜÉ=éÉêÑÉÅí=ëçäìíáçå\=iÉéê=oÉî=OMMTX=TUW=PNTJOMK


98

√ The neuropathy induced by Mycobacterium leprae mainly depends on the

immunological response of the host.

√ In lepromatous leprous neuropathy, nerves contain many bacilli, while in the

tuberculoid form bacilli are scarce.

√ Reversal reactions that occur during treatment can destroy peripheral nerves

more than the infection itself.

√ Lyme disease is a meningoradiculoneuritis due to infection with Borrelia

burgdorferi which is transmitted via a tick bite.

√ Lyme disease is a self-limiting disease which may, however, leave residual

deficits.

√ HIV infection can be associated with a variety of peripheral neuropathies.

Ü Key points


VK= jçÇäáå=oiK=qÜÉ=áåå~íÉ=áããìåÉ=êÉëéçåëÉ=áå=äÉéêçëóK=`ìêê=léáå=fããìåçä OMNMX=OOW=QUJ

RQK

NMK gçÄ=`hK=oÉÅÉåí=Üáëíçé~íÜçäçÖáÅ~ä=ëíìÇáÉë=áå=äÉéêçëóI=ïáíÜ=é~êíáÅìä~ê=êÉÑÉêÉåÅÉ=íç=É~êäó

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99



100


101

Diabetic and uraemic neuropathies

Chapter 8

Overview

In this chapter the complications of diabetes on the peripheral nervous system are

discussed, in particular, axonal sensory neuropathy, as well as the less common

manifestations of diabetes. In this review we will also consider the classical aspects

of diabetic neuropathy, the recent contributions on the subject, the current

treatments of diabetic neuropathy and the practical management of diabetic

patients with neuropathy. In addition, the effects of severe renal impairment on

the peripheral nervous system are described.

Diabetic neuropathies

Introduction

aá~ÄÉíÉë= ãÉääáíìë= áë= íÜÉ= ÅçããçåÉëí= Å~ìëÉ= çÑ= åÉìêçé~íÜó= áåáåÇìëíêá~äáëÉÇ= ÅçìåíêáÉëK= fí= Å~å= éêÉëÉåí= ïáíÜ= ~= î~êáÉíó= çÑ= ã~åáÑÉëí~íáçåëáåÅäìÇáåÖ= Çáëí~ä= ëóããÉíêáÅ~ä= éçäóåÉìêçé~íÜóI= ~ìíçåçãáÅI= ~í= íáãÉë= äáÑÉJíÜêÉ~íÉåáåÖ=~ìíçåçãáÅ=ÇóëÑìåÅíáçåI=~åÇ=ÑçÅ~ä=çê=ãìäíáÑçÅ~ä=áåîçäîÉãÉåí=çÑíÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉãK=få=~ÇÇáíáçå=íç=íÜÉëÉ=Åä~ëëáÅ=~ëéÉÅíëI=ëçãÉáåÑä~ãã~íçêó= åÉìêçé~íÜáÉëI= ëìÅÜ= ~ë= ÅÜêçåáÅ= áåÑä~ãã~íçêó= ÇÉãóÉäáå~íáåÖéçäóåÉìêçé~íÜó=E`famFI=ëÉÉã=ãçêÉ=Åçããçå=áå=Çá~ÄÉíáÅ=é~íáÉåíë=ïÜç=~êÉ


ÉñéçëÉÇI= àìëí= ~ë= åçåJÇá~ÄÉíáÅ= ëìÄàÉÅíëI= íç= ~ää= íÜÉ= çíÜÉê= Å~ìëÉë= çÑåÉìêçé~íÜóK=

Epidemiology

få= OMNPI= ~ÅÅçêÇáåÖ= íç= íÜÉ= fåíÉêå~íáçå~ä= aá~ÄÉíÉë= cÉÇÉê~íáçåI= ~åÉëíáã~íÉÇ=PUN=ãáääáçå=éÉçéäÉ=Ü~îÉ=Çá~ÄÉíÉë=NK=fíë=áåÅáÇÉåÅÉ=áë=áåÅêÉ~ëáåÖê~éáÇäó=~åÇ=Äó=OMPMI= íÜáë=åìãÄÉê= áë=Éëíáã~íÉÇ=íç=~äãçëí=ÇçìÄäÉK=qÜìëIUKPB=çÑ=~Çìäíë=~êÉ=Éëíáã~íÉÇ= íç=Ü~îÉ=Çá~ÄÉíÉë=~åÇ=PNS=ãáääáçå=éÉçéäÉÜ~îÉ=~å=áãé~áêÉÇ=ÖäìÅçëÉ=íçäÉê~åÅÉ=íÉëíK=qÜÉ=ä~êÖÉëí=áåÅêÉ~ëÉë=ïáää=í~âÉéä~ÅÉ=áå=íÜÉ=êÉÖáçåë=ïÜÉêÉ=ÇÉîÉäçéáåÖ=ÉÅçåçãáÉë=~êÉ=éêÉÇçãáå~åíK=

qÜÉ= éêÉî~äÉåÅÉ= çÑ= åÉìêçé~íÜó= áå= ëÉîÉê~ä= éçéìä~íáçåJÄ~ëÉÇ= ëìêîÉóë= áë~êçìåÇ= PMB= áå= ëíìÇáÉë= ìëáåÖ= êÉëíêáÅíáîÉ= ÇÉÑáåáíáçåë= OK= qÜÉ= éêÉî~äÉåÅÉ= çÑëÉåëçêó=ëóãéíçãë=çê=ëáÖåë=íÜ~í=áåÅäìÇÉÇ=åìãÄåÉëëI=~=äçëë=çÑ=ÑÉÉäáåÖI=é~áåçê=íáåÖäáåÖI=~=ÇÉÅêÉ~ëÉÇ=~Äáäáíó=íç=ÑÉÉä=Üçí=çê=ÅçäÇI=ï~ë=ÑçìåÇ=íç=ÄÉ=PMKOB~ãçåÖ=é~íáÉåíë=ïáíÜ=íóéÉ=N=Çá~ÄÉíÉë=PK=qÜÉ=éêÉî~äÉåÅÉ=çÑ=Çá~ÄÉíáÅ=éÉêáéÜÉê~äåÉìêçé~íÜó=áåÅêÉ~ëÉë=ïáíÜ=~ÖÉ=~åÇ=ïáíÜ=íÜÉ=Çìê~íáçå=çÑ=Çá~ÄÉíÉë=ìåíáä= áí= áëéêÉëÉåí= áå=ãçêÉ=íÜ~å=RMB=çÑ=íóéÉ=N=Çá~ÄÉíáÅë=~ÖÉÇ=çîÉê=SM=óÉ~êë=QK= fí= áëáãéçêí~åí= íç= í~âÉ= áåíç= ~ÅÅçìåí= íÜ~í= íÜÉ= çåëÉí= çÑ= íóéÉ= O= Çá~ÄÉíÉë= áëÉëíáã~íÉÇ=íç=çÅÅìê=QJT=óÉ~êë=ÄÉÑçêÉ=ÅäáåáÅ~ä=Çá~Öåçëáë=PK=cìêíÜÉêãçêÉI=~íäÉ~ëí=PMB=çÑ=éÉçéäÉ=áå=íÜÉ=rp=ïáíÜ=íóéÉ=O=Çá~ÄÉíÉë=~êÉ=ìåÇá~ÖåçëÉÇK

qç=ëìãã~êáëÉ=íÜÉ=ÑáåÇáåÖë=çÑ=íÜÉ=î~êáçìë=ÉéáÇÉãáçäçÖáÅ~ä=ëíìÇáÉëW=

√ Çá~ÄÉíáÅ=åÉìêçé~íÜó=ã~ó=ÅçãéäáÅ~íÉ=íóéÉ=N=çê=íóéÉ=O=Çá~ÄÉíÉëX=√ åÉìêçé~íÜó= ã~ó= ÄÉ= éêÉëÉåí= ~í= íÜÉ= íáãÉ= çÑ= ÇáëÅçîÉêó= çÑ= íóéÉ= O

Çá~ÄÉíÉë=ÇìÉ=íç=íÜÉ=éêÉëÉåÅÉ=çÑ=ìåÇáëÅçîÉêÉÇ=Çá~ÄÉíÉë=ÇìêáåÖ=íÜÉéêÉÅÉÇáåÖ=óÉ~êë=çê=ÇÉÅ~ÇÉëX=

√ íÜÉ= éêÉî~äÉåÅÉ= çÑ= åÉìêçé~íÜó= áåÅêÉ~ëÉë= ïáíÜ= Çìê~íáçå= ~åÇ= éççêÇá~ÄÉíáÅ=ÅçåíêçäK=

Clinicopathological aspects

páåÅÉ=Çá~ÄÉíáÅ=åÉìêçé~íÜó=ï~ë=Ñáêëí=ÇÉëÅêáÄÉÇI= íÜÉ=ÇáîÉêëáíó=çÑ=ÅäáåáÅ~äã~åáÑÉëí~íáçåë= Ü~ë= äÉÇ= íç= ëÉîÉê~ä= Åä~ëëáÑáÅ~íáçå= ëóëíÉãëK= ^äíÜçìÖÜ= åç


102


ë~íáëÑ~Åíçêó=Åä~ëëáÑáÅ~íáçå=Å~å=~ÅÅçìåí=Ñçê=íÜÉ=î~êáÉíó=çÑ=ã~åáÑÉëí~íáçåë=çÑÇá~ÄÉíáÅ=åÉìêçé~íÜóI=ãçëí=çÑ=íÜÉã=Ñ~ää=áåíçW=

√ íÜÉ= Çáëí~ä= ëóããÉíêáÅ~ä= ëÉåëçêáãçíçêI= äÉåÖíÜJÇÉéÉåÇÉåíIéçäóåÉìêçé~íÜóX=

√ ~ìíçåçãáÅ=åÉìêçé~íÜóX=√ ÑçÅ~ä=~åÇ=ãìäíáÑçÅ~ä=åÉìêçé~íÜóK=

Distal symmetrical diabetic polyneuropathy

aáëí~ä= ëóããÉíêáÅ~ä= éçäóåÉìêçé~íÜó= áë= Äó= Ñ~ê= íÜÉ= ãçëí= ÅçããçååÉìêçé~íÜáÅ=é~ííÉêå=áå=Çá~ÄÉíÉëK=fí=áë=~=éêÉÇçãáå~åíäó=ëÉåëçêó=åÉìêçé~íÜóïáíÜ= åç= ãçíçê= ÇÉÑáÅáí= áå= ãçëí= Å~ëÉëK= aáëí~ä= ãçíçê= ÇÉÑáÅáíI= áÑ= éêÉëÉåíI= áëìëì~ääó=ëäáÖÜí=~åÇ=çÅÅìêë=ã~áåäó=áå=íÜÉ=ãçëí=ëÉîÉêÉ=ÑçêãëK

Distal symmetrical predominantly sensory polyneuropathyaáëí~ä= ëóããÉíêáÅ~ä= ëÉåëçêó= éçäóåÉìêçé~íÜó= EappmF= Ü~ë= ~å= áåëáÇáçìë

çåëÉí=~åÇ=áë=íÜÉ=ãçëí=Åçããçå=é~ííÉêå=çÑ=åÉìêçé~íÜó=áå=Çá~ÄÉíáÅ=é~íáÉåíëKfí= ìëì~ääó= ÄÉÅçãÉë= ëóãéíçã~íáÅ= ëÉîÉê~ä= óÉ~êë= ~ÑíÉê= íÜÉ= çåëÉí= çÑ= íóéÉ= NÇá~ÄÉíÉë= Äìí= çÑíÉå= áë= íÜÉ= áåáíá~ä= éêÉëÉåí~íáçå= çÑ= íóéÉ= O= Çá~ÄÉíÉë= Eã~íìêÉçåëÉíFK= få~ìÖìê~ä= éçëáíáîÉ= ã~åáÑÉëí~íáçåë= çÑ= ëÉåëçêó= åÉìêçé~íÜó= áåÅäìÇÉåìãÄåÉëëI= ÄìêåáåÖ= ÑÉÉíI= ~= éáåë= ~åÇ= åÉÉÇäÉë= ëÉåë~íáçåI= ~åÇ= ä~åÅáå~íáåÖé~áåë= ïÜáÅÜ= ~êÉ= çÑíÉå= ïçêëÉ= ~í= åáÖÜíK= qÜÉ= ëÉåëçêó= åÉìêçé~íÜó= Å~å= ÄÉíçí~ääó= ~ëóãéíçã~íáÅ= ~åÇ= ÇÉíÉÅíÉÇ= çåäó= Äó= ëóëíÉã~íáÅ= åÉìêçäçÖáÅ~äÉñ~ãáå~íáçå=çÑ=íÜÉ=ÑÉÉíK=få=ëìÅÜ=Å~ëÉë=íÜÉ=åÉìêçé~íÜó=áë=áÇÉåíáÑáÉÇ=ÑçääçïáåÖé~áåäÉëë= íê~ìã~= çê= ÄìêåëI= çê= Äó= íêçéÜáÅ= ÅÜ~åÖÉë= ïáíÜ= éä~åí~ê= ìäÅÉêë= çêåÉìêçJçëíÉç~êíÜêçé~íÜó=E`Ü~êÅçíÛë=àçáåíFK

aÉéÉåÇÉåí=çå=íÜÉ=ÇÉÖêÉÉ=çÑ=Ç~ã~ÖÉ=íç=íÜÉ=éÉêáéÜÉê~ä=åÉêîÉëI=íÜÉ=Çáëí~äëóããÉíêáÅ~ä= ëÉåëçêó= äçëë=Å~å=ÄÉ= êÉëíêáÅíÉÇ= íç= íÜÉ= íçÉëI=ÉñíÉåÇ=çîÉê= íÜÉÑÉÉíI=ëéêÉ~Ç=çîÉê=íÜÉ=äÉÖë=çê=ÜáÖÜÉê=~ÄçîÉ=íÜÉ=âåÉÉ=äÉîÉäK=tÜÉå=ëÉåëçêóäçëë= ÉñíÉåÇë= ~ÄçîÉ= íÜÉ= âåÉÉI= íÜÉêÉ= áë= äáâÉäó= íç= ÄÉ= íÜÉ= ÇÉîÉäçéãÉåí= çÑëÉåëçêó=ëáÖåë=~åÇ=ëóãéíçãë=áå=íÜÉ=ÑáåÖÉêë=ïáíÜ=ëéêÉ~Ç=ìé=çîÉê=íÜÉ=Ü~åÇë~åÇ= íÜÉ= ÑçêÉ~êãë=~ë= áí=éêçÖêÉëëÉë=éêçñáã~ääóK=pìÄëÉèìÉåíäóI= íÜÉ=~åíÉêáçê~ëéÉÅí= çÑ= íÜÉ= íêìåâ= Å~å= ÄÉÅçãÉ= ~ÑÑÉÅíÉÇ= ÇìÉ= íç= íÜÉ= áåîçäîÉãÉåí= çÑ= íÜÉ

Chapter 8 Diabetic and uraemic neuropathies

103


Çáëí~ä=íÉêêáíçêó=çÑ=íÜÉ=ëÉåëçêó=åÉêîÉ=ÑáÄêÉë=çÑ=íÜÉ=áåíÉêÅçëí~ä=åÉêîÉëK=få=íÜÉãçëí= ëÉîÉêÉ= Å~ëÉë= íÜÉ= íçé= çÑ= íÜÉ= ëÅ~äé= Å~å= ÄÉ= ~ÑÑÉÅíÉÇ= ÇìÉ= íç= íÜÉáåîçäîÉãÉåí=çÑ=íÜÉ=äçåÖÉëí=ÑáÄêÉë=çÑ=íÜÉ=íêáÖÉãáå~ä=åÉêîÉ=~åÇI=ÉñÅÉéíáçå~ääóIäçëë= çÑ= ëÉåë~íáçå= Å~å= ëéêÉ~Ç= çîÉê= íÜÉ= ïÜçäÉ= ÄçÇóK= qÜáë= é~ííÉêå= çÑÇáëíêáÄìíáçå=çÑ=ëÉåëçêó=äçëë=ëìÖÖÉëíë=~=ÑáÄêÉJäÉåÖíÜ=ÇÉÖÉåÉê~íáçå=çÑ=åÉêîÉÑáÄêÉëK=pÉåëçêó=äçëë=éêÉÇçãáå~íÉëI=Äìí=íÜáë=áë=åçí=êÉëíêáÅíÉÇ=íç=íÜÉêã~ä=~åÇé~áå=éÉêÅÉéíáçåK=

qÜÉ=ã~ñáãìã=ÇáëëçÅá~íáçå=çÑ= ÑìåÅíáçå=ÄÉíïÉÉå=ëã~ää=~åÇ= ä~êÖÉ= ÑáÄêÉëçÅÅìêë= áå= íÜÉ= ëçJÅ~ääÉÇ= ÚéëÉìÇçëóêáåÖçãóÉäáÅ= íóéÉÛ= ïÜáÅÜ= ï~ë= çêáÖáå~ääóêÉéçêíÉÇ=Äó=sÉêÖÉäó= ENUVPF= áå=Çá~ÄÉíáÅ=é~íáÉåíë= RK= få= ëã~ää= ÑáÄêÉ=Çá~ÄÉíáÅappmI=íÜÉ=äÉåÖíÜJêÉä~íÉÇ=ëÉåëçêó=äçëë=ã~áåäó=~ÑÑÉÅíë=é~áå=~åÇ=íÉãéÉê~íìêÉéÉêÅÉéíáçå=~åÇ=äÉ~Çë=íç=íÜÉ=çÅÅìêêÉåÅÉ=çÑ=é~áåäÉëë=ÄìêåëI=éÉêëáëíÉåí=ÑççíìäÅÉêë=~åÇ=åÉìêçé~íÜáÅ=çëíÉç~êíÜêçé~íÜó= E`Ü~êÅçíÛë= àçáåíF= SK= qÜÉ= äçëë=çÑä~êÖÉ=ãóÉäáå~íÉÇ=ÑáÄêÉë=Å~å=äÉ~Ç=íç=~=ÇáëíìêÄ~åÅÉ=çÑ=äáÖÜí=íçìÅÜ=ëÉåëÉI=íç=~ëÉåëáÄáäáíó=íç=éêÉëëìêÉI=îáÄê~íáçå=~åÇ=àçáåí=éçëáíáçå=ëÉåëÉI=áå=~ÇÇáíáçå=íç=íÜ~íçÑ= çíÜÉê= éêçéêáçÅÉéíáîÉ= ~ÑÑÉêÉåí= ÑáÄêÉëK= qÜáë= Å~å= êÉëìäí= áå= ~å= áåÅêÉ~ëÉÇáåëí~Äáäáíó=çÑ=éçëíìêÉ=ïáíÜI=áå=íÜÉ=ãçëí=ëÉîÉêÉ=Å~ëÉëI=~=éçëáíáîÉ=oçãÄÉêÖÛëëáÖåK= ^äíÜçìÖÜ= íÜáë= ÚéëÉìÇçí~ÄÉíáÅÛ= é~ííÉêå= çÑ= Çá~ÄÉíáÅ= åÉìêçé~íÜó= ï~ëêÉÅçÖåáëÉÇ=ãçêÉ=íÜ~å=~=ÅÉåíìêó=~Öç=Äó=`Ü~êÅçí=áå=NUVMI=áí=ëÉÉãë=ê~íÜÉêìåÅçããçå=åçï~Ç~óë=TK

Painful symmetrical polyneuropathy^ÅìíÉ= é~áåÑìä= åÉìêçé~íÜó= ~åÇ= Å~ÅÜÉñá~= çÅÅìê= áå= åÉïäó= Çá~ÖåçëÉÇ

Çá~ÄÉíáÅ= é~íáÉåíë= UK= pÉåëçêó= äçëë= áë= ãáäÇ= çê= ~ÄëÉåí= ~åÇ= êÉÑäÉñ= äçëë= çêÇÉéêÉëëáçå= ~êÉ= åçí= áåî~êá~ÄäÉ= VK= qÜÉëÉ= ëÉîÉêÉ= ã~åáÑÉëí~íáçåë= ìëì~ääóëìÄëáÇÉ= ïáíÜáå= NM= ãçåíÜëI= ~åÇ= áãéêçîÉ= ïáíÜ= ÖççÇ= Çá~ÄÉíáÅ= ÅçåíêçäKm~ê~ÇçñáÅ~ääóI= éêÉÅáéáí~íáçå= çÑ= ~å= ~ÅìíÉ= é~áåÑìä= åÉìêçé~íÜó= Ü~ë= ÄÉÉåçÄëÉêîÉÇ= ~ÑíÉê= íÜÉ= Éëí~ÄäáëÜãÉåí= çÑ= íáÖÜí= ÖäóÅ~ÉãáÅ= Åçåíêçä= ÑçääçïáåÖ= ~äçåÖ=éÉêáçÇ=çÑ=ÉñíêÉãÉäó=éççê=Çá~ÄÉíáÅ=Åçåíêçä=NMK

Motor involvement in distal symmetrical diabetic neuropathyjáäÇ= Çáëí~ä= ãìëÅäÉ= ïÉ~âåÉëë= ~åÇ= ï~ëíáåÖ= ã~ó= ~ÅÅçãé~åó= ëÉîÉêÉ

appmI= Äìí= éêÉÇçãáå~åíäó= ~= ãçíçê= åÉìêçé~íÜó= áë= åçí= ~= ÑÉ~íìêÉ= çÑ= Çáëí~äåÉìêçé~íÜó=áå=Çá~ÄÉíáÅ=é~íáÉåíëK=jçíçê=ÇÉÑáÅáí=áë=~=ä~íÉ=ÉîÉåí=áå=íÜÉ=å~íìê~äÜáëíçêó=çÑ=appmK=tÜÉå=çÄëÉêîÉÇ= áå= íÜáë= ëÉííáåÖI=ãçíçê=ÇÉÑáÅáí= áë= ~äï~óë


104


Äáä~íÉê~äI=Çáëí~ä=~åÇ=êçìÖÜäó=ëóããÉíêáÅ~äK=fíë=çåëÉí=áë=~äëç=ãçêÉ=Öê~Çì~ä=~åÇáåÇçäÉåíI= Äìí= ïáíÜ= ~å= ìåêÉäÉåíáåÖ= ÅçìêëÉK= qÜÉ= çÅÅìêêÉåÅÉ= çÑ= ~éêÉÇçãáå~åíäó= ãçíçê= Çáëí~ä= áåîçäîÉãÉåí= áå= Çá~ÄÉíáÅ= é~íáÉåíë= áë= ãçêÉëìÖÖÉëíáîÉ=çÑ=~=ëìéÉêáãéçëÉÇ=ãçíçê=åÉìêçå=ÇáëÉ~ëÉ=çê=çÑ=~å=áåÑä~ãã~íçêóéçäóåÉìêçé~íÜóK=

Trophic changes in distal symmetrical sensory polyneuropathyqêçéÜáÅ=ÅÜ~åÖÉë=çÄëÉêîÉÇ= áå=Çá~ÄÉíáÅ=é~íáÉåíë= áåÅäìÇÉ= Ñççí=ìäÅÉê~íáçå

~åÇ=åÉìêçÖÉåáÅ=çëíÉç~êíÜêçé~íÜó=E`Ü~êÅçíÛë= àçáåíFK=qÜÉëÉ=ÅçãéäáÅ~íáçåëIïÜáÅÜ= êÉëìäí= Ñêçã= äçëë= çÑ= é~áå= ëÉåë~íáçåI= ~äëç= çÅÅìê= áå= ~= åìãÄÉê= çÑÅçåÇáíáçåë= íÜ~í= ~ëëçÅá~íÉ= äçëë= çÑ= é~áå= ëÉåë~íáçå= ïáíÜ= éêÉëÉêî~íáçå= çÑåçêã~ä= çê= ëìÄåçêã~ä= ëíêÉåÖíÜI= äÉ~ÇáåÖ= íç= é~áåäÉëë= íê~ìã~= ~åÇ= íÜÉÇÉîÉäçéãÉåí=çÑ=éä~åí~ê=ìäÅÉêë=~åÇ=çëíÉç~êíÜêçé~íÜóK

Foot ulcerationcççí= äÉëáçåë= ~êÉ= Åçããçå= áå= ãáÇÇäÉJ~ÖÉÇ= ~åÇ= ÉäÇÉêäó= Çá~ÄÉíáÅëK

mÉêáéÜÉê~ä= åÉìêçé~íÜó= Å~ìëÉë= ëÉåëçêó= áãé~áêãÉåí= ~åÇ= ïÉ~âåÉëë= çÑ= íÜÉáåíêáåëáÅ=Ñççí=ãìëÅäÉëI=äÉ~ÇáåÖ=íç=Ñççí=ÇÉÑçêãáíáÉë=áå=~Çî~åÅÉÇ=ÑçêãëK=qÜÉÉ~êäáÉëí= ÅÜ~åÖÉ= áë= çÑíÉå= ~= Å~ääìëI= ïÜáÅÜ= ã~ó= êÉÅìê= ÇÉëéáíÉ= êÉÖìä~ê= ÑççíÅ~êÉK=få=çíÜÉê=Å~ëÉëI=íÜÉ=Ñáêëí=ã~åáÑÉëí~íáçå=áë=~=é~áåäÉëë=éÜäóÅíÉåìä~ê=äÉëáçåçÑ=íÜÉ=éä~åí~ê=~ëéÉÅí=çÑ=íÜÉ=ÑççíK=få=ÄçíÜ=Å~ëÉëI=íÜÉó=~êÉ=é~áåäÉëë=~åÇ=~êÉ~ëëçÅá~íÉÇ=ïáíÜ=~=äçëë=çÑ=é~áå=ëÉåë~íáçå=çîÉê=íÜÉ=ÑÉÉíK=`ÜêçåáÅ=ìäÅÉêë=ãçëíÅçããçåäó= ~êáëÉ= çå= íÜÉ= ëçäÉ= çÑ= íÜÉ= ÑççíI= áå= íÜÉ= êÉÖáçå= çÑ= íÜÉ= ãÉí~í~êë~äÜÉ~ÇëK= få= ~ÇÇáíáçå= íç= íÜÉ= ëÉåëçêó= äçëëI= íÜÉ= ãáÅêçJ= ~åÇ= ã~ÅêçJáëÅÜ~ÉãáÅÉÑÑÉÅíë=çÑ=Çá~ÄÉíÉë=ÅçåíêáÄìíÉë=íç=Ñççí=ìäÅÉê~íáçåK

Neuropathic osteoarthropathym~áåäÉëë=Ñççí=ÇÉÑçêãáíóI=ëçãÉíáãÉë=çÑ=~ÅìíÉ=çåëÉíI=áë=~=ã~àçê=ëáÖå=çÑ=íÜáë

ÅçãéäáÅ~íáçåK= lå= uJê~óI= ÑÉÉí= ã~ó= ëÜçï= áåÅêÉ~ëÉÇ= ê~Çáçíê~åëé~êÉåÅóIé~áåäÉëë= Ñê~ÅíìêÉë=ÉëéÉÅá~ääó=~ÑÑÉÅíáåÖ= íÜÉ=ãÉí~í~êë~ä=ÄçåÉI=Çáëêìéíáçå=çÑ~êíáÅìä~ê= ëìêÑ~ÅÉëI= ~åÇ= ÇáëçêÖ~åáë~íáçå= çÑ= àçáåíëI= ÉëéÉÅá~ääó= çÑ= íÜÉãÉí~í~êëçéÜ~ä~åÖÉ~ä= àçáåíëK= mÉåÉíê~íáçå= çÑ= Ä~ÅíÉêá~= íÜêçìÖÜ= åÉìêçé~íÜáÅìäÅÉêë=Å~å=äÉ~Ç=íç=ÅÜêçåáÅ=çëíÉçãóÉäáíáëI=~åÇ=íç=íÜÉ=çîÉêÉëíáã~íáçå=çÑ=íÜÉÉñíÉåí= çÑ= áêêÉîÉêëáÄäÉ= ÄçåÉ= ÇÉëíêìÅíáçå= çÑ= åÉìêçé~íÜáÅ= çêáÖáåK= kÉìêçJçëíÉç~êíÜêçé~íÜó=E`Ü~êÅçíÛë=àçáåíF=Ü~ë=~å=áåÅáÇÉåÅÉ=çÑ=~ééêçñáã~íÉäó=OBéÉê=~ååìã=áå=é~íáÉåíë=ïáíÜ=Çá~ÄÉíáÅ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=NNK


105


Autonomic neuropathy in diabetic patients

få= NVQRI= oìåÇäÉë= áÇÉåíáÑáÉÇ= ãçëí= ã~åáÑÉëí~íáçåë= çÑ= Çá~ÄÉíáÅÇóë~ìíçåçãá~=NOK=`äáåáÅ~ä=Å~êÇáçî~ëÅìä~ê=ÇáëíìêÄ~åÅÉë=ìëì~ääó=ëí~êí=ïáíÜ=~êÉÇìÅíáçå= çê= äçëë= çÑ= oJo= î~êá~íáçåë= ÇìêáåÖ= ëí~åÇáåÖ= çê= ÄêÉ~íÜáåÖK= ^ÑíÉêëçãÉ=íáãÉI=ãçåíÜëI=ìëì~ääóI=íÜÉêÉ=áë=~=êÉëíáåÖ=í~ÅÜóÅ~êÇá~I=íÜÉå=íÜÉ=ÜÉ~êíê~íÉ=ã~ó= êÉíìêå= íç=åçêã~ä= î~äìÉë=Äìí=ïáíÜçìí=åçêã~ä= î~êá~íáçåëK=mçëíìê~äÜóéçíÉåëáçå= E~= Ñ~ää= áå=ëóëíçäáÅ=ÄäççÇ=éêÉëëìêÉ=çÑ=ãçêÉ= íÜ~å=PMããeÖ=çåÅÜ~åÖáåÖ=Ñêçã=~=äóáåÖ=íç=~=ëí~åÇáåÖ=éçëáíáçåI=ïáíÜçìí=áåÅêÉ~ëáåÖ=íÜÉ=ÜÉ~êíê~íÉF= ã~ó= ÄÉ= ~å= ÉñíêÉãÉäó= Çáë~ÄäáåÖ= ëóãéíçã= çÑ= ~ìíçåçãáÅ= åÉìêçé~íÜóïáíÜ= éçëíìê~ä= ëóåÅçéÉK= mçëíìê~ä= ÜóéçíÉåëáçå= Å~å= ÄÉ= ~ÖÖê~î~íÉÇ= ÄóíêáÅóÅäáÅ=~åíáÇÉéêÉëë~åíë=ïÜáÅÜ=~êÉ=çÑíÉå=ìëÉÇ=Ñçê=íÜÉ=íêÉ~íãÉåí=çÑ=ÅÜêçåáÅé~áå=áå=Çá~ÄÉíáÅ=åÉìêçé~íÜóK=^=êÉîáÉï=çÑ=ÅçåÅçãáí~åí=ãÉÇáÅ~íáçåI=áåÅäìÇáåÖãÉÇáÅ~íáçå= ëìÅÜ= ~ë= ÇáìêÉíáÅë= íÜ~í= ã~ó= ÄÉ= ÅçãéçìåÇáåÖ= éçëíìê~äÜóéçíÉåëáçå= ïáíÜ= Üóéçîçä~Éãá~I= ëÜçìäÇ= ÄÉ= ìåÇÉêí~âÉå= éêáçê= íç= ëí~êíáåÖíêÉ~íãÉåíë=ëìÅÜ=~ë=ÑäìÇêçÅçêíáëçåÉK

d~ëíêçé~êÉëáë=áë=~=Åçããçå=ã~åáÑÉëí~íáçå=çÑ=ÇáëíìêÄ~åÅÉë=çÑ=~äáãÉåí~êóíê~Åí=ÑìåÅíáçåK=fí=áë=çÑíÉå=~ëóãéíçã~íáÅI=Äìí=~í=íáãÉë=ã~ó=ÄÉ=êÉîÉ~äÉÇ=Äó=~ëÉåë~íáçå= çÑ= ÑìääåÉëëK= iÉëë= Åçããçåäó= áí= áë= ~ÅÅçãé~åáÉÇ= Äó= îçãáíáåÖKd~ëíêçé~êÉëáë= ã~ó= êÉëìäí= áå= éççê= Çá~ÄÉíáÅ= Åçåíêçä= ïáíÜ= ÜóéçÖäóÅ~Éãá~ÄÉÅ~ìëÉ= çÑ= íÜÉ= ëí~Öå~íáçå= çÑ= ÑççÇ= áå= íÜÉ= ëíçã~ÅÜK= aá~ÄÉíáÅ= Çá~êêÜçÉ~çÅÅìêë=~í=åáÖÜí=çê=~ÑíÉê=ãÉ~äë=~åÇ= áë=ï~íÉêóK= fí=ã~ó=ÄÉ=~ÅÅçãé~åáÉÇ=ÄóÑ~ÉÅ~ä=áåÅçåíáåÉåÅÉ=ÇìÉ=íç=êÉÇìÅÉÇ=éêÉëëìêÉ=áå=íÜÉ=áåíÉêå~ä=~å~ä=ëéÜáåÅíÉêK=

_ä~ÇÇÉê= ~íçåó= äÉ~Çë= íç= íÜÉ= éêÉëÉåÅÉ= çÑ= ~= ä~êÖÉ= êÉëáÇì~ä= îçäìãÉ= ~ÑíÉêãáÅíìêáíáçåI=ëçãÉíáãÉë=ÅçãéäáÅ~íÉÇ=Äó=áåÑÉÅíáçåK=oÉíêçÖê~ÇÉ=Éà~Åìä~íáçå=áëÑêÉèìÉåí= áå= é~íáÉåíë= ïáíÜ= ~å= ~íçåáÅ= Ää~ÇÇÉêK= fãéçíÉåÅÉ= ïÜáÅÜ= Å~å= ÄÉÉî~äì~íÉÇ= Äó= Åçåíáåìçìë= åçÅíìêå~ä= éÉåáäÉ= íìãÉëÅÉåÅÉ= ~åÇ= êáÖáÇáíóãçåáíçêáåÖI=áë=~=Åçããçå=ÅçãéäáÅ~íáçå=áå=ã~äÉ=Çá~ÄÉíáÅ=é~íáÉåíëK=s~ëÅìä~ê~åÇ= éëóÅÜçÖÉåáÅ= Ñ~Åíçêë= ~ë= ïÉää= ~ë= ~ÖÉáåÖ= ã~ó= ~äëç= ÅçåíêáÄìíÉ= íçáãéçíÉåÅÉK=

rå~ï~êÉåÉëë=çÑ=ÜóéçÖäóÅ~Éãá~=ã~ó=ÅçãéäáÅ~íÉ=~ìíçåçãáÅ=åÉìêçé~íÜóÇìÉ= íç= Ñ~áäìêÉ= çÑ= Å~íÉÅÜçä~ãáåÉ= êÉäÉ~ëÉI= ïÜáÅÜ= áåÇìÅÉë= Åìí~åÉçìëî~ëçÅçåëíêáÅíáçå= ~åÇ= ëïÉ~íáåÖK= qÜÉ= êÉäÉ~ëÉ= çÑ= é~åÅêÉ~íáÅ= ÖäìÅ~Öçå= áåêÉëéçåëÉ=íç=ÜóéçÖäóÅ~Éãá~I=ïÜáÅÜ=áë=ãÉÇá~íÉÇ=Äó=íÜÉ=î~ÖìëI=ã~ó=~äëç=ÄÉÇÉÑáÅáÉåí=áå=Çá~ÄÉíáÅ=~ìíçåçãáÅ=åÉìêçé~íÜó=~åÇ=äÉ~Ç=íç=~=ãçêÉ=ê~éáÇ=çåëÉíçÑ=ÜóéçÖäóÅ~Éãá~K


106


aáëçêÇÉêë= çÑ= ëïÉ~íáåÖ= ~êÉ= Åçããçå= áå= íÜÉ= ÑÉÉíK= eÉ~í= áåíçäÉê~åÅÉ= ~åÇéêçÑìëÉ=ëïÉ~íáåÖ=çîÉê=íÜÉ=ÜÉ~Ç=~åÇ=ìééÉê=íêìåâ=ã~ó=ÄÉ=ÇìÉ=íç=~åÜóÇêçëáëçîÉê= íÜÉ= äÉÖëI= äçïÉê= íêìåâ= ~åÇ= ëçãÉíáãÉë= íÜÉ= ~êãë= ïáíÜ= ÅçãéÉåë~íçêóëïÉ~íáåÖ= ÉäëÉïÜÉêÉK= ^Äåçêã~ä= éìéáää~êó= êÉëéçåëÉë= ~êÉ= Åçããçå= áåÇá~ÄÉíáÅ=é~íáÉåíëK=qÜÉ=íïç=ãçëí=ëíêáâáåÖ=ëáÖåë=~êÉ=ãáçëáë=~åÇ=êÉÇìÅÉÇ=äáÖÜíêÉÑäÉñÉëI=íÜÉ=ãÉ~ëìêÉãÉåí=çÑ=ïÜáÅÜ=Å~å=éêçîáÇÉ=ìëÉÑìä=~ìíçåçãáÅ=ÑìåÅíáçåíÉëíëK

Pathological aspects of distal symmetrical diabetic neuropathy

^Äåçêã~äáíáÉë= êÉéçêíÉÇ= áå= Çá~ÄÉíáÅ= åÉìêçé~íÜó= áåÅäìÇÉ= ÑáÄêÉëìåÇÉêÖçáåÖ= ~ñçå~ä= ÇÉÖÉåÉê~íáçåI= éêáã~êó= ÇÉãóÉäáå~íáçå= ïÜáÅÜ= êÉëìäíëÑêçã= pÅÜï~åå= ÅÉää= ÇóëÑìåÅíáçåI= ~åÇ= ëÉÅçåÇ~êó= ëÉÖãÉåí~äÇÉãóÉäáå~íáçåI= ïÜáÅÜ= áë= êÉä~íÉÇ= íç= áãé~áêãÉåí= çÑ= íÜÉ= ~ñçå~ä= Åçåíêçä= çÑãóÉäáå~íáçåK= oÉãóÉäáå~íáçåI= éêçäáÑÉê~íáçå= çÑ= pÅÜï~åå= ÅÉääëI= ~íêçéÜó= çÑÇÉåÉêî~íÉÇ= Ä~åÇë= çÑ= pÅÜï~åå= ÅÉääëI= çåáçå= ÄìäÄ= Ñçêã~íáçå= ~åÇÜóéÉêíêçéÜó= çÑ= íÜÉ= Ä~ë~ä= ä~ãáå~= ã~ó= ~äëç= ÄÉ= çÄëÉêîÉÇ= EcáÖìêÉë= NJPFKaóáåÖJÄ~Åâ=ÑáÄêÉë=~åÇ=ÑáÄêÉë=ïáíÜ=Çáëí~ä=ëéêçìíáåÖ=çÑ=íÜÉ=éêçñáã~ä=ëíìãéI


107

Figure 1. One-micron-thick plastic section of a sural nerve

biopsy of a patient with a severe sensory and autonomic

diabetic polyneuropathy. Several fibres are undergoing axonal

degeneration (arrows), while a few clusters of regenerating

axons can be seen (asterisks). (Bar 10μm.)

**



108

Figure 2. One-micron-thick plastic section of a sural nerve

biopsy of a patient with an extremely severe sensory and

autonomic diabetic polyneuropathy. Note the almost complete

disappearance of nerve fibres. (Thionine blue staining. Bar 10μm.)

Figure 3. Electron micrograph of a sural nerve biopsy of apatient with poorly controlled type 1 diabetes and longstandingsensory polyneuropathy. No nerve fibre, myelinated orunmyelinated, can be seen on this field; only fibroblasts (blackarrows) and Schwann cell processes (red arrows) can beidentified. This is the most severe pathological aspect of length-dependent diabetic neuropathy. (Uranyl acetate and leadcitrate staining. Bar 1μm.)


ëìÄëÉèìÉåí=íç=ÇÉÖÉåÉê~íáçå=çÑ=íÜÉ=Çáëí~ä=~ñçåI=Ü~îÉ=~äëç=ÄÉÉå=áÇÉåíáÑáÉÇ=áåäÉåÖíÜJÇÉéÉåÇÉåí=Çá~ÄÉíáÅ=åÉìêçé~íÜó=SK=

Focal and multifocal neuropathy

^=åìãÄÉê=çÑ=ÑçÅ~ä=~åÇI=çÅÅ~ëáçå~ääóI=ãìäíáÑçÅ~ä=åÉìêçé~íÜáÅ=ëóåÇêçãÉëçÅÅìê= áå= íÜÉ= ëÉííáåÖ= çÑ= Çá~ÄÉíÉë= ãÉääáíìëK= qÜÉó= áåÅäìÇÉ= Åê~åá~ä= åÉêîÉáåîçäîÉãÉåíI= äáãÄ= ~åÇ= íêìåÅ~ä= åÉìêçé~íÜáÉëI= ~åÇ= éêçñáã~ä= Çá~ÄÉíáÅåÉìêçé~íÜó= EmakF= çÑ= íÜÉ= äçïÉê= äáãÄëK= få= íÜáë= Öêçìé= çÑ= åÉìêçé~íÜáÉë= íÜÉÇáëçêÇÉê=íÉåÇë=íç=çÅÅìê=ÄçíÜ=áå=ãÉå=~åÇ=ïçãÉå=çîÉê=íÜÉ=~ÖÉ=çÑ=RMI=ãçëíïáíÜ=äçåÖëí~åÇáåÖ=íóéÉ=N=çê=íóéÉ=O=Çá~ÄÉíÉëK=fí=áë=êÉä~íáîÉäó=Åçããçå=íç=ëÉÉé~íáÉåíë=ïáíÜ= íÜáë=é~ííÉêå=çÑ=åÉìêçé~íÜó=ïáíÜ=åç=Üáëíçêó=çÑ=Çá~ÄÉíÉëK=qÜÉëÉîÉêáíó=çÑ=Çá~ÄÉíÉë=áë=çÑíÉå=ãáäÇK=få=íÜáë=éçéìä~íáçå=ÑçÅ~ä=åÉìêçé~íÜó=çÑíÉåÅçÉñáëíë=ïáíÜ=ëáäÉåí=çê=é~ìÅáJëóãéíçã~íáÅ=appmK=qÜÉ=äçåÖJíÉêã=éêçÖåçëáëçÑ= ÑçÅ~ä= åÉìêçé~íÜó= áë= ÖççÇ= áå= ãçëí= Å~ëÉë= Äìí= ëÉèìÉä~É= çÅÅìê= áå= ëçãÉé~íáÉåíë=ïáíÜ=éêçñáã~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜóK

Cranial diabetic neuropathylÅìäçãçíçê=åÉêîÉ=é~äëáÉë=~êÉ=íÜÉ=ãçëí=Åçããçå=áÑ=åçí=íÜÉ=çåäó=Åê~åá~ä

åÉìêçé~íÜó=çÄëÉêîÉÇ=áå=Çá~ÄÉíáÅ=é~íáÉåíëK

fí=áë=áåíÉêÉëíáåÖ=íç=åçíÉ=íÜ~í=íÜÉ=ÑêÉèìÉåÅó=çÑ=çÅìäçãçíçê=é~äëó=áå=Çá~ÄÉíáÅé~íáÉåíë=áë=ÄÉíïÉÉå=NJOBK=qÜÉ=ëáñíÜ=~åÇ=íÜáêÇ=Åê~åá~ä=åÉêîÉë=~êÉ=íÜÉ=ãçëíÅçããçåäó=~ÑÑÉÅíÉÇK=få=îáêíì~ääó=~ää=Å~ëÉë=Çá~ÄÉíáÅ=çéÜíÜ~äãçéäÉÖá~=çÅÅìêëáå=Çá~ÄÉíáÅ=é~íáÉåíë=çîÉê=RM=óÉ~êë=çÑ=~ÖÉK=qÜÉ=çåëÉí=áë=ê~éáÇI=ïáíÜáå=~=Ç~óçê= íïçK= få=ã~åó=Å~ëÉë= íÜÉ=é~íáÉåí=ÉñéÉêáÉåÅÉë=é~áå= ê~åÖáåÖ= Ñêçã=~= ÑÉïÜçìêë=íç=~=ÑÉï=Ç~óë=ÄÉÑçêÉ=åçíáÅáåÖ=Çáéäçéá~K=m~áå=áë=ìëì~ääó=~ÅÜáåÖI=ÄÉÜáåÇçê=~ÄçîÉ= íÜÉ=ÉóÉI=~åÇ=ëçãÉíáãÉë=ãçêÉ=ÇáÑÑìëÉ=Äìí=~äï~óë=çå= íÜÉ=ë~ãÉëáÇÉ= ~ë= íÜÉ= çÅìäçãçíçê= é~äëóK= m~áå= ÇçÉë= åçí= éÉêëáëí= ~ÑíÉê= íÜÉ= çåëÉí= çÑÇáéäçéá~K

lÅìäçãçíçê= ÇóëÑìåÅíáçå= áë= çÑíÉå= áåÅçãéäÉíÉ= ïÜÉå= íÜÉ= íÜáêÇ= åÉêîÉ= áëáåîçäîÉÇK=låÉ=çê=íïç=ãìëÅäÉë=çåäó=ã~ó=ÄÉ=é~ê~äóëÉÇK=míçëáë=áë=ã~êâÉÇI=íÜÉÉóÉ=áë=ÇÉîá~íÉÇ=çìíï~êÇ=ïÜÉå=íÜÉ=áåíÉêå~ä=êÉÅíìë=ãìëÅäÉ= áë=~ÑÑÉÅíÉÇ=~åÇíÜÉ= é~íáÉåí= áë= ìå~ÄäÉ= íç= ãçîÉ= íÜÉ= ÉóÉ= ãÉÇá~ääóI= ìéï~êÇ= çê= Ççïåï~êÇKmìéáää~êó=áååÉêî~íáçå=áë=ëé~êÉÇ=áå=ãçëí=Å~ëÉëK=péçåí~åÉçìë=~åÇ=ÅçãéäÉíÉ


109


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Focal limb neuropathyfëçä~íÉÇ=áåîçäîÉãÉåí=çÑ=éÉêáéÜÉê~ä=åÉêîÉë=çÑ=íÜÉ=äáãÄëI=áåÅäìÇáåÖ=ê~Çá~äI

ãÉÇá~å=~åÇ=ìäå~ê=åÉêîÉë=áå=íÜÉ=ìééÉê=äáãÄë=~åÇ=çÑ=íÜÉ=éÉêçåÉ~ä=åÉêîÉ=ÑçêíÜÉ=äçïÉê=äáãÄëI=ëÉäÇçã=çÅÅìêë=áå=Çá~ÄÉíáÅ=é~íáÉåíëK=tÜáäëí=ïÉ=Ü~îÉ=ëÉÉåçÅÅ~ëáçå~ä= Éñ~ãéäÉë= çÑ= áëçä~íÉÇ= áåîçäîÉãÉåí= çÑ= íÜÉ= ê~Çá~äI= ãÉÇá~å= ~åÇìäå~ê= åÉêîÉëI= ëìÅÜ= Å~ëÉë= ~êÉ= ÉñíêÉãÉäó= ê~êÉ= ~åÇ= ëÜçìäÇ= ~äï~óë= ÄÉáåîÉëíáÖ~íÉÇ=~ë=áå=åçåJÇá~ÄÉíáÅ=é~íáÉåíëK=mêçñáã~ä=ïÉ~âåÉëë=çÑ=íÜÉ=ìééÉêäáãÄëI=~ë= áí= ~ééÉ~êë= áå= íÜÉ= äçïÉê= äáãÄëI= áë= îÉêó=ìåÅçããçåK= få= íÜÉ= äçïÉêäáãÄëI=íÜÉ=ãçëí=Åçããçå=é~ííÉêå=çÑ= ÑçÅ~ä=åÉìêçé~íÜó= áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äóéêçñáã~ä=ëÉåëçêó=~åÇ=ãçíçê=ã~åáÑÉëí~íáçåëK

Proximal diabetic neuropathy of the lower limbsaá~ÄÉíáÅ=é~íáÉåíëI=ìëì~ääó=çîÉê=íÜÉ=~ÖÉ=çÑ=RMI=ã~ó=~äëç=éêÉëÉåí=éêçñáã~ä

åÉìêçé~íÜó=çÑ=íÜÉ=äçïÉê=äáãÄë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=~=î~êá~ÄäÉ=ÇÉÖêÉÉ=çÑ=é~áå~åÇ= ëÉåëçêó= äçëë= ~ëëçÅá~íÉÇ= ïáíÜ= ìåáJ= çê= Äáä~íÉê~ä= éêçñáã~ä= ãìëÅäÉïÉ~âåÉëë=~åÇ=~íêçéÜóK=qÜáë=ëóåÇêçãÉI=ïÜáÅÜ=ï~ë=çêáÖáå~ääó=ÇÉëÅêáÄÉÇ=Äó_êìåë= áå= NUVM= NPI= Ü~ë= ÄÉÉå= ëìÄëÉèìÉåíäó= êÉéçêíÉÇ= ìåÇÉê= íÜÉ= íÉêãë= çÑéêçñáã~ä= Çá~ÄÉíáÅ= åÉìêçé~íÜó= EmakF= NQI= NR çê= ÑÉãçê~äJëÅá~íáÅ= åÉìêçé~íÜóKqÜÉ=ãÉ~å=~ÖÉ=~í=Çá~Öåçëáë=ï~ë=SO=óÉ~êëK=jÉå=ïÉêÉ=ãçêÉ=çÑíÉå=~ÑÑÉÅíÉÇíÜ~å=ÑÉã~äÉëK

qÜÉ= çåëÉí= çÑ= íÜÉ= åÉìêçé~íÜó= áë= ~ÅìíÉ= çê= ëìÄ~ÅìíÉK= qÜÉ= é~íáÉåíÅçãéä~áåë=çÑ=åìãÄåÉëë=çê=é~áå=çÑ=íÜÉ=~åíÉêáçê=~ëéÉÅí=çÑ=íÜÉ=íÜáÖÜI=çÑíÉå=çÑíÜÉ= ÄìêåáåÖ= íóéÉ= ~åÇ= ïçêëÉ= ~í= åáÖÜíK= aáÑÑáÅìäíó= áå= ï~äâáåÖ= ~åÇ= ÅäáãÄáåÖëí~áêë=çÅÅìêëI=ÇìÉ=íç=ïÉ~âåÉëë=çÑ=íÜÉ=èì~ÇêáÅÉéë=~åÇ=áäáçéëç~ë=ãìëÅäÉëKjìëÅäÉ=ï~ëíáåÖ=áë=~äëç=~å=É~êäó=~åÇ=Åçããçå=éÜÉåçãÉåçåI=ïÜáÅÜ=áë=çÑíÉåÉ~ëáÉê=íç=é~äé~íÉ=íÜ~å=íç=ëÉÉ=áå=Ñ~ííÉê=é~íáÉåíë=EcáÖìêÉ=QFK=qÜÉ=é~íÉää~ê=êÉÑäÉñáë= ÇÉÅêÉ~ëÉÇ= çê= ãçêÉ= çÑíÉå= ~ÄçäáëÜÉÇK= qÜÉ= ëóåÇêçãÉ= éêçÖêÉëëÉë= çîÉêïÉÉâë=çê=ãçåíÜë= áå=ãçëí=Å~ëÉëI=íÜÉå=ëí~ÄáäáëÉëI=~åÇ=ëéçåí~åÉçìë=é~áåëÇÉÅêÉ~ëÉI= ëçãÉíáãÉë= ê~éáÇäóK= få= ã~åó= áåëí~åÅÉëI= ~ë= áå= íÜçëÉ= çêáÖáå~ääóêÉéçêíÉÇI= íÜÉêÉ= áë= åç= ã~êâÉÇ= çê= ~åó= ëÉåëçêó= äçëëI= ~ë= ÉãéÜ~ëáòÉÇ= Äód~êä~åÇ= NSK= få= ~ééêçñáã~íÉäó= çåÉ= íÜáêÇ= çÑ= é~íáÉåíë= íÜÉêÉ= áë= ~= ÇÉÑáåáíÉëÉåëçêó=äçëë=çîÉê=íÜÉ=~åíÉêáçê=~ëéÉÅí=çÑ=íÜÉ=íÜáÖÜI=~åÇ=áå=çíÜÉêë=~=é~áåÑìäÅçåí~Åí=Çóë~ÉëíÜÉëá~=áå=íÜÉ=ÇáëíêáÄìíáçå=çÑ=íÜÉ=Åìí~åÉçìë=Äê~åÅÜÉë=çÑ=íÜÉÑÉãçê~ä=åÉêîÉI=ïáíÜçìí=~=ÇÉÑáåáíÉ=ëÉåëçêó=äçëëK


110


få= ãçëí= Å~ëÉë= íÜÉ= é~íáÉåíÛë= ÅçåÇáíáçå= áãéêçîÉë= ~ÑíÉê= ãçåíÜëI= ÄìíëÉèìÉä~ÉI= áåÅäìÇáåÖ= Çáë~ÄäáåÖ= ïÉ~âåÉëë= ~åÇ= ~ãóçíêçéÜóI= ëÉåëçêó= äçëë~åÇ=é~íÉää~ê=~êÉÑäÉñá~I=~êÉ=ÅçããçåK=få=~=ëìêîÉó=çÑ=äçåÖJíÉêã=ÑçääçïJìé=çÑìé= íç= NQ= óÉ~êëI= êÉÅçîÉêó= ÄÉÖ~å= ~ÑíÉê= ~= ãÉÇá~å= áåíÉêî~ä= çÑ= P= ãçåíÜëEê~åÖÉ=NJNO=ãçåíÜëFNTK=m~áå=ï~ë=íÜÉ=Ñáêëí=ëóãéíçã=íç=áãéêçîÉI=êÉëçäìíáçåÄÉáåÖ= Åçãé~ê~íáîÉäó= ê~éáÇI= ÄÉÖáååáåÖ= ïáíÜáå= ~= ÑÉï= ïÉÉâë= ~åÇ= ÄÉáåÖ~äãçëí= ÅçãéäÉíÉ= Äó= NO= ãçåíÜëK= oÉëáÇì~ä= ÇáëÅçãÑçêí= áå= íÜÉ= é~íáÉåíë= çÑ`çéé~Åâ= ~åÇ= t~íâáåë= íççâ= ìé= íç= P= óÉ~êë= íç= ëìÄëáÇÉK= jçíçê= êÉÅçîÉêóï~ë= ë~íáëÑ~Åíçêó= Äìí= ëÉîÉå= é~íáÉåíë= Åçãéä~áåÉÇ= çÑ= ëçãÉ= éÉêëáëíáåÖïÉ~âåÉëëK= páÖåáÑáÅ~åí= ï~ëíáåÖ= çÑ= íÜÉ= íÜáÖÜ= ï~ë= ÉîáÇÉåí= áå= Ü~äÑ= çÑ= íÜÉÅ~ëÉë=NTK=oÉä~éëÉëI=çå=íÜÉ=çíÜÉê=Ü~åÇI=~êÉ=ÅçããçåI=ëçãÉíáãÉë=áå=ëéáíÉçÑ=ÖççÇ=Çá~ÄÉíáÅ=ÅçåíêçäK=få=çåÉ=ÑáÑíÜ=çÑ=é~íáÉåíë=íÜ~í=ïÉ=áåîÉëíáÖ~íÉÇ=ÑçêíÜáë= ëóåÇêçãÉI= êÉä~éëÉë= çÅÅìêêÉÇ= ïáíÜáå= ~= ÑÉï= ãçåíÜëI= íÜÉ= ë~ãÉéêçéçêíáçå= ~ë= áå= íÜÉ= `çéé~Åâ= ~åÇ= t~íâáåë= ëíìÇóK= qÜìëI= íÜÉ= ÅäáåáÅ~äÑÉ~íìêÉë= çÑ= mak= ïáíÜ= áíë= ÑêÉèìÉåí= ãçíçê= áåîçäîÉãÉåíI= ~ëóããÉíêáÅ~äÇÉÑáÅáíI= Öê~Çì~ä= óÉí= çÑíÉå= áåÅçãéäÉíÉ= ëéçåí~åÉçìë= êÉÅçîÉêóI= ã~êâÉÇäóÇáÑÑÉê= Ñêçã= íÜçëÉ= çÑ= appm= áå= ïÜáÅÜ= íÜÉ= äÉåÖíÜJÇÉéÉåÇÉåí= ëóããÉíêáÅ~äëÉåëçêó=ÇÉÑáÅáí=áë=~ëëçÅá~íÉÇ=ïáíÜ=ãçíçê=ëáÖåë=çåäó=áå=ÉñíêÉãÉ=Å~ëÉëI=~åÇîáêíì~ääó= åÉîÉê= áãéêçîÉë= ëéçåí~åÉçìëäóK= få= íÜÉ= ëóåÇêçãÉ= ÇÉëÅêáÄÉÇ= Äód~êä~åÇ= ~ë= ÚÇá~ÄÉíáÅ= ~ãóçíêçéÜóÛI= ãçíçê= ã~åáÑÉëí~íáçåë= ~êÉ= ãçêÉ


111

Figure 4. Massive amyotrophy of the right thigh in a patient

with type 2 diabetes and severe proximal neuropathy of the

lower limbs.


éêçãáåÉåí=Äìí=äÉëáçåë=çÑ=íÜÉ=ëÉåëçêó=Äê~åÅÜ=çÑ=íÜÉ=ÑÉãçê~ä=åÉêîÉ=~êÉ=~äëçéêÉëÉåí= áå= ëìÅÜ= é~íáÉåíë= NUK= pçãÉ= ~ìíÜçêë= Ü~îÉ= ~ÇîçÅ~íÉÇ= íÜÉ= ìëÉ= çÑëíÉêçáÇë=áå=íÜÉ=~ÅìíÉ=éÜ~ëÉI=Äìí=íÜáë=áë=Ä~ëÉÇ=çå=Å~ëÉ=êÉéçêíë=ê~íÜÉê=íÜ~åíêá~ä=ÉîáÇÉåÅÉ=NVK

Pathological aspects of PDNpíìÇó=çÑ=Äáçéëó=ëéÉÅáãÉåë=çÑ=íÜÉ=áåíÉêãÉÇá~íÉ=Åìí~åÉçìë=åÉêîÉ=çÑ=íÜÉ

íÜáÖÜI=~=ëÉåëçêó=Äê~åÅÜ=çÑ=íÜÉ=ÑÉãçê~ä=åÉêîÉ=ïÜáÅÜ=ÅçåîÉóë=ëÉåë~íáçå=ÑêçãíÜÉ= ~åíÉêáçê= ~ëéÉÅí= çÑ= íÜÉ= íÜáÖÜI= ~= íÉêêáíçêó= Åçããçåäó= áåîçäîÉÇ= áå= makIëÜçïÉÇ= äÉëáçåë= ÅÜ~ê~ÅíÉêáëíáÅ= çÑ= ëÉîÉêÉ= åÉêîÉ= áëÅÜ~Éãá~= áå= ~= ä~êÖÉéêçéçêíáçå=çÑ=é~íáÉåíë=NUI=OMK=oÉÅÉåí=çÅÅäìëáçå=çÑ=éÉêáåÉìêá~ä=ÄäççÇ=îÉëëÉäë~åÇ= éÉêáî~ëÅìä~êI= éÉêáåÉìêá~ä= ~åÇ= ëìÄéÉêáåÉìêá~ä= áåÑä~ãã~íçêó= áåÑáäíê~íáçåïáíÜ= ãçåçåìÅäÉ~ê= ÅÉääë= ïÉêÉ= ÇÉãçåëíê~íÉÇ= áå= ëçãÉI= ~äçåÖ= ïáíÜ= ~ñçå~äÇÉÖÉåÉê~íáçå=çÑ=íÜÉ=ã~àçêáíó=çÑ=åÉêîÉ=ÑáÄêÉë=EcáÖìêÉ=RFK= få=çíÜÉê=é~íáÉåíëIäÉëáçåë=çÑ=åÉêîÉ=ÑáÄêÉë=~åÇ=çÑ=ÉåÇçåÉìêá~ä=Å~éáää~êáÉë=ïÉêÉ=ëáãáä~ê=íç=íÜçëÉçÄëÉêîÉÇ=áå=íÜÉ=ëìê~ä=åÉêîÉ=áå=Çá~ÄÉíáÅ=é~íáÉåíë=ïáíÜ=ëóãéíçã~íáÅ=appmK


112

Figure 5. Section of a biopsy specimen of the superficial

peroneal nerve from a patient with multifocal neuropathy

affecting the femoral nerve and the peroneal nerves on both

sides. Note the important inflammatory infiltrate made up

of mononuclear cells surrounding the venule (asterisk).

(Hematein eosin staining. Bar 20μm.)

*


qÜÉ=éêÉëÉåÅÉ=çÑ= áåÑä~ãã~íçêó= áåÑáäíê~íÉë=ÇçÉë=åçí=éêÉÅäìÇÉ=ëéçåí~åÉçìëêÉÅçîÉêóK=

Thoracic neuropathyqêìåÅ~ä= çê= íÜçê~Åç~ÄÇçãáå~ä= åÉìêçé~íÜó= çÅÅìêë= ~äãçëí= çåäó= áå= çäÇÉê

Çá~ÄÉíáÅ=ëìÄàÉÅíë=ONK=fí=áë=ìåáä~íÉê~ä=çê=éêÉÇçãáå~åíäó=ëçK=qÜÉ=çåëÉí=áë=~Äêìéíçê= ê~éáÇI= ïáíÜ= é~áå= çê= Çóë~ÉëíÜÉëá~= ~ë= íÜÉ= ã~áå= ÑÉ~íìêÉëK= qÜÉ= é~áå= ã~óÜ~îÉ=~=ê~ÇáÅìä~ê=ÇáëíêáÄìíáçå=~åÇ=áë=ã~ÇÉ=ïçêëÉ=Äó=Åçåí~Åí=~åÇ=~í=åáÖÜíKtÉ~âåÉëë=çÑ=~ÄÇçãáå~ä=ãìëÅäÉë=Å~å=çÅÅìêK=

Multifocal diabetic neuropathyfå= ~= ëã~ää= éêçéçêíáçå= çÑ= Çá~ÄÉíáÅ= é~íáÉåíëI= ~= ãìäíáÑçÅ~ä= åÉìêçé~íÜó= áë

çÄëÉêîÉÇ=ïáíÜ=ëìÅÅÉëëáîÉ=çê= ëáãìäí~åÉçìë= áåîçäîÉãÉåíI=çîÉê=ãçåíÜëI=çÑêççíë=~åÇ=åÉêîÉë=çÑ=íÜÉ=äçïÉê=äáãÄëI=íÜÉ=íêìåâ=~åÇ=íÜÉ=ìééÉê=ÉñíêÉãáíáÉëKfå= çìê= ëÉêáÉë= çÑ= OO= é~íáÉåíëI= íÜêÉÉ= é~íáÉåíë= Ü~Ç= ~= êÉä~éëáåÖ= ÅçìêëÉK= ^é~áåÑìä=ãìäíáÑçÅ~ä=ëÉåëçêóJãçíçê=ÇÉÑáÅáí=Ü~Ç=éêçÖêÉëëÉÇ=çîÉê=OJNO=ãçåíÜëKqÜÉ=Çáëí~ä= äçïÉê= äáãÄë=ïÉêÉ= áåîçäîÉÇ= áå=~ää=é~íáÉåíëI=ìåáä~íÉê~ääó= áå=ëÉîÉåIÄáä~íÉê~ääó=áå=çíÜÉêëI=ïáíÜ=~å=~ëóåÅÜêçåçìë=çåëÉí=áå=ãçëí=Å~ëÉëK=få=~ÇÇáíáçåI~= éêçñáã~ä= ÇÉÑáÅáí= çÑ= íÜÉ= äçïÉê= äáãÄë= ï~ë= éêÉëÉåí= çå= çåÉ= ëáÇÉ= áå= ëÉîÉåé~íáÉåíëI= çå= ÄçíÜ= ëáÇÉë= áå= ëáñK= qÜçê~ÅáÅ= ê~ÇáÅìäçåÉìêçé~íÜó= ï~ë= éêÉëÉåíÄáä~íÉê~ääó=áå=íïç=é~íáÉåíëI=ìåáä~íÉê~ääó=áå=çåÉK=qÜÉ=ìäå~ê=åÉêîÉ=ï~ë=áåîçäîÉÇáå= çåÉ= é~íáÉåíI= íÜÉ= ê~Çá~ä= åÉêîÉ= áå= íïçK= qÜÉ= ÅÉêÉÄêçëéáå~ä= ÑäìáÇ= E`pcFéêçíÉáå= ê~åÖÉÇ= Ñêçã= MKQMJPKRRÖLiI= ïáíÜ= ~= ãÉ~å= çÑ= MKUTÖLiKbäÉÅíêçéÜóëáçäçÖáÅ~ä= íÉëíáåÖ= ëÜçïÉÇ= ëÉîÉêÉI= ãìäíáÑçÅ~äI= ~ñçå~ä= åÉêîÉäÉëáçåë=áå=~ää=Å~ëÉë=OOK=kÉÅêçíáëáåÖ=î~ëÅìäáíáë=çÑ=éÉêáåÉìêá~ä=~åÇ=ÉåÇçåÉìêá~äÄäççÇ=îÉëëÉäë=ï~ë=ÑçìåÇ=áå=ëáñ=é~íáÉåíëI=ÉåÇçåÉìêá~ä=ëÉÉé~ÖÉ=çÑ=êÉÇ=ÅÉääëáå=NN=ëéÉÅáãÉåëI=ÉåÇçåÉìêá~ä=Ü~ÉãçêêÜ~ÖÉ=áå=ÑáîÉI=~åÇ=éçëíJÜ~ÉãçêêÜ~ÖáÅÑÉêêáÅ= áêçå= ÇÉéçëáíë= áå= NM= é~íáÉåíëK= _ÉëáÇÉë= íÜÉ= ÜáÖÜ= ÑêÉèìÉåÅó= ÄçíÜ= çÑÉåÇçåÉìêá~ä=ÄäÉÉÇáåÖ=~åÇ=çÑ=áåÑä~ãã~íçêó=áåÑáäíê~íÉëI=çÅÅäìëáçå=çÑ=ëã~ää=~åÇãáÇÇäÉJëáòÉÇ= ÉéáåÉìêá~ä= ~åÇ= éÉêáåÉìêá~ä= ~êíÉêáÉë= ÇáÑÑÉêÉåíá~íÉë= ãìäíáÑçÅ~äÇá~ÄÉíáÅ= åÉìêçé~íÜó= EjakF= Ñêçã= appmK= qÜÉ= çìíÅçãÉ= áë= ÄÉííÉê= áå= jakíÜ~å=áå=appmK=fãéêçîÉãÉåí=çÅÅìêë=áå=~ää=é~íáÉåíë=~ÑíÉê=~=ÑÉï=ãçåíÜëI=ÄìíäçåÖJíÉêã=ëÉèìÉä~É=~êÉ=ÅçããçåK

Non-diabetic neuropathies more common in diabetic patientsfå=~ÇÇáíáçå=íç=ëéÉÅáÑáÅ=åÉìêçé~íÜáÉëI=Çá~ÄÉíáÅ=é~íáÉåíë=ëÉÉã=ãçêÉ=éêçåÉ

íç=ÇÉîÉäçé=ëçãÉ=íóéÉë=çÑ=åÉìêçé~íÜó=íÜ~å=åçåJÇá~ÄÉíáÅ=é~íáÉåíë=E~=ÇçìÄäÉÜáíFK


113


Increased liability to pressure palsymêÉëëìêÉ=é~äëó= áë=ãçêÉ=Åçããçå= áå=Çá~ÄÉíáÅ= áåÇáîáÇì~äëK=`~êé~ä= íìååÉä

ëóåÇêçãÉ=çÅÅìêë=áå=NOB=çÑ=Çá~ÄÉíáÅ=é~íáÉåíëI=~åÇ=íÜÉ=áåÅáÇÉåÅÉ=çÑ=ìäå~êåÉìêçé~íÜó= ÇìÉ= íç= ãáÅêçäÉëáçåë= ~í= íÜÉ= ÉäÄçï= äÉîÉä= áë= ÜáÖÜ= áå= Çá~ÄÉíáÅé~íáÉåíë=íççK=

Acquired inflammatory demyelinative polyneuropathyfåÑä~ãã~íçêóI= éêÉÇçãáå~åíäó= ÇÉãóÉäáå~íáåÖ= åÉìêçé~íÜó= ~äëç= ãìëí= ÄÉ

ÇáÑÑÉêÉåíá~íÉÇ=Ñêçã=Çá~ÄÉíáÅ=éçäóåÉìêçé~íÜóI=~åÇ=ã~ó=çÅÅìê=ïáíÜ=~=ÖêÉ~íÉêÑêÉèìÉåÅó=áå=íÜáë=éçéìä~íáçåK=qÜáë=Çá~Öåçëáë=ãìëí=ÄÉ=ëìëéÉÅíÉÇ=ïÜÉå=~å~ÅìíÉ= çê= ëìÄ~ÅìíÉI= çÑíÉå= éêÉÇçãáå~åíäó= ãçíçêI= ÇÉãóÉäáå~íáîÉéçäóåÉìêçé~íÜó=çÅÅìêë=áå=~=Çá~ÄÉíáÅ=é~íáÉåíK=bäÉÅíêçéÜóëáçäçÖáÅ~ä=ÑÉ~íìêÉë~êÉ=íÜçëÉ=çÑ=~=ÇÉãóÉäáå~íáîÉ=åÉìêçé~íÜó=OPI=OQK=qÜÉ=ÅçìêëÉ=~åÇ=êÉëéçåëÉíç=íêÉ~íãÉåí=áë=çÑíÉå=íÜÉ=ë~ãÉ=~ë=áå=åçåJÇá~ÄÉíáÅ=é~íáÉåíëK

MucormycosisqÜáë=ê~êÉ=ÅçåÇáíáçå=áë=~å=~ÅìíÉ=ÇáëÉ~ëÉ=íÜ~í=ëìÅÅÉëëáîÉäó=~ÑÑÉÅíë=íÜÉ=~áê

Å~îáíáÉë=çÑ=íÜÉ=Ñ~ÅÉI=íÜÉ=çêÄáí=~åÇ=íÜÉ=Äê~áåI=~ë=~=êÉëìäí=çÑ=éêçäáÑÉê~íáçå=çÑ=~ÑìåÖìë=çÑ= íÜÉ=Åä~ëë=mÜóÅçãóÅÉí~K= få=PSB=çÑ=Å~ëÉë= áí= áë=~ëëçÅá~íÉÇ=ïáíÜÇá~ÄÉíÉëI=ÉëéÉÅá~ääó=Çá~ÄÉíáÅ=~ÅáÇçëáëK=qÜáë=ã~ó=éêÉëÉåí=ïáíÜ=~å=ÉéáëçÇÉ=çÑêÜáåçäçÖáÅ~ä=áåîçäîÉãÉåí=ïáíÜ=Ééáëí~ñáëI=îáçäÉåí=ÜÉ~Ç~ÅÜÉëI=~åÇ=çêÄáíçå~ë~äé~áå=ïáíÜ=ëïÉääáåÖ=çÑ=íÜÉ=äáÇë=~åÇ=çéÜíÜ~äãçéäÉÖá~K=qÜÉ=ÇáëÉ~ëÉ=ëéêÉ~Çë=íçíÜÉ=ãÉåáåÖÉë=~åÇ=íç=íÜÉ=Äê~áå=íÜêçìÖÜ=íÜÉ=~êíÉêáÉëI=áåÇìÅáåÖ=íÜêçãÄçëáë=çÑíÜÉ= çéÜíÜ~äãáÅ= íÜÉå= çÑ= íÜÉ= áåíÉêå~ä= Å~êçíáÇ= ~êíÉêóI= ïáíÜ= ëìÄëÉèìÉåíÜÉãáéäÉÖá~K= qÜÉ= éêçÖåçëáë= áë= ÉñíêÉãÉäó= éççêK= qÜÉ= Çá~Öåçëáë= ëÜçìäÇ= ÄÉã~ÇÉ=~ë=ëççå=~ë=éçëëáÄäÉ=ïáíÜ=~=Äáçéëó=çÑ=íÜÉ=å~ë~ä=äÉëáçåë=ïÜáÅÜ=~ääçïëáÇÉåíáÑáÅ~íáçå=çÑ=íÜÉ=Å~ìë~íáîÉ=éÜóÅçãóÅÉíÉ=~åÇ=íÜÉ=ëí~êí=çÑ=íêÉ~íãÉåíK

Renal failure in diabetic patients

aá~ÄÉíÉë= áë= íÜÉ= äÉ~ÇáåÖ=Å~ìëÉ=çÑ=ÉåÇJëí~ÖÉ= êÉå~ä=ÇáëÉ~ëÉ= áå= íÜÉ=rp~åÇ= bìêçéÉK= oÉå~ä= áåëìÑÑáÅáÉåÅó= áë= ~= Åçããçå= ÅçãéäáÅ~íáçå= çÑ= Çá~ÄÉíáÅåÉéÜêçé~íÜóI= ïÜáÅÜ= Å~å= åÉÅÉëëáí~íÉ= éÉêáçÇáÅ= Çá~äóëáë= ~åÇLçê= ~= êÉå~äíê~åëéä~åíK=få=íÜáë=éçéìä~íáçå=ïáíÜ=ÄçíÜ=Çá~ÄÉíÉë=~åÇ=ã~àçê=êÉå~ä=Ñ~áäìêÉIåÉìêçé~íÜó=áë=Åçããçå=~åÇ=çÑíÉå=ëÉîÉêÉK=qÜÉ=ÇÉäÉíÉêáçìë=ÉÑÑÉÅí=çÑ=êÉå~äÑ~áäìêÉ= çå= åÉêîÉ= ÑìåÅíáçå= áë= êÉëéçåëáÄäÉ= Ñçê= ãìÅÜ= çÑ= íÜÉ= ëÉîÉêÉ= ãçíçêÇÉÑáÅáíI= ëçãÉíáãÉë= çÑ= ê~éáÇ= çåëÉíI= íÜ~í= é~íáÉåíë= ã~ó= éêÉëÉåí= áå= íÜáë


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ÅçåíÉñíK= oÉÅçîÉêó= Ñêçã= ãçíçê= ÇÉÑáÅáí= áë= ìëì~ääó= ÖççÇ= ~ÑíÉê= âáÇåÉóíê~åëéä~åí~íáçåK

Differential diagnosis

få= ÑçÅ~ä= åÉìêçé~íÜó= çÅÅìêêáåÖ= áå= Çá~ÄÉíáÅ= é~íáÉåíëI= ~= åÉìêçé~íÜó= çÑ~åçíÜÉê=çêáÖáå=ãìëí=~äï~óë=ÄÉ=ÉñÅäìÇÉÇK=få=é~íáÉåíë=ïáíÜ=çéÜíÜ~äãçéäÉÖá~IíÜÉ=éêÉëÉêî~íáçå=çÑ=éìéáää~êó=ãçíêáÅáíó=áå=~=åÉ~êäó=ÅçãéäÉíÉ=íÜáêÇ=Åê~åá~ä=åÉêîÉé~äëó=ëíêçåÖäó=ëìÖÖÉëíë=~=Çá~ÄÉíáÅ=çêáÖáåX=ÜçïÉîÉêI=ÉîÉå=áå=ëìÅÜ=Å~ëÉëI=áí=áëïáëÉ= íç= éÉêÑçêã= åçåJáåî~ëáîÉ= áåîÉëíáÖ~íáçåë= çÑ= íÜÉ= ~êÉ~K= j~ÖåÉíáÅêÉëçå~åÅÉ=~åÖáçÖê~éÜó=ïáää=êìäÉ=çìí=íÜÉ=éçëëáÄáäáíó=çÑ=~=ÅçãéêÉëëáîÉ=äÉëáçåçÑ=íÜÉ=íÜáêÇ=Åê~åá~ä=åÉêîÉ=Äó=~=ä~êÖÉ=~åÉìêóëã=çÑ=íÜÉ=Å~êçíáÇ=~êíÉêó=ïáíÜáå=íÜÉÅ~îÉêåçìë=ëáåìëI=~å=~åÉìêóëã=áå=íÜÉ=éçëíÉêáçê=ÅçããìåáÅ~íáåÖ=~êíÉêóI=çê=~ÑìëáÑçêã=~åÉìêóëã=~í=íÜÉ=íçé=çÑ=íÜÉ=Ä~ëáä~ê=~êíÉêóK=fã~ÖáåÖ=ïáää=~äëç=ÜÉäé=íçÉñÅäìÇÉ=íìãçìêë=çÅÅìêêáåÖ=~í=íÜÉ=Ä~ëÉ=çÑ=íÜÉ=Äê~áå=çê=áå=íÜÉ=Ä~ë~ä=ëâìääK=fåé~íáÉåíë= ïáíÜ= éêçÖêÉëëáîÉ= áåîçäîÉãÉåí= çÑ= ëÉîÉê~ä= Åê~åá~ä= åÉêîÉë= ïáíÜçìíáã~ÖáåÖ=~Äåçêã~äáíáÉëI=Éñ~ãáå~íáçå=çÑ=íÜÉ=`pc=ã~ó=ÇÉíÉÅí=ã~äáÖå~åí=ÅÉääëÅÜ~ê~ÅíÉêáëíáÅ= çÑ= ~= Å~êÅáåçã~íçìë= ãÉåáåÖáíáëK= få= Çá~ÄÉíáÅ= é~íáÉåíë= ïÜçÇÉîÉäçé=~= ÑçÅ~ä= çê=ãìäíáÑçÅ~ä= åÉìêçé~íÜó=çÑ= íÜÉ= äáãÄëI=çíÜÉê=Å~ìëÉë= íÜ~åÇá~ÄÉíÉë= ëÜçìäÇ= ~äëç= ÄÉ= ÅçåëáÇÉêÉÇK= qÜÉ= Ñáêëí= ëíÉé= áå= íÜáë= ÅçåíÉñí= áë= íçÇÉíÉêãáåÉ=áÑ=íÜÉ=äÉëáçåë=~êÉ=äçÅ~íÉÇ=áå=íÜÉ=ëéáå~ä=êççíë=çê=áå=íÜÉ=éÉêáéÜÉê~äåÉêîÉëI= ~= ÇáëíáåÅíáçå= ïÜáÅÜ= ã~ó= ÄÉ= ÇáÑÑáÅìäí= ÅäáåáÅ~ääó= ~åÇÉäÉÅíêçéÜóëáçäçÖáÅ~ääóK=få=~ÇÇáíáçåI=íÜÉ=äÉëáçåë=ã~ó=ÄÉ=ãáñÉÇK=^=åÉêîÉ=~åÇ=~ãìëÅäÉ= Äáçéëó= ëÜçìäÇ= ÄÉ= éÉêÑçêãÉÇI= ÉëéÉÅá~ääó= áå= ÑçÅ~ä= çê= ãìäíáÑçÅ~äåÉìêçé~íÜóK=tÜÉå=~=Çá~ÄÉíáÅ=é~íáÉåí=ÇÉîÉäçéë=éêçñáã~ä=ïÉ~âåÉëë=ïáíÜçìíãìÅÜ=é~áåI=~=ëìéÉêáãéçëÉÇ=Å~ìëÉ=çÑ=ãçíçê=åÉìêçé~íÜó=çê=çÑ=ãçíçê=åÉìêçåÇáëÉ~ëÉ=ãìëí=ÄÉ=ÅçåëáÇÉêÉÇI=~åÇ=~ééêçéêá~íÉ=áåîÉëíáÖ~íáçåë=ìåÇÉêí~âÉåK

Nerve conduction studies

^= åìãÄÉê= çÑ= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= Ü~îÉ= ÄÉÉå= éÉêÑçêãÉÇ= áåÇá~ÄÉíáÅ= é~íáÉåíëK= `Ü~åÖÉë= áå= ÅçåÇìÅíáçå= îÉäçÅáíó= Å~å= ÄÉ= ÇÉíÉÅíÉÇ= áå~ëóãéíçã~íáÅ= é~íáÉåíë= Äìí= íÜÉáê= éêÉëÉåÅÉ= áë= åçí= éêÉÇáÅíáîÉ= çÑ= íÜÉçÅÅìêêÉåÅÉ= çÑ= ëóãéíçã~íáÅ= åÉìêçé~íÜóK= póëíÉã~íáÅ= ÉäÉÅíêçéÜóëáçäçÖáÅ~äÉî~äì~íáçå=çÑ=Çá~ÄÉíáÅ=é~íáÉåíë=ÇçÉë=åçí=ëÉÉã=~Çîáë~ÄäÉK= få=ëóãéíçã~íáÅÇá~ÄÉíáÅ=åÉìêçé~íÜó= íÜÉêÉ= áë=~=ãáñíìêÉ=çÑ=~=ëäçïáåÖ=çÑ=åÉêîÉ=ÅçåÇìÅíáçå


115


îÉäçÅáíó=ÇìÉ=íç=ÇÉãóÉäáå~íáçå=~åÇ=~=äçëë=çÑ=ä~êÖÉ=ãóÉäáå~íÉÇ=ÑáÄêÉëI=~åÇ=~ÇÉÅêÉ~ëÉ= áå= åÉêîÉ= ~Åíáçå= éçíÉåíá~äë= ~äëç= ÇìÉ= íç= äçëë= çÑ= ~ñçåëI= ~åÇáåÅêÉ~ëÉÇ= íÉãéçê~ä= ÇáëéÉêëáçåK= ^= éìêÉäó= ÇÉãóÉäáå~íáîÉ= åÉìêçé~íÜó= áëÉñÅÉéíáçå~ä=áå=Çá~ÄÉíáÅ=é~íáÉåíë=~åÇ=áë=ãçêÉ=ëìÖÖÉëíáîÉI=Ñçê=áåëí~åÅÉI=çÑ=íÜÉçÅÅìêêÉåÅÉ= çÑ= ~= ÇÉãóÉäáå~íáåÖ= åÉìêçé~íÜó= çÑ= áåÑä~ãã~íçêó= çêÇóëÖäçÄìäáå~ÉãáÅ= çêáÖáåK= póëíÉã~íáÅ= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= íÉëíáåÖ= çÑÇá~ÄÉíáÅ=é~íáÉåíë=ïáíÜ=íóéáÅ~ä=éÉêáéÜÉê~ä=åÉìêçé~íÜó=áë=åçí=åÉÅÉëë~êóK=

Pathophysiology of diabetic neuropathy

_çíÜ=ãÉí~ÄçäáÅ=~åÇ=áëÅÜ~ÉãáÅ=ãÉÅÜ~åáëãë=ã~ó=éä~ó=~=êçäÉ=áå=Çá~ÄÉíáÅåÉìêçé~íÜáÉëW=íÜÉ=ãÉí~ÄçäáÅ=Ñ~Åíçê=ëÉÉãë=íç=éêÉî~áä=áå=appk=~åÇ=áå=ãáäÇÑçêãë=çÑ=makI=ïÜáäÉ=~=ëìéÉêáãéçëÉÇ=áåÑä~ãã~íçêó=éêçÅÉëë=~åÇ=áëÅÜ~ÉãáÅåÉêîÉ=äÉëáçåë=ëÉÉã=êÉëéçåëáÄäÉ=Ñçê=ëÉîÉêÉ=Ñçêãë=çÑ=makK=qÜÉ=íÜáÅâÉåáåÖ~åÇ=Üó~äáåáë~íáçå=çÑ= íÜÉ=ï~ääë=çÑ=ëã~ää=ÄäççÇ=îÉëëÉäë=ëìÖÖÉëíë=~= êçäÉ= ÑçêåÉêîÉ=áëÅÜ~Éãá~=áå=Çá~ÄÉíáÅ=åÉìêçé~íÜóK=

qÜÉ=êçäÉ=çÑ=íÜÉ=~ÅÅìãìä~íáçå=çÑ=éçäóçäë=çÄëÉêîÉÇ=áå=~åáã~äë=~äëç=çÅÅìêëáå=Üìã~åë=Äìí=ïÜÉíÜÉê=íÜáë=~ÅÅìãìä~íáçå=áå=åÉêîÉë=äÉ~Çë=íç=åÉìêçé~íÜó=áë=åçíÉëí~ÄäáëÜÉÇK= qÜÉ= ìëÉÑìäåÉëë= çÑ= íêÉ~íãÉåí= ïáíÜ= ~äÇçëÉJêÉÇìÅí~ëÉ= áåÜáÄáíçêëÜ~ë=åçí=ÄÉÉå=ÅçåÑáêãÉÇK=qÜÉ=êÉÇìÅíáçå=çÑ=éêçíÉáå=ëóåíÜÉëáë=~åÇ= íê~åëéçêíÜ~ë=ÄÉÉå= ÑçìåÇ= áå=~åáã~ä=ãçÇÉäë=~åÇ=ã~ó=~ÅÅçìåí= Ñçê= íÜÉ=çÅÅìêêÉåÅÉ=çÑÇóáåÖJÄ~Åâ=ÑáÄêÉë=áå=íÜÉ=åÉêîÉëK=fãé~áêãÉåí=çÑ=~ñçå~ä=ÉäçåÖ~íáçå=~åÇ=Å~äáÄêÉÖêçïíÜ=çÑ= êÉÖÉåÉê~íáåÖ= ÑáÄêÉë=Ü~ë=ÄÉÉå= ÑçìåÇ= áå=Çá~ÄÉíáÅ= ê~íëI= ~äçåÖ=ïáíÜÇçïåêÉÖìä~íáçå=çÑ=ãok^=~åÇ=åÉìêçÑáä~ãÉåí=éêçíÉáå=ÉñéêÉëëáçåK=̂ =åìãÄÉê=çÑçíÜÉê=Ñ~Åíçêë=~åÇ=ãÉÅÜ~åáëãë=ã~ó=éä~ó=~=êçäÉ=ORI=OSK=

Treatment of diabetic neuropathy EÜ ëÉÉ= Å~ëÉ= ëíìÇáÉë= çåééNNUJOOF

Preventative treatmentmêÉîÉåíáçå=çÑ=Çá~ÄÉíáÅ=åÉìêçé~íÜó=~åÇ=çÑ=áíë=ÅçãéäáÅ~íáçåë=êÉã~áåë=íÜÉ

ÄÉëí=íêÉ~íãÉåíK=léíáãìã=Çá~ÄÉíáÅ=Åçåíêçä=ÇáãáåáëÜÉë=íÜÉ=êáëâ=çÑ=ÇÉîÉäçéáåÖ~=Çáë~ÄäáåÖ=éÉêáéÜÉê~ä=åÉìêçé~íÜóI=Äìí=áí=áë=åçí=çÑíÉå=~ÅÜáÉî~ÄäÉI=ÄÉÅ~ìëÉ=áíÅ~êêáÉë=~=ÜáÖÜÉê=êáëâ=çÑ=ÜóéçÖäóÅ~Éãá~K=mêÉîÉåíáçå=çÑ=íÜÉ=ÅçåëÉèìÉåÅÉë=çÑÜóéÉêÖäóÅ~Éãá~= çå= åÉêîÉë= Å~å= áåÅäìÇÉ= íÜÉ= ìëÉ= çÑ= ~äÇçëÉJêÉÇìÅí~ëÉ


116



117

áåÜáÄáíçêëI=ïÜÉêÉ=íÜÉ=ÉÑÑáÅ~Åó=çÑ=ëìÅÜ=íêÉ~íãÉåí=Ü~ë=ÄÉÉå=ÇÉãçåëíê~íÉÇKjçëí= ~äÇçëÉJêÉÇìÅí~ëÉ= áåÜáÄáíçêë= Ü~îÉ= ~Åíì~ääó= Ñ~áäÉÇ= íç= ëáÖåáÑáÅ~åíäóáãéêçîÉ=é~íáÉåíë=ïáíÜ=Çá~ÄÉíáÅ=åÉìêçé~íÜóK

qÜÉ= éêÉîÉåíáçå= çÑ= ÅçãéäáÅ~íáçåë= çÑ= Çá~ÄÉíáÅ= åÉìêçé~íÜó= áåÅäìÇÉëéêÉîÉåíáçå=çÑ=íêçéÜáÅ=ÅÜ~åÖÉë=áå=íÜÉ=ÑÉÉíK=aá~ÄÉíáÅë=åÉÉÇ=~ÇîáÅÉ=~Äçìí=ÑççíÅ~êÉ= ~åÇ= ÑççíïÉ~ê= ~åÇ= ~Äçìí= íÜÉ= éêçíÉÅíáçå= çÑ= ÜóéçëÉåëáíáîÉ= ~êÉ~ë= ~åÇéêÉëëìêÉ= éçáåíëI= íç= éêÉîÉåí= íÜÉ= çÅÅìêêÉåÅÉ= çÑ= é~áåäÉëë= ìäÅÉêë= ~åÇÇÉÅêÉ~ëÉ= íÜÉ= êáëâ= çÑ= ÄçåÉ= áåÑÉÅíáçåK= mêÉîÉåíáçå= ~åÇ= íêÉ~íãÉåí= çÑ= íÜÉÇá~ÄÉíáÅ= Ñççí= ~êÉ= ÄÉëí= ÖáîÉå= áå= ëéÉÅá~äáëÉÇ= Ñççí= ÅäáåáÅëK= aá~ÄÉíáÅë= ïáíÜëÉåëçêó=äçëë=~äëç=åÉÉÇ=~ÇîáÅÉ=~Äçìí=é~áåäÉëë=Äìêåë=~åÇ=áåàìêáÉëK

Treatment of symptomsfå= ÑçÅ~ä= åÉìêçé~íÜóI= áåÅäìÇáåÖ= Åê~åá~ä= åÉêîÉ= é~äëóI= éêçñáã~ä= Çá~ÄÉíáÅ

åÉìêçé~íÜó= çê= íêìåÅ~ä= åÉìêçé~íÜóI= íÜÉ= ÅçìêëÉ= áë= ëÉäÑJäáãáíáåÖ= ïáíÜ= ~= ÖççÇëéçåí~åÉçìë= êÉÅçîÉêóI= áå=ãçëí=Å~ëÉë=ïáíÜáå=~= ÑÉï=ãçåíÜëK=vÉí=Åçåíêçä=çÑé~áå= ã~ó= ÄÉ= ÇáÑÑáÅìäí= áå= íÜÉëÉ= åÉìêçé~íÜáÉë= ~ë= ïÉää= ~ë= áå= ëóããÉíêáÅ~äåÉìêçé~íÜáÉëK= `~êÄ~ã~òÉéáåÉI= ÇáéÜÉåóäÜóÇ~åíçáåI= Ö~Ä~éÉåíáåI= éêÉÖ~Ä~äáåIÇìäçñÉíáåÉ=çê=Åäçå~òÉé~ã=~êÉ=ëçãÉíáãÉë=ìëÉÑìä=çê=é~ê~ÅÉí~ãçä=~ëëçÅá~íÉÇïáíÜ=ÅçÇÉáåÉK=qêáÅóÅäáÅ=~åíáÇÉéêÉëë~åíë=Eáãáéê~ãáåÉI=~ãáíêóéíóäáåÉI=ÉíÅKF=~êÉçÑíÉå= ÉÑÑÉÅíáîÉK= qÜÉ= ìëì~ä= ÇçëÉ= î~êáÉë= Ñêçã= PMJNRMãÖ= éÉê= Ç~óK= qêáÅóÅäáÅ~åíáÇÉéêÉëë~åíë= ã~ó= ~ÖÖê~î~íÉ= éçëíìê~ä= ÜóéçíÉåëáçåK= låäó= ëóãéíçã~íáÅéçëíìê~ä=ÜóéçíÉåëáçå=êÉèìáêÉë=íêÉ~íãÉåíK=qÜÉ=ãçëí=ÉÑÑÉÅíáîÉ=íêÉ~íãÉåí=áë=VJÑäìçêçÜóÇêçÅçêíáëçåÉ=ïÜáÅÜ=Å~êêáÉë=~=êáëâ=çÑ=ÜóéÉêíÉåëáçå=áå=~=äóáåÖ=éçëáíáçåK=

Distal symmetrical sensorimotor length-dependent polyneuropathy qÜÉêÉ= áë= åç= ëéÉÅáÑáÅ= íêÉ~íãÉåí= Ñçê= Çáëí~ä= ëóããÉíêáÅ~ä= éçäóåÉìêçé~íÜóK

låäó= íÜÉ= ÅçãéäáÅ~íáçåë= çÑ= íÜáë= ÅçåÇáíáçå= Å~å= ÄÉ= íêÉ~íÉÇ= çê= éêÉîÉåíÉÇIáåÅäìÇáåÖ= íêçéÜáÅ= ÅÜ~åÖÉëI= åÉìêçé~íÜáÅ= é~áå= EëÉÉ= `Ü~éíÉê= NN= ÔkÉìêçé~íÜáÅ= é~áå= Ô= Ñçê= ãçêÉ= ÇÉí~áäF= ~åÇ= íÜÉ= ëóãéíçãë= çÑ= ~ìíçåçãáÅåÉìêçé~íÜóK

Autonomic neuropathyqêÉ~íãÉåí= çÑ= ~ìíçåçãáÅ= åÉìêçé~íÜó= êÉä~íÉë= ãçëíäó= íç= íêÉ~íáåÖ

ëóãéíçã~íáÅ=éçëíìê~ä=ÜóéçíÉåëáçåK=jÉíçÅäçéê~ãáÇÉ=~åÇ=ÇçãéÉêáÇçåÉ=Å~åÄÉ= ÜÉäéÑìä= áå= é~íáÉåíë= ïáíÜ= Çá~ÄÉíáÅ= Ö~ëíêçé~êÉëáëK= aá~êêÜçÉ~= Å~å= ÄÉÅçåíêçääÉÇ=Äó=fãçÇáìãΔ çê=Äó=çÅíêÉçíáÇÉI=~=ëçã~íçëí~íáå=~å~äçÖìÉK=sá~Öê~~åÇ=í~Ç~ä~Ñáä=Å~å=ÄÉ=íêáÉÇ=áå=é~íáÉåíë=Åçãéä~áåáåÖ=çÑ=ëÉñì~ä=áãéçíÉåÅÉK



118

Sensory ataxia and diabetic neuropathy

Richard, a 66-year-old man, had a 35-year history of non-insulin-dependent diabetes

complicated by background retinopathy, micro- and macroangiopathy,

hypothyroidism, and mild renal insufficiency. He was on permanent anticoagulation

for atrial fibrillation.

In August 2011, he started to complain of numbness in both feet, and quickly felt

balance problems. Two months later, the numbness had progressed to the hands.

Electromyography (EMG) was performed in October 2011 which failed to detect any

sensory action potential in the lower limbs and in the median and radial nerves on

both sides. F-wave distal latency of the right posterior tibial nerve was increased.

The patient was referred to the Neurology Department of the hospital in November

2011. His strength was normal in all four limbs. He had a fibre-length-dependent

alteration of superficial sensation predominantly on pinprick and temperature

sensation. All four limbs and the anterior trunk were affected. Position sense was

impaired in the right foot. He had sensory ataxia. All tendon reflexes were abolished.

There was no meaningful autonomic disturbance. The CSF contained 1g/L protein

and there were no cells found (0 cell/ml in the CSF = normal).

The superficial peroneal nerve was biopsied under local anaesthesia. The density of

nerve fibres was reduced to 40% of normal values. There were many clusters of

regenerating fibres and most surviving fibres were hypomyelinated. There were no

inflammatory infiltrates. The endoneurial blood vessels showed features of diabetic

microangiopathy.

Continued overleaf



119

Sensory ataxia and diabetic neuropathy continued

A large majority of the isolated teased fibres showed segmental abnormalities with

demyelinating-remyelinating features and short internodes. A few fibres were at a

late stage of Wallerian degeneration.

Ultra-thin sections of the nerve specimens stained with uranyl acetate and lead

citrate were examined under the electron microscope. The only salient feature was

the striking reduction of the density of unmyelinated axons, in agreement with a

longstanding diabetic polyneuropathy. An isolated endoneurial capillary showed

changes suggestive of diabetic microaneurysms.

In November 2011 there was a marked increase of sensory deficit with minimal

weakness in the hands (strength 4/5). The diagnosis of chronic inflammatory

demyelinated polyneuropathy (CIDP) was considered and treatment with high-dose

intravenous immunoglobulins (IVIGs) was given from November 13 to November 16,

without success.

In December the patient was quadriplegic. Methylprednisolone was then given

intravenously (500mg/d for 5 days), followed by oral prednisone (65mg/day). The

patient started to improve after a week. After 2 months on corticosteroids the Norris

score was 36/81. In March 2012, the Norris score was 78/81 (prednisone was

gradually decreased from 65mg to 40mg/d). Corticosteroids were tapered over a few

months. The patient remained well neurologically, until his death which occurred 2

years later, from ischaemic heart disease.

This patient had a CIDP which complicated the course of a mild diabetic

polyneuropathy. It is worth noting the good response of CIDP to corticosteroids after

the failure of IVIGs.



120

Severe length-dependent diabetic polyneuropathy

Rachel, a 24-year-old, had been diagnosed with type 1 diabetes since the age of 8

years and had been treated with insulin ever since, with poor glycaemic control. Her

glycated haemoglobin (HbA1c) was often above 10%. She had micro- and

macroproteinuria for years. She was referred at age 24.

Previously, at age 22, she started to notice that she could not feel the temperature

of the water in her bath but light touch remained normal. In the following years she

experienced increasing postural hypotension, with dizziness and fainting upon

standing. Her blood pressure which was 140/65mmHg in the lying position fell to

60mmHg systolic pressure within less than a minute upon standing (diastolic

pressure was extremely low), with dizziness and fainting if she did not lie down

quickly. Her pulse rate was 76 per minute when lying down but remained unchanged

upon standing, which is characteristic of neurogenic postural hypotension. In

addition to life-threatening postural hypotension she had episodes of vomiting and

gastroparesis which increased hypotension. Postural hypotension responded well to

treatment with 9-alpha-fluorohydrocortisone, 100μg/day.

She gradually developed proliferative retinopathy and nephropathy with subsequent

renal failure. At the age of 32 years, she had lost temperature sense over the whole

of her body, but light touch and vibratory sensation had not changed. Muscle

strength and position sense remained normal.

Periodic haemodialysis was started when she was 33 years old; she died a year later

from septic shock.

This case illustrates the occurrence of extremely severe small fibre neuropathy

affecting temperature and pain sensation, with life-threatening autonomic

disturbances.



121

Late-onset sensory diabetic polyneuropathy

Jack is a 52-year-old banker who consulted for burning pains in his feet. Pains started

approximately 6 months before referral, in both feet, slightly predominating in the

right one. Pains were worse at night and increased on contact with the bed sheets.

The patient could not sleep well and had to get up and walk around in the middle of

the night. He measured 184cm and weighed 95kg. He had lost approximately 5kg

voluntarily.

Upon examination muscle strength was normal. Temperature and pin-prick

sensations were markedly decreased in the lower limbs, up to the mid-leg. Light

touch was decreased over the feet. Position sense was normal. Vibratory sensation

was abolished in the feet. The ankle reflexes were decreased. The upper limbs were

normal as well as the rest of the neurological and general examination. His family

history included type 2 diabetes in his father and elder brother.

EMG showed a marked decrease of sensory action potentials of sural nerves on both

sides, with normal conduction velocity. Blood tests were normal with the exception

of fasting blood sugar which was at 6.8mmol. Postprandial glucose was at 12mmol

and glycosylated haemoglobin was 8.7%. The patient had blood tests done 2 years

ago, which also had slightly abnormal sugar levels. The patient was treated with

amitriptyline and was refered to a diabetologist.

In conclusion, this patient had type 2 diabetes of late onset, with an axonal

polyneuropathy predominating on small fibres. Painful peripheral neuropathy is

often the first and only clinical manifestation of type 2 diabetes.


Focal and multifocal neuropathy`ê~åá~ä= åÉêîÉ= é~äëáÉë= áãéêçîÉ= ëéçåí~åÉçìëäó= ~åÇ= Çç= åçí= êÉèìáêÉ

ëéÉÅáÑáÅ=íêÉ~íãÉåíK=mêçñáã~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜó=áë=çÑíÉå=îÉêó=é~áåÑìä=~åÇëÜçìäÇ=ÄÉ=íêÉ~íÉÇ=ïáíÜ=é~ê~ÅÉí~ãçä=~åÇ=ÅçÇÉáåÉI=Ñçê=Éñ~ãéäÉK=páåÅÉ=ëçãÉé~íáÉåíë= ïáíÜ= Çáë~ÄäáåÖ= é~áåÑìä= éêçñáã~ä= åÉìêçé~íÜó= êÉëéçåÇ= çåäó= íçÅçêíáÅçëíÉêçáÇëI=íÜáë=íêÉ~íãÉåí=ëÜçìäÇ=ÄÉ=ÅçåëáÇÉêÉÇ=áå=ëÉîÉêÉ=ÑçêãëK=qÜáëïáää= êÉèìáêÉ= ~ÇàìëíãÉåí= çÑ= Çá~ÄÉíáÅ= Åçåíêçä= ïáíÜ= áåëìäáå= áå= ãçëí= Å~ëÉëKqêÉ~íãÉåí= çÑ= ãìäíáÑçÅ~ä= Çá~ÄÉíáÅ= åÉìêçé~íÜó= áë= Ä~ëÉÇ= çå= íÜÉ= ìëÉ= çÑÅçêíáÅçëíÉêçáÇë= Ñçê= ~= ÑÉï= ãçåíÜëK= qÜáë= êÉèìáêÉë= ~ÇàìëíãÉåí= çÑ= Çá~ÄÉíáÅÅçåíêçäK


122

Proximal diabetic neuropathy of the lower limbs

Louisa is a 55-year-old woman who had been treated for type 2 diabetes for 6 years.

She was a little overweight, but in good general condition. Her diabetes was well

controlled with a fasting glucose at 6.5mmol and glycosylated haemoglobin at 6.8%.

Two months before referral she started to complain of deep pain in the right thigh,

which had gradually increased during the previous weeks. This became worse at

night. The patient noticed spectacular wasting of the right thigh along with an

increasing difficulty to climb stairs. Tight diabetic control with insulin did not help.

She was treated with amitriptyline, and then with opiates. She had lost 6kg within 3

months. Neurological examination showed amyotrophy of the quadriceps muscle on

the right side along with weakness of this muscle and of flexion of the hip. Distal

strength was normal. She had sensory loss over the anterior aspect of the thigh and

bilateral stocking hypoaesthesia. The patellar reflex was abolished on the right side.

Magnetic resonance imaging (MRI) of the lumbar spine was normal. CSF examination

showed 2.5g/L protein; there were no cells found.

In summary, this patient had a proximal neuropathy of the right lower limb in

relation to type 2 diabetes.

After a few months the pain gradually disappeared and strength improved but she

retained a marked amyotrophy of the right thigh.


Uraemic neuropathy

Introduction

mçäóåÉìêçé~íÜó=áë=~=éçíÉåíá~ääó=ÅêáééäáåÖ=ÇáëçêÇÉê=çÑ=é~íáÉåíë=çå=Çá~äóëáëéêçÖê~ããÉëI=ïÜáÅÜ=Å~å=ÄÉ=éêÉîÉåíÉÇ=Äó=É~êäó=áåáíá~íáçå=çÑ=êÉÖìä~ê=Çá~äóëáëíêÉ~íãÉåí=~åÇ=êÉå~ä=íê~åëéä~åí~íáçåK=få=~=ÑÉï=é~íáÉåíëI=ÜçïÉîÉêI=áí=ã~ó=ëíáääÅ~ìëÉ=ëÉêáçìë=éêçÄäÉãëK=qÜÉ=Ñáêëí=êÉéçêí=çå=ìê~ÉãáÅ=åÉìêçé~íÜó=ï~ë=ã~ÇÉÄó=j~êáå=~åÇ=qóäÉê=áå=é~íáÉåíë=ïáíÜ=ÜÉêÉÇáí~êó=áåíÉêëíáíá~ä=åÉéÜêáíáë=ïáíÜ=Çáëí~äëÉåëçêó=ãçíçê=éçäóåÉìêçé~íÜó=OTK=qÜÉ=íÉêã=ìê~ÉãáÅ=éçäóåÉìêçé~íÜó=ï~ëíÜÉå=ìëÉÇ=Äó=^ëÄìêó=Éí=~ä OUK

kÉìêçé~íÜó=ÖÉåÉê~ääó=çåäó=ÇÉîÉäçéë=ïÜÉå=ÖäçãÉêìä~ê=Ñáäíê~íáçå=ê~íÉë=Ñ~ääíç= NOãäLãáåìíÉ= çê= ÄÉäçï= OVK= få= ãçëí= áåëí~åÅÉëI= ÄçíÜ= ïçêëÉåáåÖ= ~åÇáãéêçîÉãÉåí= ÉîçäîÉ= ëäçïäó= çîÉê= ãçåíÜëI= Äìí= çå= çÅÅ~ëáçå= åÉìêçé~íÜó= áëÑìäãáå~åí=áå=áíë=ÅçìêëÉK

Symptoms and signs

mÉêáéÜÉê~ä=åÉìêçé~íÜó=áë=ÜÉê~äÇÉÇ=Äó=ëóãéíçãë=çÑ=ÇóëÑìåÅíáçå=çÑ=äçïÉêãçíçê=åÉìêçåë=çê=çÑ=éêáã~êó=ëÉåëçêó=åÉìêçåë=PM EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çåéNOQFK=

jìëÅäÉ= Åê~ãéë= ~åÇ= êÉëíäÉëë= äÉÖë= ëóåÇêçãÉ= EoipF= Ü~îÉ= ÄÉÉåÅçåëáÇÉêÉÇ=íç=ÄÉ=ëóãéíçãë=çÑ=éÉêáéÜÉê~ä=åÉìêçå~ä=ÇóëÑìåÅíáçå=áå=ìê~ÉãáÅé~íáÉåíëI= Äìí= ÄçíÜ= ëóãéíçãë= ~Åíì~ääó= ä~Åâ= ëéÉÅáÑáÅáíóK= `çåîÉêëÉäóI= ïÜÉåé~íáÉåíë=Åçãéä~áå=çÑ=é~ê~ÉëíÜÉëá~ë=EíáåÖäáåÖ=çê=éêáÅâäáåÖF=áå=íÜÉ=íçÉëI=ÑÉÉíçê= ÑáåÖÉêëI= ÅäáåáÅ~ä= ëáÖåë= çÑ= ìê~ÉãáÅ= åÉìêçé~íÜó= ~êÉ= ìëì~ääó= éêÉëÉåí= ~åÇã~ó=ÉîÉå=ÄÉ=ëÉîÉêÉK=m~áå=áë=~ÄëÉåí=áå=íÜÉ=É~êäó=ëí~ÖÉë=çÑ=åÉìêçé~íÜó=Äìíã~ó= ÄÉ= éêçãáåÉåí= áå= ~Çî~åÅÉÇ= ~åÇ= ëÉîÉêÉ= åÉìêçé~íÜóK= aÉéêÉëëÉÇ= çê~ÄçäáëÜÉÇ=~åâäÉ=êÉÑäÉñÉë=~åÇ=~å=áãé~áêÉÇ=îáÄê~íáçå=éÉêÅÉéíáçå=çÑ=íÜÉ=íçÉë~êÉ= É~êäó= ~åÇ= áåáíá~ääó= íÜÉ= çåäó= ÅäáåáÅ~ä= ëáÖåë= çÑ= éÉêáéÜÉê~ä= åÉìêçé~íÜóKmÉêÅÉéíáçå= çÑ= äáÖÜí= íçìÅÜ= çîÉê= Çáëí~ä= äçïÉê= äáãÄë= áë= ~ÑÑÉÅíÉÇ= ÑáêëíK= få= ~ãáåçêáíó=çÑ=é~íáÉåíëI=åÉìêçé~íÜó=éêçÖêÉëëÉë=ÑìêíÜÉêK=^íêçéÜó=~åÇ=ïÉ~âåÉëëçÑ=Çáëí~ä= äÉÖ=ãìëÅäÉë=ã~ó=ÇÉîÉäçé=~ë=ïÉää=~ë=ÇáëíìêÄ~åÅÉ=çÑ=~ää= ëÉåëçêóãçÇ~äáíáÉë=áå=~=ëíçÅâáåÖJäáâÉ=ÇáëíêáÄìíáçåK=`äáåáÅ~ä=ëáÖåë=çÑ=åÉìêçé~íÜó=áå=íÜÉìééÉê= äáãÄë= çÅÅìê= çåäó= áå= ëÉîÉêÉ= Å~ëÉëI= ÉîÉå= íÜçìÖÜ= ~äíÉê~íáçåë= çÑ


123


ÅçåÇìÅíáçå= îÉäçÅáíó= ~êÉ= éêÉëÉåí= áå= ~ää= Ñçìê= äáãÄëK= j~åáÑÉëí~íáçåë= çÑ~ìíçåçãáÅ=åÉìêçé~íÜó=áåÅäìÇáåÖ=éçëíìê~ä=ÜóéçíÉåëáçåI=áãé~áêÉÇ=ëïÉ~íáåÖIÇá~êêÜçÉ~I=Åçåëíáé~íáçåI=çê=áãéçíÉåÅÉ=~êÉ=ìëì~ääó=åçí=ÅäáåáÅ~ääó=ã~åáÑÉëíK=

kÉêîÉ=ÅçåÇìÅíáçå=ëäçïáåÖ=çÅÅìêë=íç=~äãçëí=íÜÉ=ë~ãÉ=ÇÉÖêÉÉ=áå=ãçíçê~åÇ= ëÉåëçêó= åÉêîÉ= ÑáÄêÉëI= áå= åÉêîÉë= çÑ= ìééÉê= ~åÇ= äçïÉê= äáãÄë= ~åÇ= áåéêçñáã~ä= ~åÇ= Çáëí~ä= é~êíë= çÑ= äáãÄ= åÉêîÉëK= få= íÜÉ= ãçëí= ëÉîÉêÉ= Å~ëÉëÅçåÇìÅíáçå= ã~ó= Ñ~ää= íç= SM= çê= RMB= çÑ= íÜÉ= åçêã~ä= î~äìÉëKbäÉÅíêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= ëÜçìäÇ= ÄÉ= äáãáíÉÇ= íç= é~íáÉåíë= ïÜç= ã~åáÑÉëíëóãéíçãë= çÑ= åÉìêçé~íÜóK= ^= ëáÖåáÑáÅ~åí= ÇÉÅêÉ~ëÉ= çÑ= íÜÉ= ëÉåëçêó= ~ÅíáçåéçíÉåíá~ä=ãÉ~åë=íÜ~í= íÜÉ=é~íáÉåí=Ü~ë= äçëí=~=ëáÖåáÑáÅ~åí=éêçéçêíáçå=çÑ= ä~êÖÉãóÉäáå~íÉÇ=ÑáÄêÉë=áå=íÜÉ=åÉêîÉ=ëíìÇáÉÇK=qÜÉ=éçëáíáîÉ=åÉìêçé~íÜáÅ=ëóãéíçãëÅçêêÉä~íÉ= ïáíÜ= èì~åíáí~íáîÉ= îáÄê~íçêó= ÇÉíÉÅíáçå= íÜêÉëÜçäÇë= ~åÇ= ëÉåëçêóåÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉëI=ÉëéÉÅá~ääó= íÜÉ=~ãéäáíìÇÉ=çÑ= íÜÉ=ëÉåëçêó=åÉêîÉ~Åíáçå=éçíÉåíá~ä=áå=íÜÉ=ëìê~ä=åÉêîÉ=PNK

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124

Uraemic neuropathy

Alice is a 63-year-old retired midday supervisor who had suffered with hypertension

for 20 years and mature-onset diabetes mellitus for 9 years. She had had gradually

failing renal function. Her glomerular filtration rate (eGFR) had been approximately

30ml/minute for the last couple of years, but she had failed to attend for her usual

reviews for about 6 months. She presented with painful cold feet with a dysaesthetic

component. Examination revealed a loss of light touch, vibration and pain perception

in her toes, and her ankle reflexes were absent. Her peripheral neuropathy was

initially attributed to her diabetes, which had been poorly controlled. Her eGFT came

back at 10ml/minute, however. She was referred to the renal service and started on

thrice-weekly haemodialysis. Within 3 months of starting dialysis her abnormal

sensations in the feet had resolved; however, her loss of vibration perception and her

ankle jerks did not return.


åçåJìê~ÉãáÅ=áåÇáîáÇì~äëK=få=íÜáë=ëáíì~íáçåI=ïÜáÅÜ=áë=Äó=Ñ~ê=íÜÉ=ãçëí=ÅçããçåIíÜÉ= éêÉÑÉêêÉÇ= ã~å~ÖÉãÉåí= áë= ïáíÜ= ëìêÖáÅ~ä= Å~êé~ä= íìååÉä= êÉäÉ~ëÉK= `~êÉãìëí= ÄÉ= í~âÉå= íç= çÄí~áå= ~= Äáçéëó= ëéÉÅáãÉå= çÑ= íÜÉ= ÑäÉñçê= êÉíáå~Åìäìã= íçÇÉíÉêãáåÉ=íÜÉ=éêÉëÉåÅÉ=çÑ=~ãóäçáÇK=lÅÅ~ëáçå~ääóI=íÜÉ=`qp=ã~ó=ÄÉ=êÉä~íÉÇíç= íÜÉ= éêÉëÉåÅÉ= çÑ= ~å= ~êíÉêáçîÉåçìë= Ñáëíìä~= áå= íÜÉ= ÑçêÉ~êã= ~åÇ= Å~å= ÄÉã~å~ÖÉÇ=Äó=ÅäçëìêÉ=çÑ=íÜÉ=Ñáëíìä~K

Neuropathy and the degree of renal insufficiency

få= é~íáÉåíë= êÉÖìä~êäó= ÅçåíêçääÉÇ= áå= çìíé~íáÉåí= ÇÉé~êíãÉåíëI= ëáÖåë= çÑåÉìêçé~íÜó= ~êÉ= ÖÉåÉê~ääó= ä~ÅâáåÖ= ~ë= äçåÖ= ~ë= íÜÉ= ÅêÉ~íáåáåÉ= ÅäÉ~ê~åÅÉÉñÅÉÉÇë=SMãäLãáåìíÉK=pìÅÅÉëëÑìä=âáÇåÉó=íê~åëéä~åí~íáçå=áë=ÑçääçïÉÇ=Äó=~íïçJéÜ~ëÉ=ÅçìêëÉ=çÑ=êÉÅçîÉêó=çÑ=åÉìêçé~íÜóK=qÜÉ=Ñáêëí=éÜ~ëÉ=áë=ÄêáÉÑ=~åÇÄÉÖáåë=ïáíÜáå=~=ÑÉï=Ç~óë=~ÑíÉê=íê~åëéä~åí~íáçåK=kÉêîÉ=ÅçåÇìÅíáçå=îÉäçÅáíóáåÅêÉ~ëÉë= ~åÇ= ÅäáåáÅ~ä= ëáÖåë= ê~éáÇäó= áãéêçîÉK= qÜÉ= ëÉÅçåÇ= éÜ~ëÉ= çÅÅìêëëäçïäó=~åÇ=ä~ëíë=ã~åó=ãçåíÜë=~åÇ=êÉëìäíë=Ñêçã=~ñçå~ä=êÉÖÉåÉê~íáçåK=cìääÅäáåáÅ~ä=êÉÅçîÉêó=çÅÅìêë=áå=ãçëí=é~íáÉåíë=~åÇ=åÉêîÉ=ÅçåÇìÅíáçå=ã~ó=~íí~áååçêã~ä= äÉîÉäëI= ~äíÜçìÖÜ= éÉêëáëíÉåÅÉ= çÑ= ëçãÉ= ÇÉä~ó= çÑ= ÅçåÇìÅíáçåIé~êíáÅìä~êäó=áå=äçïÉê=äáãÄ=åÉêîÉëI=áë=åçí=ìåìëì~äK

Uraemic neuropathy in diabetic patients

få=Çá~ÄÉíáÅ=é~íáÉåíë=ïáíÜ=êÉå~ä=áåëìÑÑáÅáÉåÅó=ìåÇÉê=Çá~äóëáë=íêÉ~íãÉåíI=íÜÉçÅÅìêêÉåÅÉ= çÑ= ëÉîÉêÉ= ~ñçå~äI= äÉåÖíÜJÇÉéÉåÇÉåíI= ëÉåëçêó= ~åÇ= ãçíçêéçäóåÉìêçé~íÜó= áë= ÅçããçåK= få= ëìÅÜ= é~íáÉåíë= áí= áë= îáêíì~ääó= áãéçëëáÄäÉ= íç~ëÅêáÄÉ=ïáíÜ=ÅÉêí~áåíó=ëéÉÅáÑáÅ=ëáÖåë=çê=ëóãéíçãë=íç=çåÉ=çê= íç= íÜÉ=çíÜÉêãÉí~ÄçäáÅ= ÇáëíìêÄ~åÅÉK= eçïÉîÉêI= ëÉîÉêÉ= ëóãéíçã~íáÅ= ~ìíçåçãáÅÇáëíìêÄ~åÅÉëI= é~áåI= ~åÇ= ~= äÉåÖíÜJÇÉéÉåÇÉåí= é~ííÉêå= çÑ= ~= äçëë= çÑíÉãéÉê~íìêÉ=~åÇ=é~áå=ëÉåë~íáçåI=éçáåí=íç=íÜÉ=éêÉÇçãáå~åí=áåîçäîÉãÉåí=çÑëã~ää=ãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇ=ÑáÄêÉëI=~åÇ=~êÉ=ãçêÉ=ÅÜ~ê~ÅíÉêáëíáÅ=çÑÇá~ÄÉíáÅ=åÉìêçé~íÜó=POK=`çåîÉêëÉäóI=~=éêçãáåÉåí=ãçíçê=ÇÉÑáÅáí=áë=áåÇáÅ~íáîÉçÑ= ~= éêÉÇçãáå~åí= êçäÉ= Ñçê= ìê~Éãá~K= ^ÑíÉê= êÉå~ä= íê~åëéä~åí~íáçåI= íÜÉéÉêáéÜÉê~ä= ÇÉÑáÅáí= ïÜáÅÜ= áë= êÉä~íÉÇ= íç= ìê~Éãá~= ïçìäÇ= ÄÉ= ~åíáÅáé~íÉÇ= íçáãéêçîÉK

få=ÅçåÅäìëáçåI=ìê~ÉãáÅ=åÉìêçé~íÜó=Å~å=åçï=ÄÉ=éêÉîÉåíÉÇ=Äó=É~êäó=~åÇáãéêçîÉÇ=éÉêáçÇáÅ=Ü~ÉãçÇá~äóëáë=~åÇ=êÉå~ä=íê~åëéä~åí~íáçåK=


125


References

NK= fåíÉêå~íáçå~ä= aá~ÄÉíÉë= cÉÇÉê~íáçåK= fac= aá~ÄÉíÉë= ^íä~ëI= SíÜ= ÉÇåK= _êìëëÉäëI= _ÉäÖáìãW

fåíÉêå~íáçå~ä=aá~ÄÉíÉë=cÉÇÉê~íáçåI=OMNPK=ÜííéWLLïïïKáÇÑKçêÖLÇá~ÄÉíÉë~íä~ëK

OK= máê~êí=gK=aá~ÄÉíÉë=ãÉääáíìë=~åÇ= áíë=ÇÉÖÉåÉê~íáîÉ=ÅçãéäáÅ~íáçåëW=~=éêçëéÉÅíáîÉ=ëíìÇó=çÑ

QQMM= é~íáÉåíë= çÄëÉêîÉÇ= ÄÉíïÉÉå= NVQT= ~åÇ= NVTPK= aá~ÄÉíÉë= `~êÉ NVTUX= NW= NSUJUUI

ORPJSPK

PK= e~êêáë= jI= b~ëíã~å= oI= `çïáÉ= `K= póãéíçãë= çÑ= ëÉåëçêó= åÉìêçé~íÜó= áå= ~Çìäíë= ïáíÜ

kfaaj=áå=íÜÉ=rKpK=éçéìä~íáçåK=aá~ÄÉíÉë=`~êÉ NVVPX=NSW=NQQSJROK

QK= vçìåÖ= ogI= wÜçì= vnI= oçÇêáÖìÉò= bI= Éí= ~äK= s~êá~ÄäÉ= êÉä~íáçåëÜáé= ÄÉíïÉÉå= éÉêáéÜÉê~ä

ëçã~íáÅ= ~åÇ= ~ìíçåçãáÅ= åÉìêçé~íÜó= áå= é~íáÉåíë= ïáíÜ= ÇáÑÑÉêÉåí= ëóåÇêçãÉë= çÑ= Çá~ÄÉíáÅ

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aÉ= ä~= ÇáëëçÅá~íáçå= ëóêáåÖçãó¨äáèìÉ= ÇÉ= ä~= ëÉåëáÄáäáí¨= ÅÜÉò= äÉë= Çá~Ä¨íáèìÉëK= d~òÉííÉ

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126

√ Diabetic neuropathy is the most common neuropathy in the world.

√ Distal symmetrical sensory polyneuropathy of insidious onset is the most

common pattern of diabetic neuropathy. It often reveals type 2 diabetes of

mature onset.

√ Pain and trophic lesions in the foot (arthropathy and plantar ulcers) due to

sensory denervation are common.

√ Autonomic neuropathy can induce impotence, postural hypotension and

diarrhoea.

√ Diabetic oculomotor nerve palsies are spontaneously and completely

reversible.

√ Proximal neuropathy of the lower limbs is usually painful and disabling, with

a self-limiting course.

√ Uraemic neuropathy must be prevented by early periodic haemodialysis and

renal transplantation.

Ü Key points


http://www.idf.org/diabetesatlas

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127


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129

Neuropathies in patients with monoclonal

gammopathy and malignancy

Chapter 9

Overview

In developed countries just under half of us will develop cancer at some stage in

our lives and half the people who get cancer will live with the disease for more

than 10 years. A number of cancers are associated with peripheral neuropathy. In

addition, the treatment of cancer can be associated with the development of

neuropathy. This chapter discusses the manifestations of neuropathy associated

with cancer and related conditions.

Introduction

jçåçÅäçå~ä=Ö~ããçé~íÜó=áë=~=ÅçåÇáíáçå=ïÜÉêÉÄó=~Äåçêã~ä=éêçíÉáåë=~êÉÑçìåÇ=áå=íÜÉ=ÄäççÇK=qÜÉëÉ=éêçíÉáåë=ÇÉîÉäçé=Ñêçã=~=ëã~ää=åìãÄÉê=çÑ=éä~ëã~ÅÉääë= áå=íÜÉ=ÄçåÉ=ã~êêçïK=qÜÉ=ãçëí=Åçããçå=ÅçåÇáíáçå=äáåâÉÇ=ïáíÜ=íÜÉëÉ~Äåçêã~ä= éêçíÉáåë= áë= ãçåçÅäçå~ä= Ö~ããçé~íÜó= çÑ= ìåÇÉíÉêãáåÉÇëáÖåáÑáÅ~åÅÉ= EjdrpFK= jdrp= áë= åçí= Å~åÅÉêI= Äìí= ê~íÜÉê= ~= éêÉã~äáÖå~åíéä~ëã~= ÅÉää= ÇáëçêÇÉê= ïÜáÅÜ= áë= éêÉëÉåí= áå= ãçêÉ= íÜ~å= PB= çÑ= íÜÉ= ÖÉåÉê~äéçéìä~íáçå=~ÖÉÇ=çîÉê=RM=óÉ~êë=NI=OK=mÉçéäÉ=ïáíÜ=jdrp=Ü~îÉ=~å=áåÅêÉ~ëÉÇêáëâ=çÑ=ÇÉîÉäçéáåÖ=ëÉêáçìë=ÇáëÉ~ëÉë=çÑ=íÜÉ=ÄçåÉ=ã~êêçï=~åÇ=ÄäççÇK=


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j~äáÖå~åí=ÅçåÇáíáçåë=ã~ó=Ü~îÉ=~=ÇáêÉÅí=ÉÑÑÉÅí=ìéçå=éÉêáéÜÉê~ä=åÉêîÉë=Äóáåî~ëáçå= çÑ= êççíë= çê= åÉêîÉ= íêìåâë= Äó= ã~äáÖå~åí= ÅÉääëI= çê= ~= êÉãçíÉ= ÉÑÑÉÅííÜêçìÖÜ= é~ê~åÉçéä~ëíáÅ= ã~åáÑÉëí~íáçåëK= ^äëçI= íêÉ~íãÉåí= çÑ= Å~åÅÉê= ÄóÅÜÉãçíÜÉê~éó= çê= Äó= áêê~Çá~íáçå= ã~ó= Ü~îÉ= ~= ÇÉäÉíÉêáçìë= ÉÑÑÉÅí= çå= íÜÉéÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉã=EmkpFK

Neuropathy and monoclonal gammopathy

Benign monoclonal gammopathy

_ÉåáÖå= ãçåçÅäçå~ä= Ö~ããçé~íÜó= Å~å= ÄÉ= ~ëëçÅá~íÉÇ= ïáíÜ= ÅÜêçåáÅáåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜó=E`famFK=qÜáë=áë=çÑíÉå=íÜÉ=Å~ëÉÑçê= íÜÉ= áããìåçÖäçÄìäáå= j= EfÖjF= â~éé~= ãçåçÅäçå~ä= Ö~ããçé~íÜóK^åíáÄçÇáÉë=ÇáêÉÅíÉÇ=~Ö~áåëí=íÜÉ=ãóÉäáåJ~ëëçÅá~íÉÇ=ÖäóÅçéêçíÉáå=Ej^dF=~êÉçÑíÉå=~ëëçÅá~íÉÇ=ïáíÜ= fÖj=ãçåçÅäçå~ä=Ö~ããçé~íÜó=~åÇ=éçäóåÉìêçé~íÜóKqÜÉ=éáÅíìêÉ=~åÇ=ÅçìêëÉ=çÑ=íÜÉ=åÉìêçé~íÜó=áë=îÉêó=ëáãáä~ê=íç=íÜ~í=çÑ=`famIÄìí= óÉ~êäó= êÉîáÉï= çÑ= íÜÉ= Ö~ããçé~íÜó= áë= åÉÅÉëë~êó= ÇìÉ= íç= íÜÉ= éçëëáÄäÉã~äáÖå~åí=íê~åëÑçêã~íáçå=çÅÅìêêáåÖ=áå=íÜáë=ëÉííáåÖK=

Monoclonal gammopathy and light chain amyloidosis

jçåçÅäçå~ä= Ö~ããçé~íÜó= Eãçëíäó= fÖdF= Å~å= ~äëç= ÄÉ= ~ëëçÅá~íÉÇ= ïáíÜäáÖÜí= ÅÜ~áå= ~ãóäçáÇçëáëK= qÜáë= ÇáëçêÇÉê= áë= ëìëéÉÅíÉÇ= áå= é~íáÉåíë= ïÜçÇÉîÉäçé= ~= ëìÄ~ÅìíÉ= éêçÖêÉëëáîÉ= äÉåÖíÜJÇÉéÉåÇÉåí= ëÉåëçêó= ~åÇ= ãçíçêéçäóåÉìêçé~íÜó= ~ëëçÅá~íÉÇ= ïáíÜ= ëÉêáçìë= ~ìíçåçãáÅ= ã~åáÑÉëí~íáçåë= ~åÇÅ~êé~ä= íìååÉä= ëóåÇêçãÉK= `~êÇá~Å= ã~åáÑÉëí~íáçåëI= áåÅäìÇáåÖ= ~åáåíê~Å~êÇá~Å= ÅçåÇìÅíáçå= ÇÉÑÉÅí= ~åÇ= Å~êÇá~Å= Ñ~áäìêÉI= ~êÉ= Åçããçåã~åáÑÉëí~íáçåë=áå=íÜáë=ÅçåÇáíáçåK=qÜÉ=Çá~Öåçëáë=êÉëíë=çå=ÇÉãçåëíê~íáçå=çÑ~ãóäçáÇ=ÇÉéçëáíë= áå=íáëëìÉ=Äáçéëó=~åÇ=ÉñÅäìëáçå=çÑ=~=ÜÉêÉÇáí~êó=Ñçêã=çÑ~ãóäçáÇçëáë=EcáÖìêÉ=NFK


130


The POEMS syndrome (polyneuropathy, organomegaly,endocrinopathy, monoclonal gammopathy, skin changes)

mlbjp= ëóåÇêçãÉ= áë= ~= ê~êÉ= ãìäíáëóëíÉã= ÇáëÉ~ëÉ= ~ëëçÅá~íÉÇ= ïáíÜéä~ëã~= ÅÉää= ÇóëÅê~ëá~= PK= mlbjp= áë= ÇÉÑáåÉÇ= Äó= íÜÉ= ~ëëçÅá~íáçå= çÑéÉêáéÜÉê~ä=åÉìêçé~íÜó=~åÇ=~=ãçåçÅäçå~ä=éä~ëã~=ÅÉää=ÇáëçêÇÉê=ïáíÜ=çíÜÉêëóëíÉãáÅ= ÑÉ~íìêÉë= áåÅäìÇáåÖ=çêÖ~åçãÉÖ~äó= EëéäÉåçãÉÖ~äóI=ÜÉé~íçãÉÖ~äóIçê= äóãéÜ~ÇÉåçé~íÜóFI= ÉåÇçÅêáåçé~íÜó= E~ÇêÉå~äI= íÜóêçáÇI= éáíìáí~êóI= ëÉîÉêÉÇá~ÄÉíÉëI= Öçå~Ç~äI= ~åÇ= çíÜÉêëFI= ~åÇ= ëâáå= ÅÜ~åÖÉë= Eé~êíáÅìä~êäóÜóéÉêéáÖãÉåí~íáçåI= ~ÅêçÅó~åçëáëI= ïÜáíÉ= å~áäëI= ÜóéÉêíêáÅÜçëáëI= ~åÇÜ~Éã~åÖáçã~í~FK= mÉêáéÜÉê~ä= çÉÇÉã~I= éäÉìê~ä= ÉÑÑìëáçåë= çê= ~ëÅáíÉëIéìäãçå~êó= ÜóéÉêíÉåëáçåI= êÉå~ä= áåîçäîÉãÉåí= EãÉë~åÖáçÅ~éáää~êóJäáâÉÖäçãÉêìäçåÉéÜêáíáëFI=é~éáääçÉÇÉã~I=íÜêçãÄçÅóíçëáëI=~åÇ=éçäóÅóíÜÉãá~=~äëççÅÅìê= áå= íÜáë= ëóåÇêçãÉK= sáêíì~ääó= ~ää= é~íáÉåíë= Ü~îÉ= ÉáíÜÉê= ~í= äÉ~ëí= çåÉëÅäÉêçíáÅ=ÄçåÉ=äÉëáçå=Eê~êÉäó=äóíáÅ=äÉëáçåëF=çê=ÅçÉñáëíÉåí=`~ëíäÉã~å=ÇáëÉ~ëÉEcáÖìêÉ= OFK= jçåçÅäçå~ä= éêçíÉáåë= ~êÉ= ìëì~ääó= fÖd= çê= fÖ^= ~åÇ= ~äãçëíáåî~êá~Ääó=ÄÉ~ê= íÜÉ= ä~ãÄÇ~= äáÖÜí=ÅÜ~áåK=qÜÉáê= äÉîÉä= áë=çÑíÉå=èìáíÉ= äçïK=qÜÉ

Chapter 9 Neuropathies in patients with monoclonal gammopathy and malignancy

131

Figure 1. Nerve biopsy from a patient with monoclonal

gammopathy and a severe sensory-motor polyneuropathy due

to light chain amyloidosis. Endoneurial amyloid deposits are

illustrated by arrows. Note the nearly complete disappearance

of nerve fibres, in keeping with this severe neuropathy. (Bar

10μm.)


åÉìêçé~íÜó=áå=mlbjp=áë=èìáíÉ=ëÉîÉêÉ=~åÇ=çÑíÉå=éêÉëÉåíë=ëìÄ~ÅìíÉäó=~åÇáë=çÑíÉå=ê~éáÇäó=Çáë~ÄäáåÖK=fí=áë=éêÉÇçãáå~åíäó=~=ãçíçê=åÉìêçé~íÜóI=ÑêÉèìÉåíäóëí~êíáåÖ=ïáíÜ=ëÉåëçêó=ëóãéíçãëK=lÑíÉåI=é~íáÉåíë=~êÉ=áåáíá~ääó=Çá~ÖåçëÉÇ=ïáíÜ`fam= ÇìÉ= íç= íÜÉ= ëìÄ~ÅìíÉ= éêçÖêÉëëáçå= áå= íÉêãë= çÑ= ãçåíÜë= ~åÇ= íÜÉéêçãáåÉåí=ÇÉãóÉäáå~íáåÖ=ÑÉ~íìêÉë=çå=åÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉë=EcáÖìêÉ=PFKmä~ëã~=~åÇ=ëÉêìã=äÉîÉäë=çÑ=î~ëÅìä~ê=ÉåÇçíÜÉäá~ä=ÖêçïíÜ=Ñ~Åíçê=EsbdcF=~êÉã~êâÉÇäó=ÉäÉî~íÉÇ=áå=é~íáÉåíë=ïáíÜ=mlbjp=~åÇ=ÅçêêÉä~íÉ=ïáíÜ=íÜÉ=~ÅíáîáíóçÑ= íÜÉ= ÇáëÉ~ëÉ= ~åÇ= êÉëéçåëÉ= íç= íÜÉê~éóK= fêê~Çá~íáçå= çê= ëìêÖáÅ~ä= ÉñÅáëáçåêÉã~áå= íÜÉ= íÜÉê~éó= çÑ= ÅÜçáÅÉ= Ñçê= é~íáÉåíë= ïáíÜ= Ççãáå~åí= ëÅäÉêçíáÅéä~ëã~Åóíçã~=PI=ïÜáäÉ=é~íáÉåíë=ïáíÜ=ÇáÑÑìëÉ=äÉëáçåë=çê=ÇáëëÉãáå~íÉÇ=ÄçåÉã~êêçï=áåîçäîÉãÉåí=ëÜçìäÇ=ìåÇÉêÖç=ëóëíÉãáÅ=ÅÜÉãçíÜÉê~éóK


132

Figure 2. CT scan of the sacrum showing a sclerotic bone

lesion (arrow) in a patient with POEMS syndrome.

Figure 3. Teased fibre preparation from a nerve biopsy of a patient with POEMS

syndrome, showing demyelination of nerve fibres.


Malignant cell infiltration of the PNS

fåÑáäíê~íáçå=çÑ=íÜÉ=mkp=Äó=ã~äáÖå~åí=ÅÉääë=áë=êÉä~íáîÉäó=ê~êÉI=Åçãé~êÉÇ=íçåÉìêçé~íÜáÉë= êÉä~íÉÇ= íç= íçñáÅáíó= çÑ= ãÉÇáÅ~íáçåë= ìëÉÇ= íç= íêÉ~í= ã~äáÖå~åíÇáëçêÇÉêëI=çê=íç=ê~Çá~íáçåJáåÇìÅÉÇ=åÉêîÉ=äÉëáçåëK=j~äáÖå~åí=ÅÉää= áåÑáäíê~íáçåãçêÉ=Åçããçåäó=~ÑÑÉÅíë=ëéáå~ä=êççíë=íÜ~å=éÉêáéÜÉê~ä=åÉêîÉëK=fåÑáäíê~íáçå=çÑíÜÉ= mkp= ïáíÜáå= íÜÉ= ëìÄ~ê~ÅÜåçáÇ= ëé~ÅÉ= çÅÅìêë= áå= äÉìâ~Éãá~= ~åÇ= áåÇáÑÑÉêÉåí=íóéÉë=çÑ=Å~êÅáåçã~=EcáÖìêÉ=QFK=^=ãìäíáÑçÅ~ä=ëÉåëçêóJãçíçê=ÇÉÑáÅáíI~ÑÑÉÅíáåÖ=ëÉîÉê~ä= êççí= íÉêêáíçêáÉëI=ã~ó=ÄÉ= íÜÉ= áåáíá~ä=~åÇ=çåäó=ëóãéíçã~íáÅã~åáÑÉëí~íáçå=çÑ=Å~åÅÉêK=j~ÖåÉíáÅ=êÉëçå~åÅÉ=áã~ÖáåÖ=EjofF=çÑ=ëéáå~ä=êççíëã~ó= ëÜçï= ãìäíáéäÉ= ëã~ää= íìãçìêëI= ~åÇ= ÅÉêÉÄêçëéáå~ä= ÑäìáÇ= E`pcFÉñ~ãáå~íáçå= êÉîÉ~äë= ~= ÜáÖÜ= éêçíÉáå= ÅçåíÉåí= ïáíÜ= äçï= ÖäìÅçëÉ= ~åÇ= íÜÉéêÉëÉåÅÉ= çÑ= ã~äáÖå~åí= ÅÉääëK= pìÄ~ê~ÅÜåçáÇ= ÅÜÉãçíÜÉê~éó= çÑíÉå= ÖáîÉëÖççÇ=êÉëìäíë=QK

fåî~ëáçå=çÑ= íÜÉ=Äê~ÅÜá~ä=éäÉñìë=çÅÅìêë= áå=é~íáÉåíë=ïáíÜ=Å~êÅáåçã~=çÑíÜÉ= äìåÖI=ëçãÉíáãÉë=çå=ÄçíÜ=ëáÇÉë=Em~åÅç~ëí=íìãçìêFK=qÜáë= áåî~ëáçå=çÑíÜÉ=mkp=~äëç=ã~ó=çÅÅìê= áå=~ëëçÅá~íáçå=ïáíÜ= äóãéÜçã~ë=EÜ ëÉÉ=Å~ëÉëíìÇó=çîÉêäÉ~ÑF=~åÇ=ëçãÉíáãÉë=áë=íÜÉ=áåáíá~ä=ã~åáÑÉëí~íáçå=çÑ=äóãéÜçã~=~ë


133

Figure 4. Nerve biopsy findings in a patient with plasma cell

leukaemia and relapsing multifocal neuropathy. Note the

massive infiltration of malignant plasma cells in the nerve

fascicle (asterisk). (Bar 50μm.)

*


áå= åÉìêçäóãéÜçã~íçëáëI= ïÜáÅÜ= áë= ~ëëçÅá~íÉÇ= ïáíÜ= ãçåçÅäçå~äÖ~ããçé~íÜóK

Paraneoplastic neuropathies

m~ê~åÉçéä~ëíáÅ=ëóåÇêçãÉë=~ÑÑÉÅí=íÜÉ=mkp=áå=ìé=íç=çåÉ=íÜáêÇ=çÑ=é~íáÉåíëïáíÜ=ëçäáÇ=íìãçìêëK=qÜÉ=Åä~ëëáÅ=ëóåÇêçãÉ=Åçåëáëíë=çÑ=~=ëìÄ~ÅìíÉ=ëÉåëçêóåÉìêçåçé~íÜóK= eçïÉîÉêI= çíÜÉê= î~êá~íáçåë= áå= íÜÉ= ê~íÉ= çÑ= éêçÖêÉëëáçå= çÑåÉìêçé~íÜóI= é~ííÉêå= çÑ= åÉìêçé~íÜóI= ÇÉÖêÉÉ= çÑ= ëÉåëçêóI= ãçíçêI= ~åÇ~ìíçåçãáÅ= áåîçäîÉãÉåíI= ~åÇ= íÜÉ= éêÉëÉåÅÉ= çê= ~ÄëÉåÅÉ= çÑ= ëéÉÅáÑáÅçåÅçåÉìê~ä=~åíáÄçÇáÉë=Å~å=çÅÅìê=RK=

qÜÉ=íÉêã=ÚçåÅçåÉìê~ä=~åíáÄçÇáÉëÛ=Eé~ê~åÉçéä~ëíáÅ=~åíáÄçÇáÉëF=~ééäáÉë=íç~åíáÄçÇáÉë=íÜ~í=í~êÖÉí=~åíáÖÉåë=éêÉëÉåí=áå=åÉêîçìë=íáëëìÉ=~åÇ=íìãçìêëI=íÜìëé~ê~åÉçéä~ëíáÅ= åÉìêçäçÖáÅ~ä= ëóåÇêçãÉë= ÅçìäÇ= ÄÉ= íÜÉ= êÉëìäí= çÑ= ~å


134

Demyelinating polyneuropathy

Mohammed is a 31-year-old security guard who developed a swelling in the left side

of his neck. He was investigated and found to have widespread lymphadenopathy. A

biopsy was undertaken and misinterpreted as showing changes consistent with a

diagnosis of tuberculosis (TB). He was started on quadruple therapy of isoniazid,

rifampicin, pyrazinamide and ethambutol with pyridoxine 10mg a day. He developed

painful feet and by the time he was admitted to hospital he could not walk. Initially,

consideration was given to a drug-induced neuropathy. The diagnosis was reviewed

and a further biopsy demonstrated lymphoma. Peripheral neurophysiology showed

changes consistent with a demyelinating polyneuropathy. Within a month of

commencing his chemotherapy for his lymphoma his neuropathy had started to

improve. By 6 months after the initiation of chemotherapy he was able to walk,

albeit unsteadily. After 3 years, he remains free of lymphoma, but he has a loss of

pain perception in his feet as well as a loss of vibration sense and proprioception.

There is wasting and weakness of the muscles in his lower legs. He has significant

persistent neuropathic pain. He remains unemployed.


áããìåçäçÖáÅ~ä= êÉëéçåëÉ= íêáÖÖÉêÉÇ= ~Ö~áåëí= íìãçìê~ä= ~åíáÖÉåë= ~åÇãáëÇáêÉÅíÉÇ= íç=ëáãáä~ê=~åíáÖÉåë=ÉñéêÉëëÉÇ= áå= íÜÉ=åÉêîçìë=ëóëíÉãK=pã~ääJÅÉää= äìåÖ= Å~åÅÉê= áë= íÜÉ= Åçããçå= Å~ìëÉ= çÑ= é~ê~åÉçéä~ëíáÅ= éÉêáéÜÉê~äåÉìêçé~íÜóI=Äìí= áí= áë=~äëç=ëÉÉå= áå= äóãéÜçã~I=ÄêÉ~ëíI=~åÇ=Öóå~ÉÅçäçÖáÅ~äÅ~åÅÉêëI=~åÇ=íÜóãçã~K

pìÄ~ÅìíÉ= ëÉåëçêó= åÉìêçåçé~íÜó= áë= Å~ìëÉÇ= Äó= Ç~ã~ÖÉ= íç= ëÉåëçêóåÉìêçåë=áå=íÜÉ=Ççêë~ä=êççí=Ö~åÖäá~=EÖ~åÖäáçåçé~íÜóFK=póãéíçãë=ÉîçäîÉ=áåÇ~óë=íç=~=ÑÉï=ïÉÉâëK=qÜÉ=éêÉÇçãáå~åí=Åçãéä~áåíë=~í=íÜÉ=çåëÉí=~êÉ=é~áå~åÇ= é~ê~ÉëíÜÉëá~ëI= ïáíÜ= ~å= ~ëóããÉíêáÅ~ä= ÇáëíêáÄìíáçå= íÜ~í= áåîçäîÉë= íÜÉ~êãë=ê~íÜÉê=íÜ~å=íÜÉ=äÉÖëK=kìãÄåÉëëI= äáãÄ=~í~ñá~I=~åÇ=éëÉìÇç~íÜÉíçíáÅãçîÉãÉåíë= çÑ= íÜÉ= Ü~åÇë= ~êÉ= çÑíÉå= çÄëÉêîÉÇK= qÜÉ= íÉåÇçå= êÉÑäÉñÉë= ~êÉäçëí= ~åÇ= ~ää= ãçÇ~äáíáÉë= çÑ= ëÉåë~íáçå= ~êÉ= áåîçäîÉÇ= Äìí= ïáíÜ= ~= ÅäÉ~êéêÉÇçãáå~åÅÉ= çÑ= àçáåí= éçëáíáçå= ~åÇ= îáÄê~íçêó= ëÉåëÉK= qÜÉ= Ñ~ÅÉ= Å~å= ÄÉ~ÑÑÉÅíÉÇ=ÇìÉ=íç=íêáÖÉãáå~ä=Ö~åÖäá~=Ç~ã~ÖÉK=pÉåëçêáåÉìê~ä=ÇÉ~ÑåÉëë=~åÇëÉåëçêó= ÇÉÑáÅáíë= áå= íÜÉ= íêìåâ= ~êÉ= ìåìëì~äK= qÜÉ= íóéáÅ~ä= ëÉåëçêóåÉìêçåçé~íÜó= Ü~ë= ~= ëìÄ~ÅìíÉ= Éîçäìíáçå= ïÜáÅÜ= ëìÄëÉèìÉåíäó= ëí~ÄáäáòÉëK_ó=íÜÉ=íáãÉ=áí=ÇçÉë=ëçI=ÜçïÉîÉêI=íÜÉ=é~íáÉåí=áë=ìëì~ääó=ÅçåÑáåÉÇ=íç=~=ÄÉÇçê=ÅÜ~áê=ÇìÉ=íç=~í~ñá~=SK=få=NMB=çÑ=é~íáÉåíëI=íÜÉ=åÉìêçé~íÜó=êìåë=~=ãáäÇ~åÇ=îÉêó=ëäçï=ÅäáåáÅ~ä=ÉîçäìíáçåK=qÜÉëÉ=é~íáÉåíë=ã~ó=êÉã~áå=~ãÄìä~íçêó~åÇ= áåÇÉéÉåÇÉåí= Ñçê= óÉ~êëK= qÜÉ= ëéÉÅáÑáÅáíó= ~åÇ= ëÉåëáíáîáíó= çÑ= ~åíáJeì~åíáÄçÇáÉë=áë=ÜáÖÜK=låäó=NUB=çÑ=é~íáÉåíë=ïáíÜ=~=é~ê~åÉçéä~ëíáÅ=ëÉåëçêóåÉìêçåçé~íÜó= ïÉêÉ= eìJ^Ä= åÉÖ~íáîÉK= pã~ääJÅÉää= äìåÖ= Å~åÅÉê= ï~ë= íÜÉäÉ~ÇáåÖ= åÉçéä~ëã= áå= íÜÉ= eìJéçëáíáîÉ= ETVBF= ~åÇ= eìJåÉÖ~íáîÉ= EQQBFÖêçìéë= TK= m~íáÉåíë= ïáíÜçìí= ~åíáJeì= ~åíáÄçÇáÉë= ã~ó= Ü~êÄçìê= `sO= çê~ãéÜáéÜóëáå=~åíáÄçÇáÉëK=líÜÉê=é~ê~åÉçéä~ëíáÅ=åÉìêçé~íÜáÉë=áåÅäìÇÉ=äÉëëÅçããçå= ã~åáÑÉëí~íáçåë= ëìÅÜ= ~ë= ëÉåëçêóJãçíçê= åÉìêçé~íÜóI= ~ìíçåçãáÅåÉìêçé~íÜó=~åÇ=ÅÜêçåáÅ=áåíÉëíáå~ä=éëÉìÇçJçÄëíêìÅíáçåK=

Neuropathy in patients treated for malignant disorders

få=~ÇÇáíáçå=íç=äÉëáçåë=çÑ=íÜÉ=mkp=ÇìÉ=íç=áåî~ëáçå=Äó=ã~äáÖå~åí=ÅÉääë=çêíç= é~ê~åÉçéä~ëíáÅ= ëóåÇêçãÉëI= é~íáÉåíë= ïáíÜ= Å~åÅÉê= ~êÉ= ÉñéçëÉÇ= íçÅçãéäáÅ~íáçåë=äáåâÉÇ=íç=ÅÜÉãçíÜÉê~éó=çê=íç=ê~Çá~íáçåK


135


Postradiation neuropathies

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Postchemotherapy peripheral neuropathy

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136


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138


References

NK hóäÉ=oÎ=qÜÉêåÉ~ì=qjI=o~àâìã~ê=psI=Éí=~äK=mêÉî~äÉåÅÉ=çÑ=ãçåçÅäçå~ä=Ö~ããçé~íÜó=çÑ

ìåÇÉíÉêãáåÉÇ=ëáÖåáÑáÅ~åÅÉK=k=båÖä=g=jÉÇ OMMSX=PRQENPFW=NPSOJVK

OK= hóäÉ=oÎ=aìêáÉ=_djI=o~àâìã~ê=psI=Éí=~äK=jçåçÅäçå~ä=Ö~ããçé~íÜó=çÑ=ìåÇÉíÉêãáåÉÇ

ëáÖåáÑáÅ~åÅÉ= EjdrpF= ~åÇ= ëãçäÇÉêáåÖ= E~ëóãéíçã~íáÅF= ãìäíáéäÉ= ãóÉäçã~W= fjtd

ÅçåëÉåëìë=éÉêëéÉÅíáîÉë=êáëâ=Ñ~Åíçêë=Ñçê=éêçÖêÉëëáçå=~åÇ=ÖìáÇÉäáåÉë=Ñçê=ãçåáíçêáåÖ=~åÇ

ã~å~ÖÉãÉåíK=iÉìâÉãá~ OMNMX=OQESFW=NNONJTK

PK aáëéÉåòáÉêá= ^K= mlbjp= ëóåÇêçãÉW= OMNN= ìéÇ~íÉ= çå= Çá~ÖåçëáëI= êáëâJëíê~íáÑáÅ~íáçåI= ~åÇ

ã~å~ÖÉãÉåíK=^ã=g=eÉã~íçä=OMNNX=USW=RVOJSMNK

QK dêáëçäÇ=tI=_êá~åá=Ì=s~ëë=^K=j~äáÖå~åí=ÅÉää=áåÑáäíê~íáçå=áå=íÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉãK

e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=SURJTNOK

RK dáçãÉííç=_I=dêáëçäÇ=tI=sáí~äá~åá=oI=Éí=~äK=m~ê~åÉçéä~ëíáÅ=åÉìêçäçÖáÅ~ä=ëóåÇêçãÉ=áå=íÜÉ

mkp=bìêçåÉíïçêâ=Ç~í~Ä~ëÉW=~=bìêçéÉ~å=ëíìÇó=Ñêçã=OM=ÅÉåíêÉëK ^êÅÜ=kÉìêçä=OMNMX=STW

PPMJRK

SK dê~ìë= cI= hÉáãÉJdìáÄÉêí= cI= oÉåÉ= oI= Éí= ~äK= ^åíáJeì= ~ëëçÅá~íÉÇ= é~ê~åÉçéä~ëíáÅ

ÉåÅÉéÜ~äçãóÉäáíáëW=~å~äóëáë=çÑ=OMM=é~íáÉåíëK=_ê~áå OMMNX=NOQW=NNPUJQUK


139

√ Benign IgM monoclonal gammopathy is often associated with a

demyelinating polyneuropathy.

√ POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy,

monoclonal gammopathy, and skin changes) with increased levels of VEGF,

responds well to treatment of the plasma cell proliferation.

√ Malignant infiltration of the PNS is usually restricted to spinal roots.

√ Small-cell lung cancer is the most common cause of subacute sensory

paraneoplastic neuronopathy and is usually associated with anti-Hu

antibodies.

√ Radiotherapy and chemotherapy for cancer can both induce peripheral nerve

damage, which should be prevented by appropriate monitoring.

Ü Key points


TK jçäáåìÉîç=giI=dê~ìë=cI=pÉêê~åç=Ì=Éí=~äK=ríáäáíó=çÑ=~åíáJeì=~åíáÄçÇáÉë=áå=íÜÉ=Çá~Öåçëáë=çÑ

é~ê~åÉçéä~ëíáÅ=ëÉåëçêó=åÉìêçé~íÜóK=^åå=kÉìêçä=NVVUX=QQW=VTSJUMK

UK j~åàá=eK=aêìÖJáåÇìÅÉÇ=åÉìêçé~íÜáÉëK=e~åÇÄ=`äáå=kÉìêçä OMNPX=NNRW=TOVJQOK=


140


141

Hereditary neuropathies

Chapter 10

Overview

This chapter deals with neuropathies that are genetically transmitted. Predominantly

motor hereditary neuropathy with the classic Charcot-Marie-Tooth disease, now

encompasses several distinct phenotypes and genotypes, axonal or demyelinating,

with a dominant or recessive transmission. Predominantly sensory and autonomic

neuropathies include several clinical and pathological patterns which can be

restricted to the peripheral nervous system, or associated with life-threatening

polysystemic manifestations as in amyloid polyneuropathies and Fabry’s disease.

Treatment of these conditions is outlined. Genetic counselling is mandatory in these

life-threatening hereditary disorders.

Introduction

cçê=~äãçëí=~=ÅÉåíìêó=~ÑíÉê=íÜÉ=ÇÉëÅêáéíáçå=çÑ=íÜÉ=Ççãáå~åíäó=íê~åëãáííÉÇãçíçê=~åÇ=ëÉåëçêó=åÉìêçé~íÜáÉë=ÇÉëÅêáÄÉÇ=Äó=`Ü~êÅçíI=j~êáÉI=~åÇ=qççíÜIëìÄëÉèìÉåíäó= âåçïå= ~ë= `Ü~êÅçíJj~êáÉJqççíÜ= ÇáëÉ~ëÉ= E`jqFI= ~åÇ= íÜÉêÉÅÉëëáîÉI=É~êäóJçåëÉí=åÉìêçé~íÜó=ÇÉëÅêáÄÉÇ=Äó=aÉàÉêáåÉ=~åÇ=pçíí~ëI= íÜÉÑáÉäÇ=êÉã~áåÉÇ=îáêíì~ääó=ìåÅÜ~åÖÉÇ=ìåíáä=íÜÉ=Åä~ëëáÑáÅ~íáçå=çÑ=`jq=ÇáëÉ~ëÉÑçääçïáåÖ=åÉêîÉ=ÅçåÇìÅíáçå=ëíìÇáÉëK=jçêÉ=êÉÅÉåíäóI=éêçÖêÉëë=áå=ãçäÉÅìä~êÖÉåÉíáÅë= Ü~ë= äÉÇ= íç= íÜÉ= ÇáëÅçîÉêó= çÑ= ~= î~êáÉíó= çÑ= ÇáÑÑÉêÉåí= ÜÉêÉÇáí~êóåÉìêçé~íÜáÉëK= _ÉëáÇÉë= íÜÉ= Åä~ëëáÅ~ä= `jq= ÇáëÉ~ëÉI= ïÜáÅÜ= åçïÉåÅçãé~ëëÉë= ëÉîÉê~ä= ÇáëíáåÅí= éÜÉåçíóéÉë= ~åÇ= ÖÉåçíóéÉëI= ÜÉêÉÇáí~êóåÉìêçé~íÜáÉë=áåÅäìÇÉ=~=î~êáÉíó=çÑ=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜáÉëK


^åçíÜÉê= Öêçìé= çÑ= ÜÉêÉÇáí~êó= åÉìêçé~íÜáÉë= ~êÉ= ~ëëçÅá~íÉÇ= ïáíÜ= äáÑÉJíÜêÉ~íÉåáåÖ= éçäóëóëíÉãáÅ= ã~åáÑÉëí~íáçåë= ~ë= áå= ~ãóäçáÇ= éçäóåÉìêçé~íÜáÉëIc~ÄêóÛë= ÇáëÉ~ëÉI= åÉìêçé~íÜáÉë= ~ëëçÅá~íÉÇ= ïáíÜ= ãáíçÅÜçåÇêáçé~íÜáÉëI= íçå~ãÉ=Äìí=~= ÑÉïK=b~ÅÜ=çÑ= íÜÉëÉ=ÇáëÉ~ëÉë=Å~å=ÄÉ=êÉä~íÉÇ= íç=ëÉîÉê~ä=ak^ãìí~íáçåëK=qÜìëI=ak^= íÉëíáåÖ= áë=åçï=~=ã~àçê= ëíÉé= áå= íÜÉ=Çá~Öåçëáë=~åÇã~å~ÖÉãÉåí=çÑ=ÜÉêÉÇáí~êó=åÉìêçé~íÜáÉëI=Äìí=áå=~ää=áåëí~åÅÉë=ak^=íÉëíáåÖãìëí=ÄÉ=çêáÉåíÉÇ=Äó=íÜÉ=ÅäáåáÅ~ä=é~ííÉêå=~åÇ=Ñ~ãáäó=ÜáëíçêóK=qÜÉ=ÅçãéäÉñáíóçÑ= Ñ~ãáäá~ä= éçäóåÉìêçé~íÜó= ã~âÉë= áí= ~Çîáë~ÄäÉ= íç= êÉÑÉê= é~íáÉåíë= ïáíÜëìëéÉÅíÉÇ=çê=ÅçåÑáêãÉÇ=Ñ~ãáäá~ä=åÉìêçé~íÜó=íç=ÉñéÉêí=ÅÉåíêÉë=ïÜÉêÉ=íÜÉóÅ~å=ÖÉí= íÜÉ=ÄÉëí=~ÇîáÅÉK=cáêëí=ïÉ=ïáää=ÅçåëáÇÉê= íÜÉ=`Ü~êÅçíJj~êáÉJqççíÜÖêçìéI=ïÜáÅÜ= áë=ÜáëíçêáÅ~ääó= íÜÉ= Ñáêëí=ÇÉëÅêáÄÉÇI=éêÉÇçãáå~åíäó=ãçíçê=~åÇíóéáÅ~ääó= Å~êêóáåÖ= ~å= ~ìíçëçã~ä= Ççãáå~åí= íê~åëãáëëáçåK= tÜÉêÉ= ïÉ= çåÅÉíÜçìÖÜí= çÑ= `jq= ÇáëÉ~ëÉ= ~ë= ~= ÜçãçÖÉåÉçìë= ÉåíáíóI= íÜÉêÉ= ~êÉ= åçïëçãÉíÜáåÖ= äáâÉ= RM= ÇáÑÑÉêÉåí= ãìí~íáçåë= ïÜáÅÜ= Å~å= áåÇìÅÉ= íÜÉ= ë~ãÉéÜÉåçíóéÉI=ïáíÜ=ëçãÉ=î~êá~åíëK

Hereditary neuropathies without polysystemic manifestations

Predominantly motor hereditary polyneuropathies

qïç= ã~áå= é~ííÉêåë= Ü~îÉ= ÄÉÉå= çêáÖáå~ääó= áÇÉåíáÑáÉÇW= íÜÉ= `jq= é~ííÉêåIïÜáÅÜ= áë= íÜÉ= ãçëí= ÅçããçåI= áë= ÄÉåáÖå= ~åÇ= Ççãáå~åíäó= íê~åëãáííÉÇK= qÜÉçíÜÉê= çåÉ= áë= ~å= É~êäóJçåëÉíI= êÉÅÉëëáîÉäó= íê~åëãáííÉÇ= éÜÉåçíóéÉI= ïÜáÅÜÅçêêÉëéçåÇë=íç=aÉàÉêáåÉJpçíí~ë=ÇáëÉ~ëÉK

Late onset and dominant transmission

The CharcotMarieTooth neuropathies N

qÜÉ= íóéáÅ~ä= `jq= éÜÉåçíóéÉ= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= çåëÉí= áå= íÜÉ= ÑáêëíÇÉÅ~ÇÉë= çÑ= äáÑÉI= çÑ= ëäçïäó= éêçÖêÉëëáîÉ= Äáä~íÉê~ä= ãìëÅäÉ= ï~ëíáåÖ= ~åÇïÉ~âåÉëë=ëí~êíáåÖ=ïáíÜ=íÜÉ=ÑÉÉí=~åÇ=äÉÖë=~åÇ=íÜÉå=Öê~Çì~ääó=~ëÅÉåÇáåÖ=íçíÜÉ=Çáëí~ä=íÜáêÇ=çÑ=íÜÉ=íÜáÖÜ=~åÇ=Ü~åÇëK=pÉåëçêó=äçëë=áë=äÉëë=éêçãáåÉåíI=~åÇé~áåë= ~êÉ= ìëì~ääó= ãáäÇ= çê= ~ÄëÉåíK= pÉåëçêó= ÅÜ~åÖÉë= Ñçääçï= íÜÉ= ë~ãÉÇáëíêáÄìíáçå=~ë=ãçíçê=ÅÜ~åÖÉëK=pâÉäÉí~ä=Ñççí=ÇÉÑçêãáíáÉë=~êÉ=îÉêó=ÅçããçåWéÉë=Å~îìë=ïáíÜ=ÜáÖÜ=~êÅÜÉë=~åÇ=Ü~ããÉê=íçÉë= áë=ÑçìåÇ= áå=íÜÉ=ã~àçêáíó=çÑ~ÑÑÉÅíÉÇ=ëìÄàÉÅíë=ëáåÅÉ=ÅÜáäÇÜççÇ=EcáÖìêÉ=NFK=pÅçäáçëáë= áë=éêÉëÉåí= áå=çåÉ


142


íÜáêÇ= çÑ= é~íáÉåíëK= réçå= Éñ~ãáå~íáçåI= ãçíçê= ÇÉÑáÅáíI= ïÜáÅÜ= ëí~êíë= ~åÇéêÉÇçãáå~íÉë= áå= íÜÉ=Çáëí~ä= ÑççíI= áë=ãáäÇ=~åÇ=éêçÖêÉëëÉë=îÉêó=ëäçïäóI=çîÉêÇÉÅ~ÇÉëK= jçëí= é~íáÉåíë= êÉã~áå= ~ÄäÉ= íç= ï~äâI= ~äíÜçìÖÜ= ïáíÜ= áåÅêÉ~ëáåÖÇáÑÑáÅìäíó= ~åÇ= íÜÉó= çÑíÉå= åÉÉÇ= ëéÉÅá~ä= ëÜçÉëK= aÉÉé= íÉåÇçå= êÉÑäÉñÉë= ~êÉ~ÄëÉåí=Ñêçã=íÜÉ=çåëÉíK=`jq=ÇáëÉ~ëÉI=ÜçïÉîÉêI=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=~=ÜáÖÜî~êá~Äáäáíó= çÑ= ÇáëÉ~ëÉ= ëÉîÉêáíó= áåÅäìÇáåÖ= ïáíÜáå= íÜÉ= ë~ãÉ= Ñ~ãáäóK= få= ëçãÉãÉãÄÉêë= íÜÉ=ÉñéêÉëëáçå=çÑ= íÜÉ=ÇáëÉ~ëÉ= áë= äáãáíÉÇ= íç=éÉë=Å~îìëI=ïÜáäÉ= áåçíÜÉêë= Ñççí= ÇÉÑçêãáíó= áë= ~ëëçÅá~íÉÇ= ïáíÜ= Ñççí= Çêçé= ~ÑíÉê= ~= ÑÉï= ÇÉÅ~ÇÉëKpçãÉ=~ÑÑÉÅíÉÇ=é~íáÉåíë=ã~ó=ÄÉ=ÅçåÑáåÉÇ=íç=~=ïÜÉÉäÅÜ~áêI=ïáíÜ=ëÉîÉêÉ=äçëëçÑ=Ü~åÇ=ãçîÉãÉåíëK=

qÜÉ= ëìÄÇáîáëáçå= áåíç= íïç= ã~áå= íóéÉë= ~ÅÅçêÇáåÖ= íç= åÉêîÉ= ÅçåÇìÅíáçåëíìÇáÉëI=ï~ë=~=ÅçêåÉêëíçåÉ=áå=íÜÉ=`jq=åçëçÖê~éÜó=~åÇ=áë=ëíáää=î~äáÇ=áå=íÜÉã~àçêáíó= çÑ= Å~ëÉëK= `jqN= ÇÉëáÖå~íÉë= íÜÉ= ÇÉãóÉäáå~íáåÖ= î~êáÉíó= ~åÇ= áëÅÜ~ê~ÅíÉêáëÉÇ= Äó= ÇáÑÑìëÉ= ëäçïáåÖ= çÑ= åÉêîÉ= ÅçåÇìÅíáçå= îÉäçÅáíáÉë= Ek`sFIïáíÜ=î~äìÉë=Äó=ÅçåîÉåíáçå=ÄÉäçï=íÜÉ=äáãáí=çÑ=PUãLëÉÅçåÇ=Ñçê=ãçíçê=åÉêîÉëáå=íÜÉ=ìééÉê=äáãÄëK=`jqO=áë=íÜÉ=~ñçå~ä=íóéÉI=áå=ïÜáÅÜ=k`së=~êÉ=éêÉëÉêîÉÇIçê= çåäó= ãáäÇäó= ëäçïÉÇI= Äó= ÇÉÑáåáíáçå= ~ÄçîÉ= íÜÉ= î~äìÉ= çÑ= PUãLëÉÅçåÇ= áåìééÉê=äáãÄ=ãçíçê=åÉêîÉëK=

Chapter 10 Hereditary neuropathies

143

Figure 1. Pes cavus and leg amyotrophy in a patient with a

Charcot-Marie-Tooth hereditary neuropathy and PMP22

duplication.


táíÜáå= íÜÉ= `jq= éÜÉåçíóéÉI= ak^= íÉëíáåÖ= Ü~ë= áÇÉåíáÑáÉÇ= ëçãÉëìÄÅä~ëëÉë=~ë=çìíäáåÉÇ=ÄÉäçïK

CMT1`jqN^=áë=êÉä~íÉÇ=íç=ÇìéäáÅ~íáçå=çÑ=íÜÉ=NTé=éÉêáéÜÉê~ä=ãóÉäáå=éêçíÉáå=OO

EmjmOOFI=çê=äÉëë=çÑíÉå=íç=mjmOO éçáåí=ãìí~íáçåK=`jqN^=~ÅÅçìåíë=Ñçê=SMJVMB=çÑ=`jqN=é~íáÉåíë=EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çîÉêäÉ~ÑFK=få=`jqN^=é~íáÉåíëIåÉêîÉ= Äáçéëó= ëÜçïë= ÉñíÉåëáîÉ= ÇÉãóÉäáå~íáçå= çÑ= åÉêîÉ= ÑáÄêÉë= ~ëëçÅá~íÉÇ


144

Figure 2. Cross-sections of a nerve biopsy specimen to show

Schwann cell proliferation forming onion bulbs common in the

hypertrophic type of demyelinating CMT disease. (Bar 10μm.)


ïáíÜ=ëéÉÅí~Åìä~ê=éêçäáÑÉê~íáçå=çÑ=pÅÜï~åå=ÅÉääë=ïÜáÅÜ=ã~ó=áåÇìÅÉ=é~äé~ÄäÉåÉêîÉ=ÜóéÉêíêçéÜó=EcáÖìêÉ=OFK


145

Charcot-Marie-Tooth disease type 1

Charles is a 53-year-old physiotherapist who consulted because of walking difficulty.

The patient was in good general condition and worked in a rehabilitation centre in a

university hospital. He first noticed some difficulty in walking 2 to 3 years previously,

in that he would become fatigued after walking for an hour or two. This fatigue

gradually increased over the subsequent years to the point where he had to rest after

walking for half an hour. Upon examination he had bilateral pes cavus and hammer

toes, and said that he always had problems to find convenient shoes. His legs were

slightly atrophic distally. He had no pain. He could not stand on the heels on either

side. The strength of foot dorsiflexion was decreased to 4/5 and plantar flexion was

normal. Muscle strength was normal in other territories. Perception of light touch

was abolished up to the mid-leg, but pinprick and temperature sensations were

preserved. Position sense of the great toe was decreased on both sides. Ankle and

patellar reflexes were abolished.

With regard to family history, the patient had a 48-year-old sister who had no walking

difficulties. The patient’s mother, who was 82 years old, had marked walking

difficulty which had necessitated the use of a walking cane since the age of 75. Her

younger sister who was 67 years old had bilateral pes cavus but no walking difficulty.

An electromyography (EMG) showed a reduced nerve conduction velocity in the

upper and lower limbs. Peroneal nerve conduction was reduced to 24m/s on the

right side; 22m/s on the left side with an important decrease of the compound

muscle action potentials. In the upper limbs, the median nerve conduction velocity

was 28m/s on both sides. DNA testing for dominant demyelinating Charcot-Marie-

Tooth disease confirmed that the patient had duplication of the 17p peripheral

myelin protein 22 gene.

This patient had a relatively mild expression of the disease. This mutation had a

variable expression amongst his relatives. The patient’s aunt only had pes cavus and

the patient’s mother was still able to walk.


`jqN_= áë= êÉä~íÉÇ= íç= ãìí~íáçå= çÑ= íÜÉ= ãóÉäáå= m= òÉêç= éêçíÉáå= EjmwFÖÉåÉK= `jqN_= ~ÅÅçìåíë= Ñçê= ~ééêçñáã~íÉäó= NMB= çÑ= `jqNK= qÜÉéÜÉåçíóéÉ=çÑ=é~íáÉåíë=ïáíÜ=jmw ãìí~íáçåë= áë=ìëì~ääó=çåÉ=çÑ= íïç=íóéÉëI~å=É~êäóJçåëÉí=åÉìêçé~íÜó=ïáíÜ=ëäçï=ãçíçê=k`sI=ïÜáÅÜ=Å~å=ÄÉ=ëÉîÉêÉ=Ô~ë=áå=íÜÉ=aÉàÉêáåÉJpçíí~ë=ëóåÇêçãÉ=Ô=çê=~å=~ÇìäíJçåëÉíI=ãáäÇÉê=~ñçå~ä=çêáåíÉêãÉÇá~íÉ= åÉìêçé~íÜóK= `jqN= Ì= aI= b= ~åÇ= c= ~êÉ= ê~êÉ= ÇÉãóÉäáå~íáåÖ`jq=íóéÉëI=~ÅÅçìåíáåÖ=Ñçê=äÉëë=íÜ~å=NB=çÑ=`jq=ÇáëÉ~ëÉK

CMT2`jqO=Ü~ë=íÜÉ=ë~ãÉ=ÅäáåáÅ~ä=éêÉëÉåí~íáçå=~åÇ=íê~åëãáëëáçå=~ë=`jqNI

Äìí= åÉêîÉ= ÅçåÇìÅíáçå= îÉäçÅáíó= áë= áå= íÜÉ= ~ñçå~ä= é~ííÉêåI= áå= âÉÉéáåÖ= ïáíÜåÉêîÉ=é~íÜçäçÖóK=qÜÉ=ãçëí=Åçããçå=íóéÉ=áë=`jqO^=êÉä~íÉÇ=íç=ãáíçÑìëáåO= ãìí~íáçå= EjckOFENéPSKO= jckOFK= fí= ~ÅÅçìåíë= Ñçê= ìé= íç= OMB= çÑ= íÜÉ


146

Figure 3. Consecutive segments of an isolated nerve fibre

from a patient with an hereditary liability to pressure palsy,

showing the thickness variation of the myelin sheath with a

‘sausage’ formation.


`jqO=ÖêçìéI=ïÜáÅÜ=ã~ó=ÄÉ=ÉáíÜÉê=ëÉîÉêÉ=ïáíÜ=~å=É~êäó=çåëÉí=çê=ãáäÇ=íçãçÇÉê~íÉ=ïáíÜ=~=ä~íÉê=çåëÉíK=qÜÉêÉ=~êÉ=ÑêÉèìÉåí=ÇÉ=åçîç ãìí~íáçåëK=qÜÉ`jqO_=íç=m=íóéÉë=~êÉ=ãìÅÜ=ê~êÉê=~åÇ=êÉä~íÉÇ=íç=~=î~êáÉíó=çÑ=ãìí~íáçåëK

CMTX1qÜÉ= uJäáåâÉÇ= Ççãáå~åí= î~êáÉíó= çÑ= `jq= ÇáëÉ~ëÉI= `jquNI= áë

ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ÅäáåáÅ~ä= ~åÇ= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= áåîçäîÉãÉåí= ãçêÉëÉîÉêÉ= áå= ã~äÉëI= ïÜç= çÑíÉå= Ü~îÉ= k`s= áå= íÜÉ= áåíÉêãÉÇá~íÉ= ê~åÖÉI= íÜ~åÑÉã~äÉëI=ïÜçëÉ=k`s=~êÉ=ìëì~ääó=áå=íÜÉ=`jqO=ê~åÖÉK=

Hereditary neuropathy with a liability to pressure palsy (HNPP)ekmm= íóéáÅ~ääó=éêÉëÉåíë=ïáíÜ= êÉÅìêêÉåí= íê~åëáÉåí= ÑçÅ~ä=ãçíçê= ~åÇLçê

ëÉåëçêó= ÇÉÑáÅáíë= áå= íÜÉ= ÇáëíêáÄìíáçå= çÑ= áåÇáîáÇì~ä= åÉêîÉë= çê= éäÉñìëÉëIìëì~ääó= éêÉÅáéáí~íÉÇ= Äó= éêÉëëìêÉK= qÜÉêÉ= áë= ëäçïáåÖ= ~í= íóéáÅ~ä= ëáíÉë= çÑÅçãéêÉëëáçåK= ^ää= é~íáÉåíë= ïáíÜ= ekmm= Ü~îÉ= ïáÇÉëéêÉ~ÇåÉìêçéÜóëáçäçÖáÅ~ä=~Äåçêã~äáíáÉë=ÉîÉå=áÑ=íÜÉáê=ëóãéíçãë=~êÉ=ÅçåÑáåÉÇ=íç~=ëáåÖäÉ=åÉêîÉ=OK=ekmm=áë=ìëì~ääó=Å~ìëÉÇ=Äó=ÇÉäÉíáçå=çÑ=íÜÉ=ë~ãÉ=NKQ=jÄêÉÖáçå= çÑ= ÅÜêçãçëçãÉ= NT= íÜ~í= áë= ÇìéäáÅ~íÉÇ= áå= `jqN^K= kÉêîÉ= ÄáçéëóëÜçïë= ÅÜ~ê~ÅíÉêáëíáÅ= ëïÉääáåÖ= çÑ= íÜÉ= ãóÉäáå= ëÜÉ~íÜ= Å~ääÉÇ= íçã~Åìä~EcáÖìêÉ=PFK

Recessive transmission of early-onset CMT neuropathies and theDejerine-Sottas phenotype^ìíçëçã~ä= êÉÅÉëëáîÉ= Å~ëÉë= ~êÉ= ê~êÉ= áå= bìêçéÉI= ~ÅÅçìåíáåÖ= Ñçê= äÉëë

íÜ~å= NMB= çÑ= `jq= é~íáÉåíëK= `çåîÉêëÉäóI= íÜáë= áåÜÉêáí~åÅÉ= é~ííÉêå= áëÑêÉèìÉåí=áå=éçéìä~íáçåë=ïáíÜ=~=ÜáÖÜ=ê~íÉ=çÑ=Åçåë~åÖìáåÉçìë=ã~êêá~ÖÉë=~ëáå= kçêíÜ= ^ÑêáÅ~= ~åÇ= qìêâÉó= PK= aÉàÉêáåÉJpçíí~ë= ëóåÇêçãÉ= ëéäáíë= áåíç= íÜÉ~ìíçëçã~ä= êÉÅÉëëáîÉ= `jqQ= éÜÉåçíóéÉëK= qÜÉ= êÉÅÉëëáîÉäó= íê~åëãáííÉÇ`jq=ëóåÇêçãÉë=Ü~îÉ=áå=Åçããçå=~å=É~êäó=çåëÉíI=ïáíÜáå=íÜÉ=Ñáêëí=óÉ~êë=çÑäáÑÉI=ÇÉä~óÉÇ=ÇÉîÉäçéãÉåí~ä=ãáäÉëíçåÉë=~åÇ=~=ëÉîÉêÉ=çìíÅçãÉK

Recessive demyelinating neuropathy — CMT4A`jqQ^=çåëÉí=áë=áå=íÜÉ=Ñáêëí=ÇÉÅ~ÇÉ=çÑ=äáÑÉ=~åÇ=íÜÉ=éêçÖêÉëëáçå=áë=ê~éáÇ

~åÇ=ëÉîÉêÉK=`ä~ëëáÅ~ä=ÑÉ~íìêÉë=çÑ=`jq=ÇáëÉ~ëÉI=äáâÉ=Çáëí~ä=ïÉ~âåÉëë=~åÇï~ëíáåÖI= êÉÇìÅÉÇ= çê= ~ÄçäáëÜÉÇ= êÉÑäÉñÉëI= ~åÇ= éÉë= Å~îìë= ~êÉ= çÑíÉå~ÅÅçãé~åáÉÇ= Äó= éêçñáã~ä= áåîçäîÉãÉåí= ~åÇ= îÉêó= É~êäó= é~ê~äóëáë= çÑ= íÜÉ


147


Ü~åÇëK= m~íáÉåíë= ~êÉ= ìëì~ääó= ïÜÉÉäÅÜ~áêJÄçìåÇ= ä~íÉ= áå= íÜÉ= ÇáëÉ~ëÉéêçÖêÉëëáçåI= óÉí= íÜÉ= ëÉîÉêáíó= çÑ= íÜÉ= ÇáëÉ~ëÉ= ã~ó= î~êó= ~ãçåÖ= ~ÑÑÉÅíÉÇáåÇáîáÇì~äë=áå=íÜÉ=ë~ãÉ=Ñ~ãáäó=ïáíÜ=íÜÉ=ë~ãÉ=ãìí~íáçåK=péÉÅá~ä=~ííÉåíáçåãìëí=ÄÉ=é~áÇ=íç=íÜÉëÉ=é~íáÉåíë=ïáíÜ=áåîÉëíáÖ~íáçå=çÑ=êÉëéáê~íçêó=ÑìåÅíáçåëáåÅÉ= áí= áë= äáÑÉJíÜêÉ~íÉåáåÖK= `jqQ^= áë= ~ëëçÅá~íÉÇ= ïáíÜ= ~= î~êáÉíó= çÑãìí~íáçåë=áå=íÜÉ=da^mN ÖÉåÉK

CMT4B1qÜÉ=çåëÉí=áë=ìëì~ääó=ÄÉÑçêÉ=íÜÉ=~ÖÉ=çÑ=Q=óÉ~êëK=tÉ~âåÉëë=ÇáëíêáÄìíáçå=áë

ëáãáä~ê=íç=íÜÉ=Åä~ëëáÅ~ä=`jq=éÜÉåçíóéÉX=áí=ëí~êíë=Çáëí~ääó=áå=íÜÉ=äçïÉê=äáãÄëIíÜÉå= ~ÑÑÉÅíë= íÜÉ= ìééÉê= äáãÄë= éêçÖêÉëëáåÖ= íç= áåîçäîÉ= éêçñáã~ä= ãìëÅäÉëKaÉÉé= íÉåÇçå=êÉÑäÉñÉë=~êÉ=ìëì~ääó=~ÄëÉåí=ïáíÜ=Çáëí~ä=ëÉåëçêó= äçëëK=^=éÉëÉèìáåçî~êìë=ÇÉÑçêãáíó= áë= êÉéçêíÉÇ= áå=ëçãÉ=é~íáÉåíëK=m~íáÉåíë=~êÉ=ìëì~ääóïÜÉÉäÅÜ~áêJÄçìåÇ=~í=~å=É~êäó=~ÖÉK=`ê~åá~ä=åÉêîÉ=áåîçäîÉãÉåí=áë=êÉéçêíÉÇK`~ìë~íáîÉ= ãìí~íáçå= çÅÅìêë= áå= íÜÉ= ãóçíìÄìä~êáåJêÉä~íÉÇ= éêçíÉáå= O= ÖÉåÉEjqjoOFK= qÜÉ= `jqQ`= íç= `jqQg= íóéÉë= ~êÉ= ê~êÉI= ïáíÜ= ~å= É~êäó= çê= îÉêóÉ~êäó=çåëÉíI=çê=~í=ÄáêíÜK=qÜÉëÉ=~êÉ=ÇÉãóÉäáå~íáåÖ=éçäóåÉìêçé~íÜáÉë=ïáíÜ=~êÉÅÉëëáîÉ=íê~åëãáëëáçåI=êÉä~íÉÇ=íç=~=î~êáÉíó=çÑ=ê~êÉ=ãìí~íáçåëI=ëçãÉíáãÉë~ÑÑÉÅíáåÖ=~=ëáåÖäÉ=Ñ~ãáäóK

qç=ëìãã~êáëÉ=íÜÉ=ÖÉåÉíáÅ=ÑáåÇáåÖë=áå=é~íáÉåíë=ïáíÜ=`jq=ÇáëÉ~ëÉI=áí=áë=çÑáåíÉêÉëí= íç=èìçíÉ=íÜÉ=ÑáåÇáåÖë=çÑ=~å= áãéçêí~åí=ëíìÇó=çÑ=QOR=é~íáÉåíëK=qÜÉëíìÇó=ÅçåëáëíÉÇ=çÑ=OQM=é~íáÉåíë=ïáíÜ=`jqN=ERSBFI=NNR=ïáíÜ=`jqO=EOTBF~åÇ= SO= ïáíÜ= `jq= ÇáëÉ~ëÉ= ~ëëçÅá~íÉÇ= ïáíÜ= áåíÉêãÉÇá~íÉäó= ëäçïÉÇ= åÉêîÉÅçåÇìÅíáçå= Ef`jqFK= káåÉíóJíïç= éÉêÅÉåí= çÑ= íÜçëÉ= é~íáÉåíë= ïáíÜ= `jqÇáëÉ~ëÉ= ~åÇ= ~= ÖÉåÉíáÅ= Çá~Öåçëáë= Ü~Ç= ÉáíÜÉê= ~= ÇìéäáÅ~íáçå= çÑ= mjmOOE`jqN^F=çê=ãìí~íáçåë=áå=íÜêÉÉ=çíÜÉê=ÖÉåÉëX=jmw E`jqN_FI=Ö~é=àìåÅíáçåÄÉí~JN=Edg_N Ô=`jqNuF=çê=jckO E`jqO^FK=fÑ=åç=ãìí~íáçå=ï~ë=ÇÉíÉÅíÉÇïáíÜ= íÜÉëÉ= Ñçìê= ÖÉåÉëI= íÜÉêÉ= ï~ë= äÉëë= íÜ~å= ~= PB= ÅÜ~åÅÉ= çÑ= ã~âáåÖ= ~ãçäÉÅìä~ê= Çá~ÖåçëáëK= qÜáë= ï~ë= íêìÉ= çÑ= ÅçìêëÉ= çåäó= Ñçê= é~íáÉåíë= ïáíÜ~ìíçëçã~ä= Ççãáå~åí= çê= uJäáåâÉÇ= `jq= ÇáëÉ~ëÉI= ~äíÜçìÖÜ= ~ë= íÜÉ= ~ìíÜçêëéçáåí=çìíI=ã~åó=çÑ=íÜÉëÉ=é~íáÉåíë=ã~ó=éêÉëÉåí=ïáíÜçìí=~=Ñ~ãáäó=Üáëíçêó=QI=RK

Hereditary sensory and autonomic neuropathies

qÜáë=Öêçìé=çÑ=ÜÉêÉÇáí~êó=åÉìêçé~íÜáÉë=ÉåÅçãé~ëëÉë=~=î~êáÉíó=çÑ=éìêÉäóçê= éêÉÇçãáå~åíäó= ëÉåëçêó= éçäóåÉìêçé~íÜáÉë= íÜ~í= ã~ó= çê= ã~ó= åçí= ÄÉ


148


~ëëçÅá~íÉÇ=ïáíÜ=ã~åáÑÉëí~íáçåë=çÑ=Çóë~ìíçåçãá~K=få=íÜáë=Öêçìé=íççI=ëçãÉåÉìêçé~íÜáÉë=~êÉ=íê~åëãáííÉÇ=~ë=~å=~ìíçëçã~ä=íê~áíI=çÑíÉå=ïáíÜ=~=ä~íÉ=çåëÉíIïÜáäÉ= ~í= íÜÉ= çíÜÉê= ÉñíêÉãáíó= çÑ= íÜÉ= ëéÉÅíêìãI= ëçãÉ= åÉìêçé~íÜáÉë= ~êÉëóãéíçã~íáÅ=~ë=É~êäó=~ë=ÇìêáåÖ=íÜÉ=Ñáêëí=Ç~óë=çÑ=äáÑÉK=

Hereditary sensory and autonomic neuropathy type I

eÉêÉÇáí~êó=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜó=íóéÉ=f= Eepk=fLep^k=fFÇáëÉ~ëÉ= áë= ~å= ~ìíçëçã~ä= Ççãáå~åí= ÖÉåÉíáÅ= ëìÄíóéÉK= fíë= çåëÉí= áë= ìëì~ääóÄÉíïÉÉå=íÜÉ=ëÉÅçåÇ=~åÇ=ÑçìêíÜ=ÇÉÅ~ÇÉ=çÑ=äáÑÉ=çê=ÉîÉå=ä~íÉê=ïÜÉå=é~íáÉåíëÑáêëí=åçíáÅÉ=ã~êâÉÇ=Çáëí~ä=ëÉåëçêó=áãé~áêãÉåí=áå=íÜÉ=äçïÉê=Äìí=ëçãÉíáãÉë~äëç= íÜÉ= ìééÉê= äáãÄëK= s~êá~ÄäÉ= Çáëí~ä= ãçíçê= ~åÇ= ãáåáã~ä= ~ìíçåçãáÅáåîçäîÉãÉåí= áë= ÑêÉèìÉåíäó=çÄëÉêîÉÇK=qÜÉ=åÉìêçäçÖáÅ~ä=éÜÉåçíóéÉ= áë=çÑíÉåÅçãéäáÅ~íÉÇ=Äó=é~áåäÉëë= áåàìêáÉë=ïÜáÅÜ= äÉ~Ç=íç=ëÉîÉêÉ= áåÑÉÅíáçå= áåÅäìÇáåÖÇÉÉé=Ñççí=ìäÅÉê~íáçåI=çëíÉçãóÉäáíáë=~åÇ=çëíÉçåÉÅêçëáëI=~åÇ=~Åêçãìíáä~íáçåëíÜ~í= ã~ó= åÉÅÉëëáí~íÉ= íçÉ= ~åÇ= äáãÄ= ~ãéìí~íáçåK= péçåí~åÉçìë= ä~åÅáå~íáåÖé~áåë= ~êÉ= ëçãÉíáãÉë= êÉéçêíÉÇK= pç= Ñ~êI= ÑáîÉ= ÖÉåÉë= Epmqi`NI= pmqiÒIo^_TÎ=^qiNI=~åÇ=akjqNF=Ü~îÉ=ÄÉÉå=áãéäáÅ~íÉÇ=áå=íÜÉ=é~íÜçÖÉåÉëáë=çÑ


149

Figure 4. Cross-section of a nerve specimen from a patient

with type 1 hereditary sensory and autonomic neuropathy.

Light microscopy shows the nearly complete disappearance of

myelinated fibres. (Bar 10μm.)


epk=fK=bäÉÅíêçãóçÖê~éÜó=EbjdF=ÑáåÇáåÖë=~êÉ=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=ÇÉÅêÉ~ëÉÇçê= ~ÄëÉåí= ëÉåëçêó= ~Åíáçå= éçíÉåíá~äë= ~åÇ= êÉä~íáîÉ= éêÉëÉêî~íáçå= çÑ= åÉêîÉÅçåÇìÅíáçå= îÉäçÅáíóK= cêçã= ~= é~íÜçäçÖáÅ~ä= éçáåí= çÑ= îáÉï= íÜáë= ëìÄíóéÉ= áëÅÜ~ê~ÅíÉêáëÉÇ= Äó= íÜÉ= Öê~Çì~ä= Çáë~ééÉ~ê~åÅÉ= çÑ= ãóÉäáå~íÉÇ= ~åÇìåãóÉäáå~íÉÇ=åÉêîÉ=ÑáÄêÉë=EcáÖìêÉë=Q=~åÇ=RFK=qÜÉ=ã~áå=ÅçãéäáÅ~íáçå=çÑ=íÜáëëìÄíóéÉ=áë=íÜÉ=çÅÅìêêÉåÅÉ=çÑ=Çáëí~ä=íêçéÜáÅ=ÅÜ~åÅÉë=êÉëìäíáåÖ=Ñêçã=é~áåäÉëëíê~ìã~K= mÜÉåçíóéáÅ= î~êá~Äáäáíó= ïáíÜáå= ~= Ñ~ãáäó= î~êáÉë= ïáÇÉäó= ~åÇI= ~äëçIëìÄÅäáåáÅ~ääó=~ÑÑÉÅíÉÇ=ãìí~íáçå=Å~êêáÉêë=Ü~îÉ=ÄÉÉå=ÇÉëÅêáÄÉÇ=SK=

Hereditary sensory and autonomic neuropathy type II

eÉêÉÇáí~êó=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜó=íóéÉ=ff=Eepk=ffLep^k=ffFáë= ~å= ~ìíçëçã~ä= êÉÅÉëëáîÉäó= áåÜÉêáíÉÇ= Ñçêã= çÑ= epk= EÜ ëÉÉ= Å~ëÉ= ëíìÇóçîÉêäÉ~ÑFK= ^í= ÇáëÉ~ëÉ= çåëÉíI= é~íáÉåíë= ìëì~ääó= ëÜçï= ëáÖåë= ~åÇ= ëóãéíçãëÅçåëáëíáåÖ=çÑ=Çáëí~ä=åìãÄåÉëë= áå= íÜÉ=ìééÉê=~åÇ= äçïÉê= äáãÄë=~åÇ=~=ÖäçîÉ~åÇ= ëíçÅâáåÖJäáâÉ= ëÉåëçêó= äçëë= áå= áåÑ~åÅó= çê= É~êäó= ÅÜáäÇÜççÇK= i~íÉê= çåIé~íáÉåíë= ÇÉîÉäçé= áãé~áêãÉåí= çÑ= é~áåI= íÉãéÉê~íìêÉ= ~åÇ= íçìÅÜ= ëÉåë~íáçåI


150

Figure 5. Electron micrograph of the same nerve specimen to

show a spectacular reduction of the number of unmyelinated

fibres. The surviving unmyelinated fibres are indicated by

arrows. (Bar 1μm.)


áåîçäîáåÖ=íÜÉ=íêìåâ=áå=ëçãÉ=é~íáÉåíë=~ë=ïÉääK=^ìíçåçãáÅ=ÇóëÑìåÅíáçå=ã~ó=ÄÉãáåáã~ä= ~åÇ= Å~å= áåÅäìÇÉ= ÜóéÉêÜáÇêçëáëI= íçåáÅ= éìéáäëI= ~åÇ= ìêáå~êóáåÅçåíáåÉåÅÉ=áå=Å~ëÉë=ïáíÜ=ãçêÉ=~Çî~åÅÉÇ=ÇáëÉ~ëÉK=^í=éêÉëÉåíI=ãìí~íáçåëáå= íÜêÉÉ= ÖÉåÉë= EepkOLtkhNI= c^jNPQ_ ~åÇ= hfcN^F= Ü~îÉ= ÄÉÉå~ëëçÅá~íÉÇ=ïáíÜ=epk=ff=éÜÉåçíóéÉëK


151

Hereditary sensory neuropathy type II

Nelly is a 29-year-old woman who consulted for chronic sensory loss with recurrent

corneal ulcers, multiple painless trauma, acral mutilation and painless burns during

infancy and childhood. She was born to non-consanguineous parents. She had one

older brother and one younger sister, none of whom were affected. She was married

and a mother of a 3-year-old daughter who did not seem affected.

The patient was hypotrophic measuring 148cm and weighed 35kg. She had a

scoliosis and delayed development milestones. She could not walk unaided until the

age of 7 years. She had no symptoms of dysautonomia. She was an intelligent

person. She had an ataxic gait, with normal motor strength. Sensory examination

revealed a universal loss of pain and temperature sensation. She could not identify

materials by palpation. Proprioception was abolished, including position sense,

vibratory sensation and astereognosia. She had a sensory ataxia with Romberg’s sign.

All tendon reflexes were abolished. Electrophysiological testing showed normal

motor action potentials and conduction velocity. The sensory action potentials were

undetectable. A biopsy of the left sural nerve was performed which showed small

nerve fascicles and nearly a complete absence of myelinated fibres. Conversely, the

unmyelinated fibres were preserved (Figure 6).

This patient had a purely sensory polyneuropathy of early onset with dysmorphic

changes, without autonomic dysfunction. This is a recessive autonomic hereditary

sensory neuropathy type II associated with mutation in the WNK1 gene, which was

later confirmed by DNA testing. The patient was followed for several years without a

significant change in her neurological condition.


Hereditary sensory and autonomic neuropathy type III — theRileyDay syndrome

eÉêÉÇáí~êó=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜó=íóéÉ=fff=Eepk=fffLep^kfffF= áë= ~å= ~ìíçëçã~ä= êÉÅÉëëáîÉ= ÇÉîÉäçéãÉåí~ä= ÇáëçêÇÉê= ~ÑÑÉÅíáåÖ= ëã~ääãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇ=åÉìêçåë=êÉëìäíáåÖ=áå=ëÉåëçêó=~åÇ=~ìíçåçãáÅÇóëÑìåÅíáçåK=póãéíçãë=~êÉ=éêÉëÉåí=Ñêçã=ÄáêíÜ=ïáíÜ=íÜÉ=É~êäáÉëí=ëáÖåë=ÄÉáåÖéççê= ëìÅâ= ~åÇ= Üóéçíçåá~K= `äáåáÅ~ä= Çá~ÖåçëíáÅ= ÅêáíÉêá~= áåÅäìÇÉ= ~ÄëÉåíä~Åêáã~íáçåI= ~ÄëÉåí= ÇÉÉé= íÉåÇçå= êÉÑäÉñÉëI= ~åÇ= ~ÄëÉåí= äáåÖì~ä= ÑìåÖáÑçêãé~éáää~ÉK= ^ìíçåçãáÅ= ÇóëÑìåÅíáçå= êÉëìäíë= áå= ~ÄëÉåí= Éãçíáçå~ä= íÉ~êëIçêçãçíçê= áåÅççêÇáå~íáçåI= ~åÇ= Å~êÇáçî~ëÅìä~ê= ä~Äáäáíó= ïáíÜ= éçëíìê~äÜóéçíÉåëáçå= ~åÇ= ÉéáëçÇáÅ= ÜóéÉêíÉåëáçåK= m~íáÉåíë= ~êÉ= ~äëç= éêçåÉ= íçéÉêáçÇáÅ= îçãáíáåÖ= ÅêáëÉë= ÅçãéêáëáåÖ= å~ìëÉ~I= ÜóéÉêë~äáî~íáçåIÄêçåÅÜçêêÜÉ~I=ÜóéÉêíÉåëáçåI= í~ÅÜóÅ~êÇá~I= ~åÇ=ÉêóíÜÉã~íçìë=ÄäçíÅÜáåÖ=çÑíÜÉ=ëâáåK=c~ãáäá~ä=Çóë~ìíçåçãá~=áë=Å~ìëÉÇ=Äó=ãìí~íáçåë=áå=íÜÉ=áåÜáÄáíçê=çÑâ~éé~= äáÖÜí= éçäóéÉéíáÇÉ= ÖÉåÉ= ÉåÜ~åÅÉê= áå= _JÅÉääëI= âáå~ëÉ= ÅçãéäÉñJ~ëëçÅá~íÉÇ=éêçíÉáå=Efh_h^mF=ÖÉåÉ=ïÜáÅÜ=ÉåÅçÇÉë=~=éêçíÉáå= íÉêãÉÇ=fhh


152

Figure 6. One-micron-thick cross-section of a plastic-

embedded sural nerve biopsy specimen. Note the nearly

complete absence of myelinated fibres (arrows) contrasting

with the large number of unmyelinated axons (arrowheads).

(Thionin blue staining. Bar: 5μm.)


ÅçãéäÉñJ~ëëçÅá~íÉÇ=éêçíÉáå= Efh^mFX= íÜáë=ÖÉåÉ= áë= äçÅ~íÉÇ= áå=ÅÜêçãçëçãÉVèPNK=^=ÜáÖÜ=ÑêÉèìÉåÅó=Ü~ë=ÄÉÉå=ëÜçïå=~ãçåÖ=^ëÜâÉå~òá=gÉïë=TK

Hereditary sensory and autonomic neuropathy type IV

eÉêÉÇáí~êó=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜó=íóéÉ=fs=Eepk=fsLep^kfsF= áë= ~äëç= âåçïå= ~ë= ÅçåÖÉåáí~ä= áåëÉåëáíáîáíó= íç= é~áå= ~åÇ= ~åÜóÇêçëáëKaáëÉ~ëÉ= çåëÉí= áë= ~í= ÄáêíÜK= ep^k= fs= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~= ÅçåÖÉåáí~äéêçÑçìåÇ=ëÉåëçêó=äçëë=~ÑÑÉÅíáåÖ=éÉêÅÉéíáçå=çÑ=é~áå=~åÇ=íÉãéÉê~íìêÉI=~åÇ~å= ~ÄëÉåÅÉ= çÑ= ëïÉ~íáåÖK= oçìíáåÉ= ÉäÉÅíêçéÜóëáçäçÖáÅ~ä= ëíìÇáÉë= ëìÅÜ= ~ëãçíçê= ~åÇ= ëÉåëçêó= ÅçåÇìÅíáçå= îÉäçÅáíáÉë= ~êÉ= ìëì~ääó= åçêã~äKeáëíçé~íÜçäçÖáÅ~ä=ÑáåÇáåÖë=Åçåëáëí=çÑ=ÇÉÅêÉ~ëÉÇ=åìãÄÉêë=çÑ=ìåãóÉäáå~íÉÇ~åÇ=ëã~ää=ãóÉäáå~íÉÇ=ÑáÄêÉë=áå=ëÉåëçêó=åÉêîÉë=EcáÖìêÉ=TFK


153

Figure 7. This nerve biopsy specimen from a patient with

type IV hereditary sensory and autonomic neuropathy shows

complete preservation of myelinated fibres. In this type of

sensory neuropathy only the unmyelinated fibres are affected.

(Bar: 10μm.)


Hereditary neuropathies with polysystemic manifestations

qÜáë= áë= ~= Öêçìé= çÑ= äáÑÉJíÜêÉ~íÉåáåÖ= ÜÉêÉÇáí~êó= éçäóåÉìêçé~íÜáÉë= ÇìÉ= íçëÉîÉêÉ= éçäóëóëíÉãáÅ= áåîçäîÉãÉåí= áåÅäìÇáåÖ= Å~êÇá~Å= ~åÇ= êÉå~äã~åáÑÉëí~íáçåëK=båçêãçìë=éêçÖêÉëë=Ü~ë=ÄÉÉå=ã~ÇÉ=áå=íÜáë=ÑáÉäÇI=åçí=çåäóçå=íÜÉ=ÖÉåÉíáÅ=~ëéÉÅíë=Äìí=~äëç=çå=íÜÉê~éóI=ÉëéÉÅá~ääó= áå=Ñ~ãáäá~ä=~ãóäçáÇéçäóåÉìêçé~íÜóK

Familial amyloid polyneuropathies

^ãóäçáÇçëÉë=~êÉ=~=Öêçìé=çÑ=ÇáëÉ~ëÉë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=íáëëìÉ=ÇÉéçëáíáçåçÑ= áåëçäìÄäÉ= éêçíÉáåë= ~åÇ= ÑáÄêáä= ~ÖÖêÉÖ~íÉë= çêáÉåíÉÇ= áå= ~=βJéäÉ~íÉÇ= ëÜÉÉíëíêìÅíìêÉ=íÜ~í=Ñçêã=ìåÄê~åÅÜÉÇ=~ãóäçáÇ=ÑáÄêáäë=çÑ=NMJNOåã=Çá~ãÉíÉêI=ïÜáÅÜ~êÉ= ÇÉäÉíÉêáçìë= íç= ëìêêçìåÇáåÖ= íáëëìÉK= ^ãóäçáÇçëáë= Å~å= ÄÉ= ~ÅèìáêÉÇ= çêÜÉêÉÇáí~êóK=qÜÉêÉ=~êÉ=íÜêÉÉ=ã~áå=íóéÉë=çÑ=Ñ~ãáäá~ä=~ãóäçáÇ=éçäóåÉìêçé~íÜóEc^mFI=ÇÉÑáåÉÇ=~ÅÅçêÇáåÖ=íç=íÜÉ=éêÉÅìêëçê=éêçíÉáå=çÑ=~ãóäçáÇW=íê~åëíÜóêÉíáåEqqoF=EÜ ëÉÉ=Å~ëÉ=ëíìÇó=çîÉêäÉ~ÑFI=~éçäáéçéêçíÉáå=^JNI=~åÇ=ÖÉäëçäáå=UJNMKqÜÉ=ãçëí=Åçããçå=c^m=áë=íÜÉ=çåÉ=êÉä~íÉÇ=íç=ãìí~íáçå=çÑ=íÜÉ=íê~åëíÜóêÉíáåÖÉåÉ=Ñáêëí=êÉéçêíÉÇ=áå=mçêíìÖ~äK

Mutated transthyretin-familial amyloid polyneuropathy (TTR-FAP)c^m= ï~ë= Ñáêëí= ÇÉëÅêáÄÉÇ= Äó= ^åÇê~ÇÉ= áå= NVROI= áå= åçêíÜÉêå= mçêíìÖ~äK

qÜÉ=ÇáëÉ~ëÉ=ï~ë=~äëç=ëìÄëÉèìÉåíäó= ÑçìåÇ=íç=ÄÉ=ÉåÇÉãáÅ= áå=g~é~å=~åÇpïÉÇÉåK= bîÉåíì~ääó= qqoJc^m= ï~ë= êÉéçêíÉÇ= áå= ~= åìãÄÉê= çÑ= çíÜÉêÅçìåíêáÉëK

qïç=ã~áå=é~ííÉêåë=çÑ=ëÉåëçêóJãçíçê=ÇÉÑáÅáí=çÅÅìê=áå=é~íáÉåíë=ïáíÜ=qqoJc^mI= ÄçíÜ= ~ëëçÅá~íÉÇ= ïáíÜ= î~êá~ÄäÉ= ~ìíçåçãáÅ= ÇáëíìêÄ~åÅÉ= ~åÇ= Éñíê~JåÉìêçäçÖáÅ~ä=ã~åáÑÉëí~íáçåëK=

Lengthdependent sensorymotor polyneuropathyqóéáÅ~ääó=íÜÉ=Ñáêëí=ëóãéíçãë=çÑ=íÜáë=íóéÉ=çÑ=éçäóåÉìêçé~íÜó=çÅÅìê=áå=~Çìäí

é~íáÉåíë=áå=íÜÉáê=ãáÇJíÜáêíáÉë=áå=mçêíìÖ~äI=~åÇ=çÑíÉå=~ÑíÉê=RM=óÉ~êë=çÑ=~ÖÉ=áåpïÉÇÉåI=g~é~å=~åÇ=cê~åÅÉK=póãéíçãë=ëí~êí=ïáíÜ=ÇáëÅçãÑçêí=çîÉê=íÜÉ=ÑÉÉíIåìãÄåÉëë=~åÇ=çÑíÉå=é~áåK=^í= íÜáë=ëí~ÖÉ=ÅäáåáÅ~ä=Éñ~ãáå~íáçå=Å~å=~äêÉ~ÇóÇÉíÉÅí=~å=áãé~áêÉÇ=íÜÉêã~ä=ëÉåëáÄáäáíó=çîÉê=íÜÉ=ÑÉÉíI=ïáíÜ=~=ÇÉÅêÉ~ëÉÇ=éáåJéêáÅâ=ëÉåë~íáçåK=`çåîÉêëÉäóI=äáÖÜí=íçìÅÜ=~åÇ=éêçéêáçÅÉéíáçå=~êÉ=éêÉëÉêîÉÇK


154



155

Late-onset transthyretin familial amyloid polyneuropathy

Susan, a 55-year-old female, had complained of hand numbness for the past 2 years,

affecting first the right, then the left hand, with paraesthesias, loss of sensation of all

fingers, and gradual wasting. She was in good general condition, with no significant

family history for neurological disorders. Examination showed a loss of sensation of all

fingers with muscle atrophy. Tendon reflexes were normal as was the rest of the

neurological examination. Carpal tunnel syndrome was considered but all fingers were

affected, which is uncommon in carpal tunnel syndrome as compression is normally

restricted to the median nerve, with no overlap on the ulnar nerve territory.

Nerve conduction studies showed a loss of compound motor and sensory action

potentials in both the median and ulnar nerve territories. There were normal

findings in the lower limbs. The right sural nerve action potential was normal (11μV).

Routine blood tests were normal. Carpal tunnel syndrome and ulnar compression

were considered and surgery performed.

A year later the patient started to complain of burning pains in the feet associated

with episodic diarrhoea and gradual weight loss. Neurological examination then

showed a loss of pain and temperature sensation over the feet, up to the mid-leg.

Light touch perception was decreased over the feet and proprioception was normal.

There was a slight decrease of foot dorsiflexion. Ankle reflexes were abolished. In the

upper limbs, temperature sensation was impaired up to the mid-forearm.

Nerve conduction studies showed a marked decrease of the sural nerve action

potentials on both sides: 2μV (normal >10μV). Nerve conduction velocity was 29m/s.

The superficial peroneal nerve action potential was absent.

Blood pressure was measured at 130/70mm Hg with a heart rate of 74/minute. Upon

standing, blood pressure fell to 70/40mm Hg, with a pulse rate at 70/minute and

some dizziness. Electrocardiography identified a left bundle branch block and

echocardiography showed a hypertrophic restrictive cardiomyopathy with an

ejection fraction of 53% and an interventricular septum measurement of 14mm. A

restrictive cardiomyopathy with increased thickness of the septum is highly

suggestive of amyloid cardiomyopathy.

Continued overleaf


jìëÅäÉ=ëíêÉåÖíÜ=~åÇ=íÉåÇçå=êÉÑäÉñÉë=~êÉ=åçêã~äK=qÜáë=åÉìêçäçÖáÅ~ä=ÇÉÑÉÅííóéáÅ~ääó=éçáåíë= íç= íÜÉ= áåîçäîÉãÉåí=çÑ=ìåãóÉäáå~íÉÇ=~åÇ=ëã~ää=ãóÉäáå~íÉÇÑáÄêÉëK

^=ÑÉï=ãçåíÜë=ä~íÉê=ëÉåëçêó=äçëë=ÉñíÉåÇë=~ÄçîÉ=íÜÉ=~åâäÉ=äÉîÉä=çå=ÄçíÜëáÇÉëI= ïáíÜ= ~= ÇáëíìêÄÉÇ= äáÖÜí= íçìÅÜ= éÉêÅÉéíáçå= Çáëí~ääó= Äìí= ÇáëëçÅá~íÉÇëÉåëçêó= äçëë= ëíáää= éêÉëÉåí= ãçêÉ= éêçñáã~ääóK= qÜÉ= åÉìêçäçÖáÅ~ä= ÇÉÑáÅáíéêçÖêÉëëÉë= êÉäÉåíäÉëëäóI= ïáíÜ= ~å= ÉñíÉåëáçå= çÑ= ëÉåëçêó= äçëë= íçï~êÇë= íÜÉéêçñáã~ä=äçïÉê=äáãÄëK=jçíçê=ÇÉÑáÅáí=~ééÉ~êë=áå=íÜÉ=Çáëí~ä=äçïÉê=äáãÄë=~äçåÖïáíÜ= áãé~áêãÉåí= çÑ= äáÖÜí= íçìÅÜ= ~åÇ= ÇÉÉé= ëÉåë~íáçåI= áå= êÉä~íáçå= íç= íÜÉáåîçäîÉãÉåí=çÑ= ä~êÖÉê=ëÉåëçêó=~åÇ=ãçíçê=åÉêîÉ=ÑáÄêÉëK=t~äâáåÖ=ÄÉÅçãÉëáåÅêÉ~ëáåÖäó=ÇáÑÑáÅìäí=ïáíÜ=~=äçëë=çÑ=Ä~ä~åÅÉ=~åÇ=ëíÉééáåÖ=Ö~áíK=kÉìêçé~íÜáÅé~áåë=~êÉ=çÑíÉå=çÑ= íÜÉ=ÄìêåáåÖ= íóéÉI=ïçêëÉ=~í=åáÖÜí=~åÇ=~ëëçÅá~íÉÇ=ïáíÜ~ääçÇóåá~K

aìêáåÖ= íÜÉ= ÑçääçïáåÖ=ãçåíÜë=~åÇ=óÉ~êë=ëÉåëçêó=ÇÉÑáÅáí=ÉñíÉåÇë= íç= íÜÉíÜáÖÜëI=íÜÉå=~ÑÑÉÅíë=íÜÉ=ÑáåÖÉêë=~åÇ=íÜÉ=ÑçêÉ~êã=Öê~Çì~ääóI=~ë=íÜÉ=~åíÉêáçêíêìåâ= áë= áåîçäîÉÇK= jçíçê= ÇÉÑáÅáí= ~äëç= Ñçääçïë= ~= äÉåÖíÜJÇÉéÉåÇÉåíéêçÖêÉëëáçå=~åÇ=ï~äâáåÖ=ïáíÜçìí=~áÇ=ÄÉÅçãÉë= áåÅêÉ~ëáåÖäó=ÇáÑÑáÅìäíK=iáÑÉJíÜêÉ~íÉåáåÖ=~ìíçåçãáÅ=ÇóëÑìåÅíáçå=áë=éêÉëÉåí=~í=íÜáë=ëí~ÖÉ=~äçåÖ=ïáíÜ=äçëëçÑ=ïÉáÖÜí=~åÇ=ãìëÅäÉ=ï~ëíáåÖK=içëë=çÑ=é~áå=ëÉåë~íáçå=ïáíÜ=éêÉëÉêî~íáçå=çÑåçêã~ä=çê=ëìÄåçêã~ä=ëíêÉåÖíÜ=~ääçïë=é~áåäÉëë=íê~ìã~=~åÇ=íÜÉ=ÇÉîÉäçéãÉåíçÑ=éä~åí~ê=ìäÅÉêë=~åÇ=Ñççí=çëíÉç~êíÜêçé~íÜó=E`Ü~êÅçíÛë=àçáåíëFK=få=~=î~êá~ÄäÉéêçéçêíáçå= çÑ= é~íáÉåíëI= ÜçïÉîÉêI= äáÖÜí= íçìÅÜ= áë= ~ÑÑÉÅíÉÇ= É~êäó= ÄìíéêçéêáçÅÉéíáçå=áë=ëé~êÉÇ=áå=ãçëí=Å~ëÉëK


156

Late-onset transthyretin familial amyloid polyneuropathycontinued

Routine blood tests including protein immunoelectrophoresis were normal. A

salivary gland biopsy was normal.

The diagnosis of a late-onset, sporadic form of transthyretin familial amyloid

polyneuropathy was suspected. A nerve biopsy showed amyloid deposits and DNA

testing showed a TTR-Val30Met mutation. The patient subsequently underwent an

orthotopic liver transplantation and was in a stable condition 3 years later.


Lateonset FAPfå= é~íáÉåíë= çîÉê= RM= óÉ~êë= çÑ= ~ÖÉ= ïáíÜ= íÜÉ= qqoJs~äPMjÉí ãìí~íáçåI

ëÅ~ííÉêÉÇ= çìíëáÇÉ= íÜÉ= ÉåÇÉãáÅ= ~êÉ~ë= áå= g~é~åI= íÜÉêÉ= áë= ~= NMLN= ã~äÉéêÉéçåÇÉê~åÅÉK=qÜÉ=ãçëí=Åçããçå=áåáíá~ä=ëóãéíçã=áë=é~ê~ÉëíÜÉëá~ë=áå=íÜÉäÉÖëI= ïáíÜ= ãáäÇ= ëóãéíçãë= çÑ= ~ìíçåçãáÅ= ÇóëÑìåÅíáçåI= ÑêÉèìÉåí= Å~êÇá~ÅáåîçäîÉãÉåíI= äçï= éÉåÉíê~åÅÉI= ~åÇ= ~= Ñ~ãáäó= Üáëíçêó= áå= çåäó= çåÉ= íÜáêÇ= çÑé~íáÉåíëK=aìÉ= íç= íÜÉ= ä~íÉ=çåëÉí=~åÇ= äçï=éÉåÉíê~åÅÉ=çÑ=qqo ãìí~íáçåë= áåëçãÉ=~êÉ~ëI=qqoJc^m=ã~ó=éêÉëÉåí=~ë=ëéçê~ÇáÅ=Å~ëÉëK=

Focal manifestations at onset^ãóäçáÇ=ÇÉéçëáíë=ã~ó=~ÅÅìãìä~íÉ=äçÅ~ääó=~åÇ=áåÇìÅÉ=~=ÑçÅ~ä=ÇÉÑáÅáí=çÑ=~

Åê~åá~ä= åÉêîÉI= ~= åÉêîÉ= íêìåâ= çê= ~= éäÉñìëK= `~êé~ä= íìååÉä= ëóåÇêçãÉ= áë= ~Åçããçå=~åÇ=É~êäó=Äìí=åçåJëéÉÅáÑáÅ=ã~åáÑÉëí~íáçå=çÑ=qqoJc^mK=

Autonomic dysfunction^ìíçåçãáÅ=åÉìêçé~íÜó=áë=åÉ~êäó=Åçåëí~åí=Ñêçã=íÜÉ=îÉêó=ÄÉÖáååáåÖ=áå=É~êäóJ

çåëÉí= qqoJc^mK= `~êÇáçJÅáêÅìä~íçêóI= Ö~ëíêçáåíÉëíáå~ä= ~åÇ= ÖÉåáíçìêáå~êóëóëíÉãë= ~êÉ= Åçããçåäó= ~ÑÑÉÅíÉÇ= áå= íÜáë= ëÉííáåÖK= qÜÉó= ëÉäÇçã= éêÉÅÉÇÉ= íÜÉëÉåëçêóJãçíçê= ã~åáÑÉëí~íáçåëI= ÉñÅÉéí= Ñçê= áåíê~Å~êÇá~Å= ÅçåÇìÅíáçå= Ñ~áäìêÉëK`~êÇáçJÅáêÅìä~íçêó=Çóë~ìíçåçãá~=áë=êÉëéçåëáÄäÉ=Ñçê=çêíÜçëí~íáÅ=ÜóéçíÉåëáçåIïÜáÅÜ=ã~ó=êÉã~áå=ëáäÉåí=çê=Å~ìëÉ=Ñ~íáÖìÉI=ÄäìêêÉÇ=îáëáçå=çê=ÇáòòáåÉëë=ïÜÉåëí~åÇáåÖ= ìéK= d~ëíêçáåíÉëíáå~ä= ã~åáÑÉëí~íáçåë= áåÅäìÇÉ= ÉéáëçÇáÅ= éçëíéê~åÇá~äÇá~êêÜçÉ~I= ëÉîÉêÉ= Åçåëíáé~íáçå= çê= ÄçíÜ= ~äíÉêå~íÉäóK= d~ëíêçé~êÉëáë= ~åÇéçëíéê~åÇá~ä=îçãáíáåÖ=Å~ìëÉ=ÇÉÜóÇê~íáçå=~åÇ=áåÅêÉ~ëÉ=éçëíìê~ä=ÜóéçíÉåëáçå~åÇ= éêçÖêÉëëáîÉ= äçëë= çÑ= ïÉáÖÜíK= få= ãÉåI= ÉêÉÅíáäÉ= ÇóëÑìåÅíáçå= áë= ~å= É~êäóÑÉ~íìêÉ= íÜ~í= ã~ó= éêÉÅÉÇÉ= ëÉåëçêó= ëóãéíçãë= çÑ= åÉìêçé~íÜóK= ^ìíçåçãáÅÇóëÑìåÅíáçå=áë=äÉëë=éêçãáåÉåí=áå=ä~íÉJçåëÉí=qqoJc^mK=

Extraneurological manifestations`~êÇá~Å= áåîçäîÉãÉåí= áë= çÄëÉêîÉÇ= áå= UMB= çÑ= Å~ëÉë= çÑ= qqoJc^mK

mêçÖêÉëëáîÉ= ~ãóäçáÇ= ÇÉéçëáíáçå= ã~ó= áåÇìÅÉ= êÉëíêáÅíáîÉ= Å~êÇáçãóçé~íÜóIÉéáëçÇÉë=çÑ=~êêÜóíÜãá~ë=~åÇ=ëÉîÉêÉ=ÅçåÇìÅíáçå=ÇáëçêÇÉêëK=^íêáçîÉåíêáÅìä~êÄäçÅâ= ~åÇ= ÄìåÇäÉ= Äê~åÅÜ= ÄäçÅâë= ~êÉ= Åçããçå= ~åÇ= çÑíÉå= êÉèìáêÉáãéä~åí~íáçå= çÑ= ~= é~ÅÉã~âÉêK= `~êÇáçãóçé~íÜó= ëÉÉãë= ãçêÉ= Åçããçå~ãçåÖ=ãÉå=ïáíÜ=~=åçåJqqoJs~äPMjÉí ãìí~íáçå=~åÇ=~=ä~íÉ=çåëÉíK

sáíêÉçìë= çé~ÅáíáÉë= ã~ó= Å~ìëÉ= Öê~Çì~ä= îáëì~ä= äçëëI= ~åÇ= íê~ÄÉÅìä~êçÄëíêìÅíáçå= êÉëéçåëáÄäÉ= Ñçê= ÅÜêçåáÅ= çéÉåJ~åÖäÉ= Öä~ìÅçã~K= oÉå~ä


157


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Clinical workupqÉãéÉê~íìêÉI= äáÖÜí= íçìÅÜI= éçëáíáçå= ~åÇ= îáÄê~íçêó= ëÉåëÉëI= ~åÇ= é~áå

ëÉåë~íáçå=ãìëí=ÄÉ=íÉëíÉÇI=~ë=ïÉää=~ë=ãìëÅäÉ=ëíêÉåÖíÜ=~åÇ=íÉåÇçå=êÉÑäÉñÉëKjçíçê=ÇÉÑáÅáí=ãìëí=ÄÉ=Öê~ÇÉÇ=~åÇ=ëÉåëçêó=ÅÜ~åÖÉë=êÉÅçêÇÉÇ=çå=~=ÅÜ~êíÑçê=Åçãé~êáëçåK=

pÉåëçêó= ~Åíáçå= éçíÉåíá~äëI= ïÜáÅÜ= ~êÉ= êÉä~íáîÉäó= ëé~êÉÇ= áå= ëã~ää= ÑáÄêÉåÉìêçé~íÜáÉëI=~êÉ=çÑíÉå=~í=íÜÉ=äçïÉê=äáãáí=çÑ=åçêã~ä=î~äìÉë=áåáíá~ääóI=Äìí=íÜÉåÖê~Çì~ääó= ÇÉÅêÉ~ëÉ= ïáíÜ= éêçÖêÉëëáçå= çÑ= íÜÉ= ÇÉÑáÅáíK= nì~åíáí~íáîÉ= ëÉåëçêóíÉëíáåÖ=~åÇ=ëóãé~íÜáÅ=ëâáå=íÉëíë=Å~å=ÅçåÑáêã=ëã~ää=ÑáÄêÉ=áåîçäîÉãÉåí=ÄÉÑçêÉ~äíÉê~íáçå= çÑ= ëÉåëçêó= ~Åíáçå= éçíÉåíá~äë= ÇÉíÉÅíÉÇ= Äó= êçìíáåÉ= ÅçåÇìÅíáçåëíìÇáÉëK

Diagnosis and diagnostic criteriaqÜÉ=Çá~Öåçëáë=çÑ=qqoJc^m=êÉëíë=çå=íÜÉ=~ëëçÅá~íáçå=çÑ=~=ëÉåëçêóJãçíçê

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få= é~íáÉåíë= ïáíÜçìí= ~= âåçïå= Ñ~ãáäó= Üáëíçêó= çÑ= ~ãóäçáÇçëáëI= qqoJc^mëÜçìäÇ=ÄÉ=ÅçåëáÇÉêÉÇ=áå=Å~ëÉë=ïáíÜ=~=éêçÖêÉëëáîÉ=~ñçå~ä=éçäóåÉìêçé~íÜóçÑ= ìåâåçïå= çêáÖáåI= ÉëéÉÅá~ääó= ïÜÉå= ~ëëçÅá~íÉÇ= ïáíÜ= ~ìíçåçãáÅÇóëÑìåÅíáçåI=Å~êÇá~Å=ã~åáÑÉëí~íáçåë=çê=Å~êé~ä=íìååÉä=ëóåÇêçãÉK

^=Äáçéëó=çÑ=~å=~ÑÑÉÅíÉÇ=çêÖ~åI=ÉëéÉÅá~ääó=~=åÉêîÉ=ÄáçéëóI=ëÜçìäÇ=íÜÉå=ÄÉÅçåëáÇÉêÉÇI=íç=ÇÉãçåëíê~íÉ=íÜÉ=éêÉëÉåÅÉ=çÑ=Éñíê~ÅÉääìä~ê=~ãóäçáÇ=ÇÉéçëáíëáå= íÜÉ= ÉåÇçåÉìêá~ä= ëé~ÅÉK= ^ãóäçáÇ= ÇÉéçëáíë= Å~å= ~äëç= ÄÉ= îáëì~äáëÉÇ= áåãìëÅäÉ= ëéÉÅáãÉåëI= ë~äáî~êó= Öä~åÇë= çê= áå= ~ÄÇçãáå~ä= Ñ~íK= fí= ãìëí= ÄÉêÉãÉãÄÉêÉÇI= ÜçïÉîÉêI= íÜ~í= åÉÖ~íáîÉ= Äáçéëó= ÑáåÇáåÖë= Çç= åçí= êìäÉ= çìí~ãóäçáÇçëáëK=^å=~ÑÑáåáíó=Ñçê=ÅçåÖç=êÉÇ=çê=íÜáçÑä~îáå=q=ëí~áåáåÖ=~äçåÖ=ïáíÜ=~ÅÜ~ê~ÅíÉêáëíáÅ=óÉääçïJÖêÉÉå=ÄáêÉÑêáåÖÉåÅÉ=ìåÇÉê=éçä~êáòÉÇ=äáÖÜí=ÅçåÑáêãë=áíë


158


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Genetics^ë= çÑ= íçÇ~óI= NNV= éçáåí= ãìí~íáçåëI= áåÅäìÇáåÖ= NNP= ~ãóäçáÇçÖÉåáÅ

ãìí~íáçåë= áå= íÜÉ= qqo ÖÉåÉI= Ü~îÉ= ÄÉÉå= áÇÉåíáÑáÉÇK= m~íÜçÖÉåáÅ= ãìí~íáçåëã~áåäó= Å~ìëÉ= åÉìêçé~íÜó= Äìí= ëçãÉ= î~êá~åíë= ~êÉ= ~ëëçÅá~íÉÇ= ïáíÜ= ~éêÉÇçãáå~åí= çê= áëçä~íÉÇ= Å~êÇáçãóçé~íÜó= íÉêãÉÇ= Ñ~ãáäá~ä= ~ãóäçáÇÅ~êÇáçãóçé~íÜóK

qqoJs~äPMjÉí=áë=íÜÉ=ãçëí=ÑêÉèìÉåí=ëìÄëíáíìíáçåI=êÉëìäíáåÖ=áå=~=Öì~åáåÉíç=ÅóíçëáåÉ=ãìí~íáçå= áå=Éñçå=O=çÑ= íÜÉ=ÖÉåÉK= fí= áë=îáêíì~ääó= íÜÉ=çåäó=î~êá~åí


159

Figure 8. Familial amyloid polyneuropathy. Cross-section of a

paraffin-embedded nerve biopsy specimen from a patient with

familial amyloid polyneuropathy. Congo red staining shows

congophilic deposits in the endoneurium of this nerve fascicle

(arrows). (Bar: 20μm.)


ÇÉíÉÅíÉÇ= áå= mçêíìÖ~äI= _ê~òáä= ~åÇ= pïÉÇÉåK= _ó= Åçåíê~ëíI= ~ë= ã~åó= ~ë= PMÇáÑÑÉêÉåí=qqo î~êá~åíë=~êÉ=êÉéçêíÉÇ=áå=g~é~å=~åÇ=áå=cê~åÅÉK=^=äÉëë=ëÉîÉêÉéÜÉåçíóéÉ=çÅÅìêë=áå=é~íáÉåíë=Å~êêóáåÖ=ÅçãéçìåÇ=ÜÉíÉêçòóÖçìë=ãìí~íáçåëIïÜáÅÜ=ã~ó=ÉåÜ~åÅÉ=íÜÉ=ëí~Äáäáíó=çÑ=qqo=íÉíê~ãÉêëK

píêáâáåÖ=ÇáÑÑÉêÉåÅÉë=~êÉ=çÄëÉêîÉÇ= áå=íÜÉ=ÉñéêÉëëáçå=çÑ= íÜÉ=ÇáëÉ~ëÉK=^í~ÖÉ= RM= óÉ~êëI= SMB= çÑ= mçêíìÖìÉëÉ= Å~êêáÉêë= ~êÉ= ëóãéíçã~íáÅ= EáKÉKéÉåÉíê~åÅÉ= SMBFI= ïÜÉêÉ~ë= áí= áë= çåäó= NUB= ~åÇ= NNB= áå= cêÉåÅÜ= ~åÇpïÉÇáëÜ=Ñ~ãáäáÉëI=êÉëéÉÅíáîÉäóK=dÉåÉíáÅ=áåÑçêã~íáçå=Å~å=ÄÉ=ÇÉäáîÉêÉÇ=íç=~íJêáëâ= Ñ~ãáäó= ãÉãÄÉêëK= ak^= íÉëíáåÖ= çÑÑÉêë= íÜÉ= éçëëáÄáäáíó= çÑ= ~éêÉëóãéíçã~íáÅ= éêÉÇáÅíáîÉ= Çá~ÖåçëáëK= qÜÉëÉ= íÉëíë= ~êÉ= éÉêÑçêãÉÇ~ÅÅçêÇáåÖ= íç= ÖìáÇÉäáåÉë= ëáãáä~ê= íç= íÜçëÉ= ~ééäáÉÇ= Ñçê= çíÜÉê= ~ìíçëçã~äÇçãáå~åí= åÉìêçÇÉÖÉåÉê~íáîÉ= ÇáëÉ~ëÉëK= qÜÉó= êÉèìáêÉ= éëóÅÜçäçÖáÅ~äëìééçêí=âÉÉéáåÖ=áå=ãáåÇ=íÜÉ=ä~íÉ=çåëÉí= áå=ëçãÉ=Ñ~ãáäáÉë=~åÇ=íÜÉ=î~êá~ÄäÉ~åÇ=áåÅçãéäÉíÉ=éÉåÉíê~åÅÉK=

Treatment of TTRFAP

Treatment of symptomskÉìêçé~íÜáÅ= é~áå= áë= çÑíÉå= ÇáëíìêÄáåÖ= áå= qqoJc^mK= d~Ä~éÉåíáåI

éêÉÖ~Ä~äáå=çê=ÇìäçñÉíáåÉ=Å~å=ÄÉ=ìëÉÑìä=áå=íÜáë=ëÉííáåÖK=mçëíìê~ä=ÜóéçíÉåëáçåçÑíÉå= êÉèìáêÉë= íÜÉ= Å~êÉÑìä= ìëÉ= çÑ= VJ~äéÜ~JÑäìçêçÜóÇêçÅçêíáëçåÉKfãéä~åí~íáçå= çÑ= ~= éÉêã~åÉåí= é~ÅÉã~âÉê= áë= êÉèìáêÉÇ= áå= ãçëí= é~íáÉåíëK^êêÜóíÜãá~ë=ëÜçìäÇ=ÄÉ=íêÉ~íÉÇ=ïáíÜ=~åíá~êêÜóíÜãáÅ=ÇêìÖë=ïáíÜ=çê=ïáíÜçìí=~åáãéä~åí~ÄäÉ=Å~êÇáçîÉêíÉêJÇÉÑáÄêáää~íçêK

Control of amyloid deposits

Liver transplantationiáîÉê= íê~åëéä~åí~íáçå= EiqF= ~áãë= íç= éêÉîÉåí= íÜÉ= Ñçêã~íáçå= çÑ= ~ÇÇáíáçå~ä

~ãóäçáÇ=ÇÉéçëáíë=Äó=êÉãçîáåÖ=íÜÉ=äáîÉêI=ïÜáÅÜ=áë=íÜÉ=ã~áå=ëçìêÅÉ=çÑ=ãìí~åíqqoK=^=ÄÉåÉÑáí=çÑ=iq=Ü~ë=ÄÉÉå=ÇÉãçåëíê~íÉÇ= Ñçê=é~íáÉåíë=ïáíÜ= íÜÉ=qqoJs~äPMjÉí=ãìí~íáçåI=ïÜç=çÑíÉå=ëìêîáîÉ=ãçêÉ=íÜ~å=OM=óÉ~êë=íÜÉëÉ=Ç~óëI=Äìíiq= ÇçÉë= åçí= éêÉîÉåí= íÜÉ= ÇÉîÉäçéãÉåí= çÑ= ÜÉ~êí= ~êêÜóíÜãá~ëK= iq= ãìëí= ÄÉéÉêÑçêãÉÇ=É~êäó= áå= íÜÉ=ÅçìêëÉ=çÑ=qqoJc^mK=lîÉê=VMB=çÑ=é~íáÉåíë=ïáíÜ=~éìêÉ= ëÉåëçêó= åÉìêçé~íÜó= êÉã~áå= ëí~ÄäÉ= ~ÑíÉê= iq= ~äíÜçìÖÜ= åç= ëáÖåáÑáÅ~åí


160


çÄàÉÅíáîÉ=áãéêçîÉãÉåí=çÑ=~ãóäçáÇ=åÉìêçé~íÜó=çÅÅìêëK=qÜÉ=ã~áå=éêçÖåçëíáÅÑ~Åíçê=~ÑíÉê=iq=áë=íÜÉ=çÅÅìêêÉåÅÉ=çê=ïçêëÉåáåÖ=çÑ=Å~êÇá~Å=ÇóëÑìåÅíáçåK

TTR stabilizersq~Ñ~ãáÇáë=~åÇ=ÇáÑäìåáë~ä=~êÉ=íïç=ïÉääJíçäÉê~íÉÇ=ÇêìÖë=ïÜáÅÜ=ÅçåíêáÄìíÉ=íç

ëí~Äáäáë~íáçå=çÑ=íÜÉ=qqo=íÉíê~ãÉê=~åÇ=éêÉîÉåí=ÑìêíÜÉê=ÇÉéçëáíáçå=çÑ=~ãóäçáÇK^äíÜçìÖÜ=íÜÉó=Çç=åçí=ëíçé=éêçÖêÉëëáçå=çÑ=qqoJc^m=ÅçãéäÉíÉäóI=íÜÉáê=ÉÑÑÉÅíáë=çÑ=áåíÉêÉëíK

Gene therapydÉåÉ= íÜÉê~éó= áë= ÅìêêÉåíäó= ìåÇÉêÖçáåÖ= ëíìÇáÉë= ïáíÜ= ëã~ää= áåíÉêÑÉêáåÖ

ok^ë=~åÇ=~åíáëÉåëÉ=çäáÖçåìÅäÉçíáÇÉëK=

Apolipoprotein A1 amyloidosis^éçäáéçéêçíÉáå= ^N= ~ãóäçáÇçëáë= áë= îÉêó= ê~êÉK= fí= áë= ÅÜ~ê~ÅíÉêáëÉÇ= Äó

éçäóëóëíÉãáÅ=ã~åáÑÉëí~íáçåë=ïáíÜ=íÜÉ=~ÖÉ=çÑ=çåëÉí=áå=íÜÉ=ÑçìêíÜ=ÇÉÅ~ÇÉ=çÑäáÑÉK=qÜÉ=ÇáëÉ~ëÉ=éêÉÇçãáå~åíäó=~ÑÑÉÅíë=íÜÉ=âáÇåÉóI=äáîÉê=~åÇ=Ö~ëíêçáåíÉëíáå~äíê~ÅíK=^= äÉåÖíÜJÇÉéÉåÇÉåí=éçäóåÉìêçé~íÜó=çÅÅìêë=Äìí= áë=åçí=~=éêçãáåÉåíÑÉ~íìêÉ=çÑ= íÜÉ=ÇáëÉ~ëÉK=mêçÖêÉëëáçå=çÑ= êÉå~ä= äÉëáçåë=Å~å= äÉ~Ç= íç=ÅÜêçåáÅêÉå~ä=Ñ~áäìêÉI=Çá~äóëáëI=~åÇ=êÉä~íÉÇ=éçäóåÉìêçé~íÜóK

Gelsolin amyloidosiseÉêÉÇáí~êó= ÖÉäëçäáå= ~ãóäçáÇçëáë= Eed^F= áë= ~å= ~ìíçëçã~ä= Ççãáå~åí

ëóëíÉãáÅ= ~ãóäçáÇçëáëI= Ñáêëí= ÇÉëÅêáÄÉÇ= áå= NVSV= áå= cáåä~åÇK= ed^= áëÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~å= ~Çìäí= çåëÉí= çÑ= ~= ëäçïäó= éêçÖêÉëëáîÉ= éÉêáéÜÉê~äåÉìêçé~íÜóI= ïáíÜ= ÅçêåÉ~ä= ä~ííáÅÉ= ÇóëíêçéÜóI= Åê~åá~ä= åÉìêçé~íÜóI= ~åÇ= Åìíáëä~ñ~K=qÜÉ=~ãóäçáÇ=ÑáÄêáäë=~êÉ=ÅçãéçëÉÇ=çÑ=Ñê~ÖãÉåíë=çÑ=ÖÉäëçäáå=ÖÉåÉê~íáåÖ^dÉä= Å~ìëÉÇ= Äó= ~= ãìí~íáçå= çÑ= íÜÉ= ÖÉäëçäáå= ÖÉåÉK= qÜÉ= åÉìêçé~íÜó= áëêÉä~íáîÉäó=ãáäÇK=låäó=~=ÑÉï=Å~ëÉë=Ü~îÉ=ÄÉÉå=êÉéçêíÉÇ=çìíëáÇÉ=cáåä~åÇK

Fabry's disease

c~Äêó=ÇáëÉ~ëÉ=áë=~å=uJäáåâÉÇ=äóëçëçã~ä=ëíçê~ÖÉ=ÇáëçêÇÉê=Å~ìëÉÇ=Äó=íÜÉÇÉÑáÅáÉåí=~Åíáîáíó=çÑ=íÜÉ=äóëçëçã~ä=ÖäóÅçÜóÇêçä~ëÉ=ÉåòóãÉI=αJÖ~ä~ÅíçëáÇ~ëÉ^= NNK= qÜáë= ÉåòóãÉ= ÇÉÑÉÅí= äÉ~Çë= íç= íÜÉ= éêçÖêÉëëáîÉ= ~ÅÅìãìä~íáçå= çÑÖäçÄçíêá~çëóäÅÉê~ãáÇÉ= EdiJPF= ~åÇ= êÉä~íÉÇ= ÖäóÅçëéÜáåÖçäáéáÇëI= ïáíÜáå


161


î~ëÅìä~ê= ÉåÇçíÜÉäá~ä= ÅÉääëI= Ççêë~ä= êççí= Ö~åÖäá~= åÉìêçå~ä= ÅÉääë= ~åÇéÉêáåÉìêá~ä=ÅÉääë= EcáÖìêÉ=VFK=qÜáë=åÉìêçé~íÜó= áë=~ëëçÅá~íÉÇ=ïáíÜ=é~áå=~åÇÇÉÅêÉ~ëÉÇ= íÜÉêã~ä= ëÉåë~íáçåI= é~êíáÅìä~êäó= íç= ÅçäÇI= ïáíÜ= êÉä~íáîÉéêÉëÉêî~íáçå=çÑ=ä~êÖÉ=ÑáÄêÉ=ÑìåÅíáçåI=ëìÅÜ=~ë=îáÄê~íáçå=ÇÉíÉÅíáçåK=táíÜ=~ÖÉIíÜÉ=áåÅáÇÉåÅÉ=çÑ=äáÑÉJíÜêÉ~íÉåáåÖ=ÅçãéäáÅ~íáçåë=áåîçäîáåÖ=íÜÉ=âáÇåÉóI=ÜÉ~êíI~åÇ= Äê~áåI= éêçÖêÉëëÉëK= píêçâÉë= ~åÇ= íê~åëáÉåí= áëÅÜ~ÉãáÅ= ~íí~Åâë= ~êÉÅçããçåK=^ÅÅçêÇáåÖ=íç=íÜÉ=c~Äêó=oÉÖáëíêóI=íÜÉ=~îÉê~ÖÉ=äáÑÉ=ÉñéÉÅí~åÅó=çÑãÉå=ïáíÜ=c~Äêó=ÇáëÉ~ëÉ= áë=RUKO=óÉ~êë=~åÇ=íÜ~í=çÑ=ïçãÉå=TRKQ=óÉ~êë=NOKbåòóãÉ= êÉéä~ÅÉãÉåí= íÜÉê~éó= Ü~ë= ~= éçëáíáîÉ= ÉÑÑÉÅí= çå= êÉå~ä= ~åÇ= Å~êÇá~ÅÑìåÅíáçå= EÉëéÉÅá~ääó= áå= íÜÉ= É~êäó= ëí~ÖÉFI= Ö~ëíêçáåíÉëíáå~ä= ÇáëíìêÄ~åÅÉIåÉìêçé~íÜáÅ=é~áåI=íÜÉêã~ä=ëÉåë~íáçåI=~åÇ=ëïÉ~í=ÑìåÅíáçåK

Hereditary neuropathy with central nervous systeminvolvement: giant axonal neuropathy

dá~åí= ~ñçå~ä= åÉìêçé~íÜó= Ed^kF= áë= ~= ê~êÉ= ÜÉêÉÇáí~êó= ~ìíçëçã~äêÉÅÉëëáîÉ=åÉìêçÇÉÖÉåÉê~íáîÉ=ÇáëÉ~ëÉ=~ÑÑÉÅíáåÖ=ÄçíÜ=íÜÉ=éÉêáéÜÉê~ä=~åÇíÜÉ=ÅÉåíê~ä=åÉêîçìë=ëóëíÉã=NPK qÜÉ=çåëÉí=çÅÅìêë=ÇìêáåÖ=íÜÉ=Ñáêëí=ÇÉÅ~ÇÉ


162

Figure 9. Fabry’s disease. Cross-section of a one-micron-

thick plastic-embedded nerve specimen to show characteristic

osmiophilic inclusions in perineurial cells (arrows). (Bar:

10μm.)


çÑ=äáÑÉ=çå=~îÉê~ÖÉK=qÜÉ=ã~áå=ã~åáÑÉëí~íáçåë=~êÉ=éÉêáéÜÉê~ä=ëÉåëçêó=ãçíçêåÉìêçé~íÜó=~ëëçÅá~íÉÇ=ïáíÜ=~=ëÉÅçåÇ~êó=~åÇ=î~êáçìë=áåîçäîÉãÉåí=çÑ=íÜÉÅÉåíê~ä=åÉêîçìë=ëóëíÉãI=~åÇ=íáÖÜíäóJÅìêäÉÇ=Ü~áêK= fí=Ü~ë=~=î~êóáåÖ=ÅçìêëÉÑêçã=Å~ëÉ=íç=Å~ëÉ=ïáíÜ=~=ÇáîÉêëÉ=éêçÖåçëáëK=lå=åÉêîÉ=Äáçéëó=~ää=é~íáÉåíëëÜ~êÉ= íÜÉ= éêÉëÉåÅÉ= çÑ= ~= î~êá~ÄäÉ= åìãÄÉê= çÑ= Öá~åí= ~ñçåë= ÑáääÉÇ= ïáíÜåÉìêçÑáä~ãÉåí= áå= íÜÉ= åÉêîÉ= ÄáçéëóI= ïÜáÅÜ= êÉéêÉëÉåíë= íÜÉ= é~íÜçäçÖáÅ~äÜ~ääã~êâ=çÑ=íÜÉ=ÇáëÉ~ëÉ=EcáÖìêÉ=NMFK=d^k=áë=Å~ìëÉÇ=Äó=ãìí~íáçåë=áå=íÜÉÖÉåÉI=d^kI=ÉåÅçÇáåÖ= íÜÉ=ÖáÖ~ñçåáå=éêçíÉáåK=qÜÉ=Çáë~Äáäáíó=éêçÖêÉëëÉëëäçïäóK= m~íáÉåíë= ÄÉÅçãÉ= ïÜÉÉäÅÜ~áêJÄçìåÇ= ~í= ~å= ~ÖÉ= íÜ~í= Å~å= î~êóÄÉíïÉÉå= íÜÉ= ëÉÅçåÇ= íç= íÜÉ= ÑçìêíÜ= ÇÉÅ~ÇÉX= ÇÉ~íÜ= çÅÅìêë= ÄÉíïÉÉå= íÜÉ~ÖÉë=çÑ=OM=~åÇ=SM=óÉ~êëK

Hereditary neuropathies and cerebellar ataxia

^= ëÉåëçêó= åÉìêçé~íÜóI= ìëì~ääó= ~ëóãéíçã~íáÅI= áë= éêÉëÉåí= áåcêáÉÇêÉáÅÜDë= ÇáëÉ~ëÉ= ïÜáÅÜ= áë= ã~áåäó= ÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~= ëÉîÉêÉéêçÖêÉëëáîÉ=~í~ñá~I=~êÉÑäÉñá~=~åÇ=ÑêÉèìÉåí=Å~êÇá~Å=áåîçäîÉãÉåíK=få=ëÉîÉê~äçíÜÉê=ÜÉêÉÇáí~êó=ÅÉêÉÄÉää~ê=~í~ñá~ë= íÜÉêÉ= áë=~=ëìÄÅäáåáÅ~ä= áåîçäîÉãÉåí=çÑíÜÉ=éÉêáéÜÉê~ä=åÉêîçìë=ëóëíÉãK


163

Figure 10. Giant axonal neuropathy. One-micron-thick cross-

section of a plastic-embedded nerve specimen to show

characteristic ‘giant’ axons (arrows) surrounded by a thin

myelin sheath. (Thionin blue staining. Bar: 20μm.)


få= ëìãã~êó= Ñçê= íÜáë= ÅÜ~éíÉêI= Ñ~ãáäá~ä= åÉìêçé~íÜáÉë= ~ÅÅçìåí= Ñçê= ~åáãéçêí~åí= éêçéçêíáçå= çÑ= ÅÜêçåáÅ= åÉìêçé~íÜáÉëI= ïÜáÅÜI= áå= ~ÇÇáíáçå= íç= íÜÉÄìêÇÉå= çÑ= íÜÉ= ÇáëÉ~ëÉI= Å~êêó= íÜÉ= êáëâ= çÑ= íê~åëãáëëáçå= çÑ= íÜÉ= ÇáëÉ~ëÉ= íççÑÑëéêáåÖK= qÜÉ= ã~áå= ã~åáÑÉëí~íáçåë= ~åÇ= ÑÉ~íìêÉë= ïÜáÅÜ= ëÜçìäÇ= ~äÉêí= íÜÉé~íáÉåí= ~åÇ= éê~ÅíáíáçåÉêë= áåÅäìÇÉ= ~= éçëáíáîÉ= Ñ~ãáäó= Üáëíçêó= Ñçê= åÉìêçé~íÜóïÜáÅÜ=áë=ÑçìåÇ=áå=ãçëí=é~íáÉåíë=ïáíÜ=~å=~ìíçëçã~ä=Ççãáå~åí=ÇáëçêÇÉêK=qÜÉ~ëëçÅá~íáçå= çÑ= åÉìêçäçÖáÅ~ä= ã~åáÑÉëí~íáçåë= ïáíÜ= ëâÉäÉí~ä= ~Äåçêã~äáíáÉëëÜçìäÇ=~äëç=êáåÖ=~=ÄÉääK=aáëçêÇÉêë=ïáíÜ=~=êÉÅÉëëáîÉ=íê~åëãáëëáçå=ëÜçìäÇ=ÄÉëìëéÉÅíÉÇ= áå= Å~ëÉë= çÑ= Åçåë~åÖìáåáíóK= tÜÉå= ~= Ñ~ãáäá~ä= åÉìêçé~íÜó= áëëìëéÉÅíÉÇ=çê=Çá~ÖåçëÉÇI=íÜÉ=é~íáÉåí=ëÜçìäÇ=ÄÉ=êÉÑÉêêÉÇ=íç=~å=ÉñéÉêí=ÅÉåíêÉÑçê=íÜÉ=ÄÉëí=ã~å~ÖÉãÉåí=~åÇ=ÖÉåÉíáÅ=ÅçìåëÉääáåÖK


164

√ The typical CMT phenotype is characterised by onset in the first decades of

life, of slowly progressive bilateral muscle wasting and weakness starting with

the feet and legs, and then gradually ascending to the distal third of the thigh

and hands. It is often associated with pes cavus.

√ The autosomal dominant CMT, CMT1A, is related to duplication of the 17p

peripheral myelin protein 22 (PMP22), or less often to PMP22 point mutation.

CMT1B is related to mutation of the myelin P zero protein.

√ Autosomal recessive cases are frequent in populations with a high rate of

consanguineous marriages. Onset is in the first decade of life and the

progression is rapid and severe.

√ Hereditary sensory neuropathies encompass a variety of purely or

predominantly sensory polyneuropathies that may or may not be associated

with manifestations of dysautonomia.

√ Hereditary neuropathies with polysystemic manifestations include autosomal

dominant familial amyloid polyneuropathies (FAP) and X-linked Fabry’s disease.

Early liver transplantation is the first-line treatment in transthyretin-FAP.

√ Genetic counselling is mandatory in these diseases.

Ü Key points


References

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kÉìêçä=OMNPX=NNRW=UVPJVMSK=

TK ^ñÉäêçÇ=c_K=c~ãáäá~ä=Çóë~ìíçåçãá~K=jìëÅäÉ=kÉêîÉ=OMMQX=OVW=PROJSPK

UK= mä~åí¨J_çêÇÉåÉìîÉ=sI=p~áÇ=dK=c~ãáäá~ä=~ãóäçáÇ=éçäóåÉìêçé~íÜóK=i~åÅÉí=kÉìêçä=OMNNX

NMW=NMUSJVTK

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VPPJUK


165



166


167

Neuropathic pain

Chapter 11

Overview

This chapter defines the different patterns of neuropathic pain, the associated

conditions and treatments available. Focal pain occurs in trigeminal neuralgia,

Herpes zoster infection, brachial neuritis, sciatica and proximal diabetic neuropathy.

Pain in generalised, length-dependent polyneuropathies is common in small fibre

diabetic neuropathy. In multifocal neuropathy, pain occurs in demyelinating and

axonal processes. First-line treatment includes the use of antidepressant and

antiepileptic drugs. Topical treatment is used in post-herpetic neuralgia. The

practical management of patients with painful neuropathy is also presented.

Introduction

m~áå= áë= íÜÉ= ãçëí= Åçããçå= ~åÇ= ÇáëíìêÄáåÖ= ëóãéíçã= çÑ= éÉêáéÜÉê~äåÉìêçé~íÜóK= péçåí~åÉçìë= é~áå= Å~å= ÄÉ= íÜÉ= éêÉëÉåíáåÖ= ëóãéíçã= çêÅçãéäáÅ~íÉ= íÜÉ= ÅçìêëÉ= çÑ= îáêíì~ääó= ~åó= åÉìêçé~íÜóI= óÉí= áí= çÅÅìêë= ãçêÉÑêÉèìÉåíäó=áå=ëçãÉ=åÉìêçé~íÜáÉëK=^åçíÜÉê=íóéÉ=çÑ=é~áåI=Å~ääÉÇ=Å~ìë~äÖá~=çê~ääçÇóåá~I=ïÜáÅÜ=~ééäáÉë= íç= íÜÉ=é~áåÑìä=éÉêÅÉéíáçå=çÑ=åçêã~ääó=åçåJé~áåÑìäëíáãìäáI= çÅÅìêë= ÑêÉèìÉåíäó= ~ë= ~= ëÉÅçåÇ~êó= éÜÉåçãÉåçå= ~ÑíÉê= åÉêîÉÇ~ã~ÖÉK= få= íÜáë= ÅÜ~éíÉê= ïÉ= ïáää= ÅçåëáÇÉê= íÜÉ= ãçëí= Åçããçå= Å~ìëÉë= çÑåÉìêçé~íÜáÅ= é~áå= ~åÇ= íÜÉ= ã~å~ÖÉãÉåí= çÑ= é~íáÉåíë= ïáíÜ= é~áåÑìäåÉìêçé~íÜáÉëK


Frequency of pain in the different patterns of neuropathy

m~áå= áë= ~= ã~àçê= ÅçãéçåÉåí= çÑ= ëóãéíçãë= íÜ~í= ÅçåíêáÄìíÉ= íç= ~äíÉê= íÜÉèì~äáíó=çÑ=äáÑÉ=áå=é~íáÉåíë=ëìÑÑÉêáåÖ=Ñêçã=ÇáÑÑÉêÉåí=é~ííÉêåë=çÑ=åÉìêçé~íÜóK=qÜÉáåÅáÇÉåÅÉ=çÑ=é~áå= î~êáÉë=ÄÉíïÉÉå=åÉìêçé~íÜáÉëI=Äìí= îáêíì~ääó=~ää= ~ÅèìáêÉÇåÉìêçé~íÜáÉë=Å~å=ÄÉ=é~áåÑìäI=ïÜáäÉ=çåäó=~=ëã~ää=éêçéçêíáçå=çÑ=é~íáÉåíë=ïáíÜÜÉêÉÇáí~êó=åÉìêçé~íÜáÉë=Åçãéä~áå=çÑ=é~áåK

Different types of pain

kÉìêçé~íÜáÅ=é~áå=ÅÜ~ê~ÅíÉêáëíáÅë=î~êó=~ÅÅçêÇáåÖ=íç=íÜÉ=èì~äáíó=çÑ=é~áåIÇáëíêáÄìíáçå=~åÇ=~ëëçÅá~íÉÇ=ã~åáÑÉëí~íáçåëK=kÉìê~äÖá~=~ééäáÉë=íç=é~áå=áå=íÜÉÇáëíêáÄìíáçå=çÑ=~=åÉêîÉ=íêìåâK=fí=Å~å=ÄÉ=é~êçñóëã~ä=çê=éÉêã~åÉåíI=ÄìêåáåÖIçê= ëÜççíáåÖ= áå= å~íìêÉI= íÜÉ= ÄÉëí= Éñ~ãéäÉ= ÄÉáåÖ= íêáÖÉãáå~ä= åÉìê~äÖá~K^ääçÇóåá~=~ééäáÉë=íç=é~áå=ÇìÉ=íç=~=ëíáãìäìë=íÜ~í=ÇçÉë=åçí=åçêã~ääó=éêçîçâÉé~áåK=^å=Éñ~ãéäÉ=ïçìäÇ=ÄÉ=íÜÉ=ìåéäÉ~ë~åí=ëÉåë~íáçå=Å~ìëÉÇ=Äó=íçìÅÜáåÖ~=ëìåÄìêåÉÇ=~êÉ~K=`~ìë~äÖá~=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=ëìëí~áåÉÇ=ÄìêåáåÖ=é~áåI~ääçÇóåá~=~åÇ=ÜóéÉêé~íÜá~=~ÑíÉê=~=íê~ìã~íáÅ=åÉêîÉ=äÉëáçåI=çÑíÉå=~ëëçÅá~íÉÇïáíÜ=î~ëçãçíçê=ÇóëÑìåÅíáçå=áå=íÜÉ=åÉêîÉ=íÉêêáíçêóK=tÜÉå=é~áå=çÅÅìêë=áå=~å~å~ÉëíÜÉíáÅ= ~êÉ~= áí= áë= Å~ääÉÇ= ~å~ÉëíÜÉëá~= Ççäçêçë~K= aóë~ÉëíÜÉëá~= áë= ~åìåéäÉ~ë~åí= ëÉåë~íáçåI= åçí= ~äï~óë= é~áåÑìäI= ìëì~ääó= éêçîçâÉÇ= Äó= ~= åçåJé~áåÑìä= ëíáãìäìëK= `çåîÉêëÉäóI= é~ê~ÉëíÜÉëá~= ~ééäáÉë= íç= ~= åçåJé~áåÑìäëéçåí~åÉçìë=çê=ÉîçâÉÇ=~Äåçêã~ä=ëÉåë~íáçåK=qÜÉ=áåÅêÉ~ëÉÇ=ëÉåëáíáîáíó=~åÇêÉ~Åíáçå=íç=~=åçåJé~áåÑìä=ëíáãìäìë=áë=Å~ääÉÇ=ÜóéÉê~ÉëíÜÉëá~=áÑ=íÜÉ=ëíáãìäìë=áëåçåJé~áåÑìäX= ÜóéÉê~äÖÉëá~= ïÜÉå= íÜÉ= ëíáãìäìë= áë= é~áåÑìäK= eóéÉêé~íÜá~= áë= ~é~áåÑìä=ëóåÇêçãÉ=áå=êÉëéçåëÉ=íç=~=é~áåÑìä=ëíáãìäìëK

qÜÉ=ÅçìêëÉ=çÑ=íÜÉ=é~áåÑìä=ëóåÇêçãÉ=áë=áãéçêí~åí=íç=ÅçåëáÇÉêK=m~áå=Å~åÄÉ= ~ÅìíÉI= ~ë= áå= ëÅá~íáÅ~= çê= Äê~ÅÜá~ä= åÉìêáíáëK= m~áå= ä~ëíáåÖ= ãçêÉ= íÜ~å= PJSãçåíÜë=áë=Å~ääÉÇ=ÅÜêçåáÅI=ëìÅÜ=~ë=áå=éçëíJÜÉêéÉíáÅ=åÉìê~äÖá~I=áå=Å~ìë~äÖá~ëÉÅçåÇ~êó=íç=~=äÉëáçå=çÑ=~=åÉêîÉ=íêìåâ=çê=áå=Çáëí~ä=é~áåÑìä=åÉìêçé~íÜóK=táíÜÅÜêçåáÅ= é~áå= áí= áë= áãéçêí~åí= íç= í~âÉ= áåíç= ~ÅÅçìåí= íÜÉ= áåÑäìÉåÅÉ= çÑé~íÜçéÜóëáçäçÖáÅ~äI= éëóÅÜçäçÖáÅ~ä= ~åÇ= ëçÅá~ä= Ñ~Åíçêë= áå= áíë= ÇÉîÉäçéãÉåí~åÇ=éÉêëáëíÉåÅÉK


168


Pain in focal neuropathies

Cranial nerves: trigeminal neuralgia

qêáÖÉãáå~ä=åÉìê~äÖá~=çê= ÚíáÅ=ÇçìäçìêÉìñ=ÇÉ= ä~= Ñ~ÅÉÛ=ëí~åÇë=~é~êí= áå= íÜÉÑáÉäÇ=çÑ=é~áåÑìä=åÉìêçé~íÜáÉë=ÄÉÅ~ìëÉ=é~áå=áë=íÜÉ=çåäó=ã~åáÑÉëí~íáçå=çÑ=íÜáëëóåÇêçãÉ=EÜ ëÉÉ=Å~ëÉ=ëíìÇó=ÄÉäçïFK=qóéáÅ~ä=íêáÖÉãáå~ä=åÉìê~äÖá~=çÅÅìêëáå=çäÇÉê=éÉçéäÉI=ãçëíäó=~ÑíÉê=íÜÉ=~ÖÉ=çÑ=RM=óÉ~êëK=fí=áë=ÅÜ~ê~ÅíÉêáëÉÇ=Äó=~çåÉJëáÇÉÇI=ëÜççíáåÖI= äáÖÜíåáåÖJäáâÉI=ìåÄÉ~ê~ÄäÉ=~íí~Åâ=çÑ=Ñ~Åá~ä=é~áå=áå=íÜÉíÉêêáíçêó= çÑ= çåÉ= çÑ= íÜÉ= íÜêÉÉ= Äê~åÅÜÉë= çÑ= íÜÉ= íêáÖÉãáå~ä= åÉêîÉK= qÜáëéêÉÇçãáå~íÉë= áå= íÜÉ= áåÑê~çêÄáí~ä= Äê~åÅÜ= çê= áå= íÜÉ= äçïÉê= à~ï= ~êÉ~K

Chapter 11 Neuropathic pain

169

Trigeminal neuralgia

Mary is a 67-year-old woman who consulted for facial pain that started 2 weeks

previously. The patient was in good general condition and had no known illness. The

pain was intermittent, occurring with attacks lasting a few minutes. The pain was

located in the lower third of the face on the right side, in the lower jaw area. Pain

was triggered by chewing, speaking and even by her face being exposed to wind. The

patient stopped eating solid food to avoid chewing. She described the pain as

repetitive and shooting, and tried to protect her face with her hand during these

attacks, which left her exhausted. Neurological examination was normal; of note,

particularly, there was no sensory or motor deficit in the third branch of the

trigeminal nerve. An MRI of the brain had been performed previously, which was

normal. Routine blood tests were also normal. The week before the consultation she

had had two teeth removed on the same side, which did not influence the pain.

In summary, this patient had a typical trigeminal neuralgia involving the third branch

of the trigeminal nerve, which is often mistaken for a dental problem.

Tegretol® 100mg three times per day was started. It was gradually increased to a total

dose of 900mg/d within a couple of weeks. No disturbing side effects occurred,

particularly no ataxia, and the blood cell count and sodium levels remained normal. The

patient still had some minor attacks of facial pain, but these were much milder than

before treatment. She will remain on treatment for an undetermined period of time.


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Herpes zoster and postherpetic neuralgia (PHN)

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170


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qÜÉ=íêÉ~íãÉåí=çÑ=mek=áë=ìëì~ääó=ÇáÑÑáÅìäí=~åÇ=íÜÉ=é~áå=áå=ã~åó=Å~ëÉë=ã~óÄÉ=áåíê~Åí~ÄäÉK=qÜÉêÉ=áë=åç=~ÅÅÉéíÉÇ=êÉÖáãÉå=~åÇ=~ÖÉåíë=íÜ~í=Ü~îÉ=ÄÉÉåëÜçïå= íç= éçëëÉëë= ëçãÉ= ÅäáåáÅ~ä= ÉÑÑáÅ~Åó= ã~ó= áåÅäìÇÉW= íêáÅóÅäáÅ~åíáÇÉéêÉëë~åíë=E~ãáíêáéíóäáåÉFI=Å~äÅáìã=ÅÜ~ååÉä=αOJδ äáÖ~åÇë=EÖ~Ä~éÉåíáåIéêÉÖ~Ä~äáåFI= é~íÅÜÉë= çÑ= íçéáÅ~ä= äáÇçÅ~áåÉI= çéáçáÇ= ~ÖçåáëíëI= ~åÇ~åíáÅçåîìäë~åíë= EëçÇáìã= î~äéêç~íÉFK= qçéáÅ~ä= ~ééäáÅ~íáçå= çÑ= Å~éë~áÅáåçáåíãÉåíI=Ñçìê=íáãÉë=~=Ç~óI=ã~ó=ÜÉäéK=

Radicular pains

^ÅìíÉ=çê=ëìÄ~ÅìíÉ=ê~ÇáÅìä~ê=é~áå=áë=~=ã~àçê=ëóãéíçã=çÑ=Äê~ÅÜá~ä=åÉìêáíáë~åÇ= áå= ëÅá~íáÅ~K= m~áå= áë= ~äëç= ~= ã~àçê= ëóãéíçã= çÑ= íÜÉãÉåáåÖçê~ÇáÅìäçåÉìêáíáë= çÑ= ióãÉ= ÇáëÉ~ëÉ= EÜ ëÉÉ= Å~ëÉ= ëíìÇó= çå= éVMI`Ü~éíÉê=T=Ô=fåÑÉÅíáçìë=åÉìêçé~íÜáÉëFK=tÜÉå=ê~ÇáÅìä~ê=é~áå= áë=êÉä~íÉÇ=íçÇáëÅ=ÅçãéêÉëëáçå=áí= áë=çÑíÉå=ëÉäÑJäáãáíáåÖI= ä~ëíáåÖ=~=ÑÉï=ïÉÉâë=çê=ãçåíÜëKo~ÇáÅìä~ê= é~áå= áë= ìëì~ääó= ïÉää= ÅçåíêçääÉÇ= Äó= áããçÄáäáë~íáçå= çÑ= íÜÉ= ëéáåÉKlÅÅ~ëáçå~ääó=ëìêÖÉêó=áë=åÉÉÇÉÇ=íç=êÉãçîÉ=~=ÇáëÅ=ÜÉêåá~íáçå=çê=~=íìãçìêK

Brachial plexus

_ê~ÅÜá~ä= åÉìêáíáë= áë= ~äëç= ~å= ~ÅìíÉ= çê= ëìÄ~ÅìíÉ= ëóãéíçãK= fí= áëÅÜ~ê~ÅíÉêáëÉÇ=Äó=ëÜ~êéI=ëÉîÉêÉ=é~áå=áå=íÜÉ=åÉêîÉë=çÑ=íÜÉ=Äê~ÅÜá~ä=éäÉñìëI


171


ÑçääçïÉÇ=Äó=ïÉ~âåÉëë=çê=åìãÄåÉëëK=qÜÉ=Å~ìëÉ=çÑ=~ÅìíÉ=Äê~ÅÜá~ä=åÉìêáíáëáë=ìåâåçïåK=qÜÉ=ÅçìêëÉ=ÉñíÉåÇë=çîÉê=~=ÑÉï=ãçåíÜë=áå=ãçëí=Å~ëÉëK=m~áåêÉëéçåÇë= íç= ÅçêíáÅçëíÉêçáÇëI= ~í= äÉ~ëí= é~êíá~ääóK= oÉëáÇì~ä= ïÉ~âåÉëë= áëÅçããçåK

få= ê~Çá~íáçåJáåÇìÅÉÇ= Äê~ÅÜá~ä= éäÉñçé~íÜóI= é~áå= áë= äÉëë= Åçããçå= ~åÇÇáëíìêÄáåÖ=íÜ~å=áå=éäÉñçé~íÜó=ÇìÉ=íç=áåî~ëáçå=Äó=ã~äáÖå~åí=ÅÉääëK=

Proximal diabetic neuropathy

aá~ÄÉíáÅ= é~íáÉåíë= ìëì~ääó= çîÉê= íÜÉ= ~ÖÉ= çÑ= RMI= ãçëíäó= ïáíÜ= íóéÉ= OÇá~ÄÉíÉëI= ã~ó= éêÉëÉåí= ïáíÜ= éêçñáã~ä= åÉìêçé~íÜó= çÑ= íÜÉ= äçïÉê= äáãÄëÅÜ~ê~ÅíÉêáëÉÇ= Äó= ~= î~êá~ÄäÉ= ÇÉÖêÉÉ= çÑ= é~áå= ~åÇ= ëÉåëçêó= äçëë= çîÉê= íÜÉ~åíÉêáçê= ~ëéÉÅí= çÑ= íÜÉ= íÜáÖÜI= ~ëëçÅá~íÉÇ= ïáíÜ= ìåáJ= çê= Äáä~íÉê~ä= éêçñáã~äãìëÅäÉ=ïÉ~âåÉëë=~åÇ=~íêçéÜóK=qÜÉ=é~íáÉåí=Åçãéä~áåë=çÑ=åìãÄåÉëë=çê=é~áåçÑ=íÜÉ=~åíÉêáçê=~ëéÉÅí=çÑ=íÜÉ=íÜáÖÜI=çÑíÉå=çÑ=íÜÉ=ÄìêåáåÖ=íóéÉI=ïçêëÉ=~í=åáÖÜí~åÇ= ~ëëçÅá~íÉÇ= ïáíÜ= ~ääçÇóåá~K= m~áå= ~åÇ= éêçñáã~ä= ïÉ~âåÉëë= ~êÉ= ìëì~ääó~ëëçÅá~íÉÇ= ïáíÜ= áåëçãåá~= ~åÇ= ~= ã~êâÉÇ= äçëë= çÑ= ïÉáÖÜíK= m~áå= ìëì~ääóëìÄëáÇÉë=~ÑíÉê=~=ÑÉï=ïÉÉâë=çê=ãçåíÜëK=få=Å~ëÉë=ïáíÜ=~=éêçíê~ÅíÉÇ=ÅçìêëÉ~åÇ=é~áå=êÉëáëíáåÖ=ëóãéíçã~íáÅ=íêÉ~íãÉåíI=éêÉëÅêáéíáçå=çÑ=ÅçêíáÅçëíÉêçáÇëÑçê=~= ÑÉï=ïÉÉâë=Å~å= áåÇìÅÉ=~=Çê~ã~íáÅ= áãéêçîÉãÉåí= EëÉÉ=`Ü~éíÉê=U=Ôaá~ÄÉíáÅ= ~åÇ= ìê~ÉãáÅ= åÉìêçé~íÜáÉëFK= ^å= ~ÇàìëíãÉåí= áå= íÜÉ= íêÉ~íãÉåí= çÑÇá~ÄÉíÉë=áë=ìëì~ääó=êÉèìáêÉÇK

Pain in generalised polyneuropathy

Lengthdependent diabetic polyneuropathy (LDDP)

Small fibre neuropathyfå=íÜáë=Öêçìé=äÉëáçåë=éêÉÇçãáå~íÉ=çå=ëã~ää=ãóÉäáå~íÉÇ=~åÇ=ìåãóÉäáå~íÉÇ

åÉêîÉ= ÑáÄêÉëI= ~ÅÅçìåíáåÖ= Ñçê= íÜÉ= áãé~áêãÉåí= çÑ= é~áå= ~åÇ= íÉãéÉê~íìêÉëÉåë~íáçåK=

iÉåÖíÜJÇÉéÉåÇÉåí= Çá~ÄÉíáÅ= éçäóåÉìêçé~íÜó= EiaamF= áë= íÜÉ= ãçëíÅçããçå=Å~ìëÉ=çÑ=é~áåÑìä=åÉìêçé~íÜó=áå=íÜÉ=ïçêäÇK=pÉîÉê~ä=ãáääáçå=éÉçéäÉïçêäÇïáÇÉ= ~êÉ= ~ÑÑÉÅíÉÇ= Äó= Çá~ÄÉíáÅ= åÉìêçé~íÜáÅ= é~áåK= iaam= ìëì~ääó


172


ÄÉÅçãÉë=ëóãéíçã~íáÅ=óÉ~êë=~ÑíÉê=íÜÉ=çåëÉí=çÑ=íóéÉ=NI=Äìí=áë=çÑíÉå=íÜÉ=Ñáêëí~åÇ= çåäó= ã~åáÑÉëí~íáçå= çÑ= íóéÉ= O= Çá~ÄÉíÉë= çÑ= ã~íìêÉ= çåëÉíK= qÜÉ= áåáíá~äÚéçëáíáîÉÛ=ã~åáÑÉëí~íáçåë=çÑ=ëÉåëçêó=åÉìêçé~íÜó=áåÅäìÇÉ=åìãÄåÉëëI=ÄìêåáåÖÑÉÉíI=~=éáåë=~åÇ=åÉÉÇäÉë=ëÉåë~íáçåI=~åÇ=ä~åÅáå~íáåÖ=é~áåë=Ô=çÑíÉå=ïçêëÉ~í=åáÖÜí=~åÇ=Äó=Åçåí~Åí=EÜ ëÉÉ=Å~ëÉ=ëíìÇáÉë=çå=ééNOMJOOI=`Ü~éíÉê=U=Ôaá~ÄÉíáÅ= ~åÇ= ìê~ÉãáÅ= åÉìêçé~íÜáÉëFK= ^ÅìíÉ= é~áåÑìä= åÉìêçé~íÜó= ïáíÜ~ääçÇóåá~= áë= ëçãÉíáãÉë= ~ëëçÅá~íÉÇ= ïáíÜ= Å~ÅÜÉñá~= ~åÇ= ÇÉéêÉëëáçåIÉëéÉÅá~ääó= áå=óçìåÖ=~Çìäíë=ïáíÜ= íóéÉ=N=Çá~ÄÉíÉëK= få=~=ëíìÇó=éÉêÑçêãÉÇ= áåiáîÉêéççäI=íÜÉ=éêÉî~äÉåÅÉ=çÑ=é~áåÑìä=åÉìêçé~íÜó=ï~ë=Éëíáã~íÉÇ=íç=ÄÉ=ÑçìêíáãÉë=ãçêÉ=Åçããçå=áå=éÉçéäÉ=ïáíÜ=Çá~ÄÉíÉë=íÜ~å=áå=íÜÉ=Åçåíêçä=ë~ãéäÉ=NKqÜÉ=éêÉî~äÉåÅÉ=çÑ=Çá~ÄÉíáÅ=éÉêáéÜÉê~ä=åÉìêçé~íÜó=áåÅêÉ~ëÉë=ïáíÜ=~ÖÉI=~åÇíÉåÇë=íç=ÄÉ=ãçêÉ=Åçããçå=áå=é~íáÉåíë=ïáíÜ=íóéÉ=O=íÜ~å=áå=íÜçëÉ=ïáíÜ=íóéÉ=NÇá~ÄÉíÉë= EëÉÉ= `Ü~éíÉê= U= Ô= aá~ÄÉíáÅ= ~åÇ= ìê~ÉãáÅ= åÉìêçé~íÜáÉëFK= qÜÉéêÉÅáéáí~íáçå=çÑ=~ÅìíÉ=é~áåÑìä=åÉìêçé~íÜó=Å~å=~äëç=Ñçääçï=íÜÉ=Éëí~ÄäáëÜãÉåíçÑ= íáÖÜí= ÖäóÅ~ÉãáÅ= ÅçåíêçäK= få= é~áåÑìä= iaamI= åÉêîÉ= Äáçéëó= ëÜçïë= ~å~ëëçÅá~íáçå=çÑ= äçëë=çÑ=åÉêîÉ= ÑáÄêÉë=ïáíÜ= êÉÖÉåÉê~íáçå=Äó=~ñçå~ä=ëéêçìíáåÖIïÜáÅÜ=ã~ó=Å~ìëÉ=ëéçåí~åÉçìë=é~áå=~åÇ=~ääçÇóåá~=Äó=ëéçåí~åÉçìë=ÑáêáåÖ=OKfå=iaamI=íÜÉ=~åíáçñáÇ~åíI=αJäáéçáÅ=~ÅáÇI= áë= äáÅÉåëÉÇ=çåäó=áå=ëçãÉ=ÅçìåíêáÉëÑçê=íÜÉ=íêÉ~íãÉåí=çÑ=Çá~ÄÉíáÅ=éçäóåÉìêçé~íÜó=~åÇ=áë=ÑçìåÇ=íç=áãéêçîÉ=é~áå=PK

^ãóäçáÇ= éçäóåÉìêçé~íÜó= áë= ~äëç= ~å= áãéçêí~åí= Å~ìëÉ= çÑ= ëã~ää= ÑáÄêÉåÉìêçé~íÜóI= ~ëëçÅá~íÉÇ= ïáíÜ= ~= ê~åÖÉ= çÑ= ëÉåëçêó= ~åÇ= ãçíçê= éêçÖêÉëëáîÉäÉåÖíÜJÇÉéÉåÇÉåí= ÇÉÑáÅáíë= íç= ëÉîÉêÉ= ~ìíçåçãáÅ= ÇóëÑìåÅíáçåK= c~ãáäá~ä= ~åÇ~ÅèìáêÉÇI=äáÖÜí=ÅÜ~áåI=~ãóäçáÇ=åÉìêçé~íÜáÉë=~êÉ=çÑíÉå=~ëëçÅá~íÉÇ=ïáíÜ=ëÉîÉêÉåÉìêçé~íÜáÅ= é~áåI= ïÜáÅÜ= Ü~îÉ= ãìÅÜ= íÜÉ= ë~ãÉ= ÅÜ~ê~ÅíÉêáëíáÅë= ~ë= íÜçëÉçÄëÉêîÉÇ=áå=Çá~ÄÉíáÅ=é~íáÉåíëK=EpÉÉ=`Ü~éíÉê=NM=Ô=eÉêÉÇáí~êó=åÉìêçé~íÜáÉëKF

Large fibre neuropathyfå=~äÅçÜçäáÅ=åÉìêçé~íÜó=~åÇ=áå=åÉìêçé~íÜáÉë=ÇìÉ=íç=ã~äåìíêáíáçåI=åÉêîÉ

äÉëáçåë=ã~êâÉÇäó=éêÉÇçãáå~íÉ=çå=ä~êÖÉê=ãóÉäáå~íÉÇ=ÑáÄêÉëK=póãéíçãë=~êÉçÑíÉå= ã~êâÉÇ= Äó= ëÉîÉêÉ= åÉìêçé~íÜáÅ= é~áå= ïáíÜ= ëéçåí~åÉçìë= é~áå= ~åÇ~ääçÇóåá~=éêÉÇçãáå~íáåÖ=çå=íÜÉ=Çáëí~ä=äçïÉê=äáãÄëK

^ÅìíÉ=é~áåI=ÉëéÉÅá~ääó=íêáÖÖÉêÉÇ=Äó=Åçåí~ÅíI=çÅÅìêë=ÇìêáåÖ=~åÇ=ëÜçêíäó~ÑíÉê=áåÑìëáçå=çÑ=çñ~äáéä~íáåK=få=VRB=çÑ=é~íáÉåíëI=çñ~äáéä~íáå=Å~ìëÉë=~å=~ÅìíÉëóåÇêçãÉ= çÑ= ëÉîÉêÉ= ÅçäÇ= ÜóéÉêëÉåëáíáîáíóI= à~ï= íáÖÜíåÉëëI= Åê~ãéëI= ~åÇéÉêáçê~äI= éÜ~êóåÖÉ~ä= ~åÇ= äáãÄ= é~ê~ÉíÜÉëá~ë= ëççå= ~ÑíÉê= áåÑìëáçå= ïÜáÅÜêÉëçäîÉë=ïáíÜáå=Üçìêë=çê=Ç~óëK


173


qÜ~äáÇçãáÇÉ= Å~å= áåÇìÅÉ= ~= é~áåÑìä= åÉìêçé~íÜó= áå= é~íáÉåíë= íêÉ~íÉÇ= ÑçêãìäíáéäÉ= ãóÉäçã~K= qÜÉ= ÅäáåáÅ~ä= ÑÉ~íìêÉë= ~êÉ= íÜçëÉ= çÑ= ~= é~áåÑìä= ëÉåëçêóäÉåÖíÜJÇÉéÉåÇÉåí= ~ñçå~ä= åÉìêçé~íÜó= çê= äÉëë= ÑêÉèìÉåíäó= ~= ëÉåëçêóåÉìêçåçé~íÜóK= m~íáÉåíë= éêÉëÉåí= ïáíÜ= ëóãéíçãë= çÑ= åìãÄåÉëëIé~ê~ÉëíÜÉëá~ë=ïÜáÅÜ=ã~ó=ÄÉ=é~áåÑìäI=~åÇ=Åê~ãéë=ëí~êíáåÖ=áå=íÜÉ=ÑÉÉíK

Nonlengthdependent polyneuropathy

Demyelinating polyneuropathy

GuillainBarré syndrome (GBS)m~áå=Ü~ë=ÄÉÉå=ãÉåíáçåÉÇ=áå=ìé=íç=RMB=çÑ=é~íáÉåíë=áå=ã~åó=êÉéçêíë=QK

d_p=é~áå=~í=çåëÉí=ìëì~ääó=ëáãìä~íÉë=íÜÉ=ëÉåë~íáçå=íÜ~í=çÅÅìêë=Üçìêë=çê=~Ç~ó=~ÑíÉê=ëíêÉåìçìë=ÉñÉêÅáëÉK=`çããçå=äçÅ~íáçåë=~êÉ=íÜÉ=äçïÉê=Ä~Åâ=~åÇä~êÖÉ= ãìëÅäÉë= çÑ= íÜÉ= íÜáÖÜë= ~åÇ= ÄìííçÅâëI= ëçãÉíáãÉë= ~ÅÅçãé~åáÉÇ= ÄóëÅá~íáÅ~= EÜ ëÉÉ= Å~ëÉ= ëíìÇó= çå= éQQI= `Ü~éíÉê= Q= Ô= dìáää~áåJ_~êê¨ëóåÇêçãÉFK= ^åçíÜÉê= é~áå= ëóåÇêçãÉ= êÉëìäíë= Ñêçã= áåíÉåëÉ= Çáëí~äé~ê~ÉëíÜÉëá~ëK=m~áå= áë=çÑíÉå=ïçêëÉ=~í=åáÖÜíK=pÉîÉêÉ=ãìëÅäÉ=é~áå=ìëì~ääóëìÄëáÇÉë=~ë=ãìëÅäÉ=ëíêÉåÖíÜ=áãéêçîÉëK=

oÉëáÇì~ä= é~áå= Å~å= çÅÅìê= N= çê= O= óÉ~êë= ~ÑíÉê= íÜÉ= çåëÉí= çÑ= d_pI= ïáíÜÇáë~ÄäáåÖ= ëÉåëçêó= ëóãéíçãë= Ü~îáåÖ= ÄÉÉå= êÉéçêíÉÇ= áå= ìé= íç= NRB= çÑé~íáÉåíëK=aáëí~ä=ÄìêåáåÖ=Çóë~ÉëíÜÉëá~ë=éêÉÅáéáí~íÉÇ=Äó=Åçåí~Åí=çê=éêÉëëìêÉã~ó= çÅÅìê= áå= é~íáÉåíë= ïÜç= Ü~îÉ= çíÜÉêïáëÉ= ÅçãéäÉíÉ= ãçíçê= êÉÅçîÉêóKm~ê~ÉëíÜÉëá~ë=ã~ó=ÄÉ=Éñ~ÅÉêÄ~íÉÇ=Äó=ÉñÉêíáçåI=ÜÉ~í=çê=ÅçäÇK

Subacute and chronic inflammatory demyelinating polyneuropathy(CIDP) m~áå=áë=äÉëë=Åçããçå=áå=ëìÄ~ÅìíÉ=~åÇ=ÅÜêçåáÅ=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáåÖ

éçäóåÉìêçé~íÜó= EcáÖìêÉ= NFK= vÉí= áå= ~= êÉéçêí= Äó= _çìÅÜ~êÇ= Éí= ~äI= PMB= çÑé~íáÉåíë=Åçãéä~áåÉÇ=çÑ=é~áå=~í=íÜÉ=çåëÉí=çÑ=íÜÉ=åÉìêçé~íÜóI=ïÜáÅÜ=ÉñíÉåÇÉÇçîÉê=~=ãçåíÜI=ïáíÜ=UB=ëíáää=Åçãéä~áåáåÖ=çÑ=é~áå=áå=~=ëíÉ~Çó=ëí~íÉ=RK

Axonal multifocal neuropathiesm~áå= áë=~å= áãéçêí~åí=~åÇ=åÉ~êäó=Åçåëí~åí=ÅçãéçåÉåí=çÑ= íÜÉ= áëÅÜ~ÉãáÅ

åÉìêçé~íÜó= çÄëÉêîÉÇ= áå= î~ëÅìäáíáëI= íÜÉ= ã~àçê= Å~ìëÉ= çÑ= ãìäíáÑçÅ~ä= ~ñçå~ä


174


åÉìêçé~íÜó= EÜ ëÉÉ= Å~ëÉ= ëíìÇó= çå= éTOI= `Ü~éíÉê= S= Ô= s~ëÅìäáíáÅåÉìêçé~íÜáÉëFK= m~áå= çÅÅìêë= ÄçíÜ= ~ë= ~å= É~êäó= ã~åáÑÉëí~íáçå= çÑ= áëÅÜ~ÉãáÅåÉìêçé~íÜóI= áå= ïÜáÅÜ= Å~ëÉ= áí= êÉëéçåÇë= ïÉää= íç= íêÉ~íãÉåí= ïáíÜÅçêíáÅçëíÉêçáÇëI= ~åÇ= ~ë= ~= êÉëáÇì~ä= ã~åáÑÉëí~íáçå= ëìÄëÉèìÉåí= íç= ~ñçå~ääÉëáçåë=çÑ=åÉêîÉ=íêìåâëI=ïáíÜ=ëÉåëçêó=äçëë=~åÇ=~ääçÇóåá~K=

Management of patients with neuropathic pain S

Symptomatic pharmacological treatment

kÉìêçé~íÜáÅ=é~áå=çÑíÉå=êÉèìáêÉë=íêÉ~íãÉåí=ëéÉÅáÑáÅ~ääó= Ñçê=é~áå=êÉäáÉÑKaìÉ= íç= íÜÉ= ÑêÉèìÉåÅó=çÑ=é~áåÑìä=Çá~ÄÉíáÅ=åÉìêçé~íÜáÉëI=ãçëí=ÅçåíêçääÉÇëíìÇáÉë= Ü~îÉ= ÄÉÉå= éÉêÑçêãÉÇ= áå= é~íáÉåíë= ïáíÜ= Çáëí~ä= Çá~ÄÉíáÅéçäóåÉìêçé~íÜóK


175

Figure 1. One-micron-thick cross-section of a plastic-

embedded sural nerve biopsy specimen from a patient with

painful chronic inflammatory demyelinating polyneuropathy.

The density of unmyelinated fibres (arrows), or C fibres, which

convey pain sensation, is normal in this case. Normal: 17,000-

35,000 unmyelinated fibres per mm². (Bar: 5μm.)


Antidepressants^åíáÇÉéêÉëë~åíë=Ü~îÉ=~=ÄÉåÉÑáÅá~ä=ÉÑÑÉÅí=çå=ãçëí=Å~ëÉë=çÑ=åÉìêçé~íÜáÅ

é~áåK

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aìäçñÉíáåÉ=áë=É~ëó=íç=~ÇãáåáëíÉêX=íÜÉ=ëí~êíáåÖ=ÇçëÉ=áë=PMãÖ=~åÇ=íÜÉ=ÇçëÉÅ~å=ÄÉ= áåÅêÉ~ëÉÇ= íç=SMãÖ=çåÅÉ=Ç~áäóK=sÉåä~Ñ~ñáåÉ= áë=~ÇãáåáëíÉêÉÇ= íïáÅÉÇ~áäó=áå=ÇçëÉë=ìé=íç=NRMJOORãÖ=Ç~áäóK

Antiepileptic drugsj~åó=~åíáÅçåîìäë~åíë=Ü~îÉ=~=éÜ~êã~ÅçäçÖáÅ~ä=~Åíáçå=íÜ~í=ã~ó=áåíÉêÑÉêÉ

ïáíÜ= åÉìêçå~ä= ÜóéÉêÉñÅáí~ÄáäáíóI= Äó= ÇÉÅêÉ~ëáåÖ= ÉñÅáí~íçêó= çê= áåÅêÉ~ëáåÖáåÜáÄáíçêó= íê~åëãáëëáçåK= qÜáë= ï~óI= ~åíáÅçåîìäë~åíë= ã~ó= ~ííÉåì~íÉ= íÜÉåÉìêçå~ä= ÜóéÉêÉñÅáí~Äáäáíó= êÉëéçåëáÄäÉ= Ñçê= ÅÜêçåáÅ= é~áå= ÅçåÇáíáçåëK`~êÄ~ã~òÉéáåÉ= áë=ã~áåäó=ìëÉÇ=ïáíÜ=ëìÅÅÉëë= áå= íêáÖÉãáå~ä=åÉìê~äÖá~K=qÜÉãçëí= Åçããçå= ëáÇÉ= ÉÑÑÉÅíë= çÑ= Å~êÄ~ã~òÉéáåÉ= ~êÉ= ëÉÇ~íáçåI= ÇáòòáåÉëëI~í~ñá~I=ÄäìêêÉÇ=îáëáçåI=Üóéçå~íêÉãá~I=ÅçåÑìëáçå= áå=ÉäÇÉêäó=é~íáÉåíëI=~åÇ= áåê~êÉ=Å~ëÉë=ÄäççÇ=ÇóëÅê~ëá~K=qÜÉ=ëí~êíáåÖ=ÇçëÉ=áë=ìëì~ääó=PMMãÖ=Ç~áäó=~åÇíÜÉ=ÇçëÉ=áë=áåÅêÉ~ëÉÇ=Äó=NMMãÖ=ÉîÉêó=çíÜÉê=Ç~ó=íç=NRMMJOMMMãÖLÇ~óK


176


d~Ä~éÉåíáå= ~åÇ= éêÉÖ~Ä~äáå= ~êÉ= ~= åÉï= ÖÉåÉê~íáçå= çÑ= ~åíáÅçåîìäë~åíëKqÜÉ=ãçëí=Åçããçå=ëáÇÉ=ÉÑÑÉÅíë=çÑ=Ö~Ä~éÉåíáå=~åÇ=éêÉÖ~Ä~äáå=~êÉ=ÅÉåíê~äåÉêîçìë= ëóëíÉãJêÉä~íÉÇ= ëáÇÉ= ÉÑÑÉÅíë= ïáíÜ= ÇáòòáåÉëë= ~åÇ= ëçãåçäÉåÅÉKd~Ä~éÉåíáå= áë= áåáíá~íÉÇ=ïáíÜ=PMMãÖ=Ç~áäóI=ïÜáÅÜ= áë=ëäçïäó= áåÅêÉ~ëÉÇ= íç=~Ñáå~ä= Ç~áäó= ÇçëÉ= çÑ= ÄÉíïÉÉå= NUMM= ~åÇ= PSMMãÖI= ~åÇ= ~ÇãáåáëíÉêÉÇ= íÜêÉÉíáãÉë= Ç~áäóK= mêÉÖ~Ä~äáå= ã~ó= ÄÉ= áåáíá~íÉÇ= ïáíÜ= TR= çê= NRMãÖ= Ç~áäó= ~åÇáåÅêÉ~ëÉÇ=ìé=íç=SMMãÖ=áå=íïç=ÇáîáÇÉÇ=ÇçëÉëK=açëÉ=êÉÇìÅíáçå=áë=åÉÉÇÉÇ=áåé~íáÉåíë=ïáíÜ=áãé~áêÉÇ=êÉå~ä=ÑìåÅíáçåK=m~íáÉåíë=Ñ~áäáåÖ=íç=êÉëéçåÇ=íç=çåÉ=çÑíÜÉëÉ=ÇêìÖë=ã~ó=ÄÉåÉÑáí=Ñêçã=íÜÉ=çíÜÉêK

OpioidsjçêéÜáåÉ=~åÇ=çñóÅçÇçåÉ=Ü~îÉ=ÄÉÉå=ëÜçïå=íç=Ü~îÉ=~å=ÉÑÑÉÅí=áå=é~áåÑìä

åÉìêçé~íÜáÉëI= ~äíÜçìÖÜ= íÜáë= áë= Ä~ëÉÇ= çå= ~= äáãáíÉÇ= åìãÄÉê= çÑ= ëíìÇáÉëKqê~ã~Ççä= áë= ~å= çéáçáÇ= ïáíÜ= ~= ãçåç~ãáåÉêÖáÅ= êÉìéí~âÉ= áåÜáÄáíçêó= ~ÅíáçåïÜáÅÜ=ã~ó=~äëç= êÉäáÉîÉ=åÉìêçé~íÜáÅ=é~áåK=_ÉÑçêÉ= áåáíá~íáåÖ= íêÉ~íãÉåí= áí= áëíÜÉêÉÑçêÉ=áãéçêí~åí=íç=~ÇÇêÉëë=êáëâ=Ñ~Åíçêë=Ñçê=~ÄìëÉK

Topical treatments^= äáÇçÅ~áåÉ= ERBF= ãÉÇáÅ~íÉÇ= é~íÅÜ= áë= áåÇáÅ~íÉÇ= Ñçê= íÜÉ= êÉäáÉÑ= çÑ= é~áå

~ëëçÅá~íÉÇ= ïáíÜ= éçëíJÜÉêéÉíáÅ= åÉìê~äÖá~I= çê= ïáíÜ= äçÅ~äáëÉÇ= åÉìêçé~íÜáÅé~áåK=eáÖÜJÇçëÉ=EUBF=Å~éë~áÅáå=é~íÅÜÉë=~êÉ=~ééêçîÉÇ=Ñçê=íÜÉ=íêÉ~íãÉåí=çÑéÉêáéÜÉê~ä= åÉìêçé~íÜáÅ= é~áå= ~åÇ= Ñçê= íÜÉ= íêÉ~íãÉåí= çÑ= éçëíJÜÉêéÉíáÅåÉìê~äÖá~K= ^ÑíÉê= íêÉ~íáåÖ= íÜÉ= ëâáå= ~êÉ~= ïáíÜ= ~= äçÅ~ä= ~å~ÉëíÜÉíáÅI= íÜÉÅ~éë~áÅáå=é~íÅÜ=êÉã~áåë=~ééäáÉÇ=Ñçê=PMJSM=ãáåìíÉëK=qÜÉ=ÉÑÑÉÅí=ëí~êíë=ïáíÜáåN=Ç~ó= íç=O=ïÉÉâë=~ÑíÉê= íÜÉ=~ééäáÅ~íáçåK=qÜÉ= íêÉ~íãÉåí=ã~ó=ÄÉ= êÉéÉ~íÉÇÉîÉêó=P=ãçåíÜëK

Nonpharmacological treatments

cçê= é~íáÉåíë= êÉÅÉáîáåÖ= éÜ~êã~ÅçäçÖáÅ~ä= íêÉ~íãÉåíI= íÜÉ= ~îÉê~ÖÉ= é~áåêÉÇìÅíáçå= áë=~Äçìí=OMJPMBI=~åÇ=çåäó=OMJPRB=çÑ=é~íáÉåíë=ïáää=~ÅÜáÉîÉ=~íäÉ~ëí= ~= RMB= é~áå= êÉÇìÅíáçå= ïáíÜ= ~î~áä~ÄäÉ= ÇêìÖëK= kçåJéÜ~êã~ÅçäçÖáÅ~äíêÉ~íãÉåíë=åÉÉÇ=íç=ÄÉ=ìëÉÇ=~äçåÖëáÇÉ=éÜ~êã~ÅçäçÖáÅ~ä=íêÉ~íãÉåíëK=qÜÉëÉíêÉ~íãÉåíë=áåÅäìÇÉ=éÜóëáçíÜÉê~éóI=ÅçÖåáíáîÉ=~åÇ=ÄÉÜ~îáçìê~ä=íÜÉê~éóI=~åÇÜóéåçëáëK=


177


qê~åëÅìí~åÉçìë= ÉäÉÅíêáÅ~ä= åÉêîÉ= ëíáãìä~íáçå= ~åÇ= ~ÅìéìåÅíìêÉ= ~êÉëçãÉíáãÉë= ìëÉÇ= Äìí= íÜÉáê= ÉÑÑáÅ~Åó= áå= é~áåÑìä= åÉìêçé~íÜó= áë= åçí= ïÉääÉëí~ÄäáëÜÉÇK=

péáå~ä=ÅçêÇ=ëíáãìä~íáçå=áë=~=íÉÅÜåáèìÉ=ïÜÉêÉÄó=ÉäÉÅíêçÇÉë=~êÉ=áåëÉêíÉÇéÉêÅìí~åÉçìëäó=çê=ÇìêáåÖ=çéÉå=ëìêÖÉêó=áåíç=íÜÉ=éçëíÉêáçê=ÉéáÇìê~ä=ëé~ÅÉíç= ëíáãìä~íÉ= íÜÉ= Ççêë~ä= Åçäìãå= çÑ= íÜÉ= ëéáå~ä= ÅçêÇ= íç= êÉéä~ÅÉ= ~= é~áåÑìäëÉåë~íáçå=ïáíÜ=é~ê~ÉëíÜÉëá~=TK=

péáå~ä= ÅçêÇ= ëíáãìä~íáçå= áë= ÖÉåÉê~ääó= êÉëÉêîÉÇ= Ñçê= é~íáÉåíë= ïÜç= Ü~îÉÑ~áäÉÇ= ÅçåëÉêî~íáîÉ= ã~å~ÖÉãÉåíK= mëóÅÜçäçÖáÅ~ä= ÅäÉ~ê~åÅÉ= áë= ÖÉåÉê~ääóêÉÅçããÉåÇÉÇK= mêáçê= íç= éÉêã~åÉåí= éä~ÅÉãÉåíI= íÜÉ= é~íáÉåí= ìåÇÉêÖçÉëáãéä~åí~íáçå= çÑ= ~= íêá~ä= ëíáãìä~íáçåK= m~áå= êÉäáÉÑ= çÑ= RMB= çê= ÖêÉ~íÉê= áëÖÉåÉê~ääó= ~ÅÅÉéí~ÄäÉ= áå= çêÇÉê= íç= éêçÖêÉëë= íç= éÉêã~åÉåí= áãéä~åí~íáçåKqÜÉ=íêá~ä=éÉêáçÇ=ìëì~ääó=ä~ëíë=ÄÉíïÉÉå=R=~åÇ=T=Ç~óë=~åÇ=éÉêã~åÉåí=ëéáå~äÅçêÇ=ëíáãìä~íçêë=~êÉ=éä~ÅÉÇ=ëÉîÉê~ä=ïÉÉâë=~ÑíÉê=~=ëìÅÅÉëëÑìä=íêá~äK=péáå~äÅçêÇ=ëíáãìä~íáçå=áë=ÅìêêÉåíäó=~ééêçîÉÇ=Ñçê=ÅÜêçåáÅ=é~áå=çÑ=íÜÉ=íêìåâ=~åÇäáãÄëK=

Practical management of patients

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få=é~íáÉåíë=ïáíÜ= ÑçÅ~ä=éÉêáéÜÉê~ä= åÉìêçé~íÜó=ïáíÜ=~ääçÇóåá~I= ~= íçéáÅ~ääáÇçÅ~áåÉ= é~íÅÜ= áë= ~äëç= ~= ÑáêëíJäáåÉ= ÇêìÖK= qêáÅóÅäáÅ= ~åíáÇÉéêÉëë~åíë= çêÇìäçñÉíáåÉ=ã~ó=ÄÉ= íÜÉ= Ñáêëí=ÇêìÖ=çÑ=ÅÜçáÅÉ= áå=é~íáÉåíë=ïáíÜ=ÇÉéêÉëëáçåK`çãÄáå~íáçå=íÜÉê~éó=ã~ó=ÄÉ=ÅçåëáÇÉêÉÇ=áå=é~íáÉåíë=ïáíÜ=~å=áåëìÑÑáÅáÉåíÉÑÑÉÅí=Ñêçã=çåÉ=ÇêìÖK

qêÉ~íãÉåí= áë= ìëì~ääó= ~= íêá~äJ~åÇJÉêêçê= éêçÅÉëë= ~åÇ= Ü~ë= íç= ÄÉáåÇáîáÇì~äáëÉÇ=íç=É~ÅÜ=é~íáÉåíI=í~âáåÖ=áåíç=~ÅÅçìåí=~ää=ÅçãçêÄáÇáíáÉë=ëìÅÜ~ë= éçëëáÄäÉ= ÅçåÅçãáí~åí= ÇÉéêÉëëáçåI= ~åñáÉíóI= ÇáëÉ~ëÉëI= ~åÇ= ÇêìÖ


178


áåíÉê~ÅíáçåëK= _ÉÑçêÉ= ÅÜççëáåÖ= ~= åÉìêçé~íÜáÅ= é~áå= íêÉ~íãÉåíI= ÅäáåáÅá~åëåÉÉÇ= íç= ÅçåëáÇÉê= íÜÉ= ÄÉåÉÑáíë= ~åÇ= éçëëáÄäÉ= ëáÇÉ= ÉÑÑÉÅíë= çÑ= ~= ëéÉÅáÑáÅíêÉ~íãÉåíK=

^ÑíÉê=Éëí~ÄäáëÜáåÖ=íÜÉ=Çá~Öåçëáë=çÑ=é~áåÑìä=åÉìêçé~íÜóI=íÜÉ=ìåÇÉêäóáåÖÇáëÉ~ëÉ=ëÜçìäÇ=ÄÉ=áÇÉåíáÑáÉÇ=~åÇ=íêÉ~íÉÇI=ïÜÉåÉîÉê=éçëëáÄäÉK=tÜÉå=íÜÉìåÇÉêäóáåÖ= ãÉÅÜ~åáëãë= Å~ìëáåÖ= íÜÉ= é~áå= Å~ååçí= ÄÉ= íêÉ~íÉÇIëóãéíçã~íáÅ= íêÉ~íãÉåí=çÑ=é~áå=~åÇ=êÉä~íÉÇ=Çáë~Äáäáíó=ëÜçìäÇ=ÄÉ=çÑÑÉêÉÇKoÉ~äáëíáÅ= ÉñéÉÅí~íáçåë= Ñçê= íÜÉ= çìíÅçãÉ= çÑ= ~= ÖáîÉå= íêÉ~íãÉåí= ëÜçìäÇ= ÄÉÇáëÅìëëÉÇ=ïáíÜ= íÜÉ=é~íáÉåíI= Éñéä~áåáåÖ= íÜ~í=çÑíÉå=çåäó=é~êíá~ä= é~áå= êÉäáÉÑÑêçã= åÉìêçé~íÜáÅ= é~áå= Å~å= ÄÉ= ÉñéÉÅíÉÇI= ~åÇ= íÜ~í= ëÉåëçêó= ÇÉÑáÅáíë= ~êÉìåäáâÉäó=íç=êÉëéçåÇ=íç=íêÉ~íãÉåíK

qÜÉ= íêÉ~íãÉåí= ÉÑÑÉÅí= áë= ìëì~ääó= ~ëëÉëëÉÇ= Äó= ~= êÉÇìÅíáçå= áå= é~áåáåíÉåëáíó=çå=~=îáëì~ä=~å~äçÖìÉ=ëÅ~äÉ=çê=~å=NNJéçáåí=åìãÉêáÅ~ä=ê~íáåÖ=ëÅ~äÉê~åÖáåÖ= Ñêçã= Úåç= é~áåÛ= íç= Úïçêëí= éçëëáÄäÉ= é~áåÛK= qÜáë= ãÉ~ëìêÉ= Å~å= ÄÉëìééäÉãÉåíÉÇ=ïáíÜ=çíÜÉê=ëáãáä~ê=ëÅ~äÉë= íÜ~í=~ëëÉëë= íÜÉ=ÇÉÖêÉÉ=çÑ=é~áåêÉäáÉÑK=s~êáçìë=ãÉ~ëìêÉë=çÑ=èì~äáíó=çÑ=äáÑÉ=Å~å=~äëç=ÄÉ=~ÇÇÉÇK=få=íÜÉ=Å~ëÉçÑ=é~êíá~ä=é~áå=êÉäáÉÑI=~åçíÜÉê=ÇêìÖ=ïáíÜ=ÅçãéäÉãÉåí~êó=ãÉÅÜ~åáëãë=Å~åÄÉ=~ÇÇÉÇK=qÜÉêÉ=áë=~=ÖççÇ=ê~íáçå~äÉ=Ñçê=ÅçãÄáåáåÖ=ÇêìÖë=ïáíÜ=~=ÇáÑÑÉêÉåíãçÇÉ= çÑ= ~Åíáçå= ÄÉÅ~ìëÉ= íÜáë= ã~ó= äçïÉê= íÜÉ= ÑêÉèìÉåÅó= ~åÇ= ëÉîÉêáíó= çÑëáÇÉ=ÉÑÑÉÅíë=~åÇ=Ü~îÉ=~å=~ÇÇáíáîÉ=~åÇ=ã~óÄÉ=ÉîÉå=~=ëóåÉêÖáëíáÅ=ÉÑÑÉÅíIÄìí=íÜÉêÉ=áë=ëíáää=äáãáíÉÇ=ÅäáåáÅ~ä=ÉîáÇÉåÅÉ=Ñçê=íÜÉëÉ=~ëëìãéíáçåëK=qÜÉêÉ=áëëçãÉ= ÉîáÇÉåÅÉ= Ñçê= ÅçãÄáåáåÖ= Ö~Ä~éÉåíáå= ïáíÜ= ~å= çéáçáÇ= çê= ~å~åíáÇÉéêÉëë~åíK=páÇÉ=ÉÑÑÉÅíë=ã~ó=ÄÉ=~=äáãáíáåÖ=Ñ~Åíçê=~åÇ=áí=áë=áãéçêí~åííç=ãçåáíçê=íÜÉ=é~íáÉåí=Ñçê=~ÇÇáíáîÉ=~ÇîÉêëÉ=ÉÑÑÉÅíëK=qêÉ~íãÉåí=íç=~ääÉîá~íÉé~áå=ëÜçìäÇ=ÄÉ=~ëëçÅá~íÉÇ=ïáíÜ=íêÉ~íãÉåí=çÑ=íÜÉ=Å~ìëÉ=çÑ=íÜÉ=åÉìêçé~íÜóïÜÉå=ÑÉ~ëáÄäÉK

m~íáÉåíë=ïáíÜ= êÉÑê~Åíçêó=ÅÜêçåáÅ=åÉìêçé~íÜáÅ=é~áå=Å~å=ÄÉ= êÉÑÉêêÉÇ= íçé~áå=ã~å~ÖÉãÉåí=ìåáíë=ïÜÉêÉ=~=ãìäíáÇáëÅáéäáå~êó=íÉ~ã=ïáíÜ=é~áå=åìêëÉëIéÜóëáçíÜÉê~éáëíëI=~åÇ=éëóÅÜçäçÖáëíë=Å~å=Ü~åÇäÉ=íÜÉ=ÇáÑÑÉêÉåí=~ëéÉÅíë=~åÇÅçåëÉèìÉåÅÉë=çÑ=ÅÜêçåáÅ=é~áå=áå=Ç~áäó=~ÅíáîáíáÉëK=


179


References

NK a~çìëá=Ì=j~Åc~êä~åÉ=fÎ=tççÇï~êÇ=Î=Éí=~äK=`ÜêçåáÅ=é~áåÑìä=éÉêáéÜÉê~ä=åÉìêçé~íÜó

áå=~å=ìêÄ~å=ÅçããìåáíóW=~=ÅçåíêçääÉÇ=Åçãé~êáëçå=çÑ=éÉçéäÉ=ïáíÜ=~åÇ=ïáíÜçìí=Çá~ÄÉíÉëK

aá~ÄÉí=jÉÇ OMMQX=ONW=VTSJUOK

OK p~áÇ=dI=_~ìÇçáå=aI=qçóççâ~=hK=^ñçå~ä=êÉÖÉåÉê~íáçåI=ãçíçê=ÇÉÑáÅáí=~åÇ=é~áåë=áå=ëÉîÉêÉ

äÉåÖíÜJÇÉéÉåÇÉåí=Çá~ÄÉíáÅ=éçäóåÉìêçé~íÜóK=g=kÉìêçä OMMUX=ORRW=NSVPJTMOK=


180

√ Typical trigeminal neuralgia occurs mostly after the age of 50 years. It is a

one-sided, shooting, lightning-like, unbearable attack of facial pain in the

territory of one of the three branches of the trigeminal nerve. There is no

neurological deficit. Trigeminal neuralgia responds well to treatment with

carbamazepine.

√ Post-herpetic pain is chronic and extremely disabling. The local application of

a lidocaine or capsaicin patch can help.

√ Length-dependent diabetic polyneuropathy is the most common cause of

painful neuropathy in the world.

√ Tricyclic antidepressants (amitriptyline and imipramine) and the mixed

serotonin and noradrenaline reuptake inhibitors (duloxetine and venlafaxine)

often work.

√ Many anticonvulsants (carbamazepine, gabapentin and pregabalin) are

indicated in chronic neuropathic pain.

√ Tramadol is an opioid with a monoaminergic reuptake inhibitory action which

may also relieve neuropathic pain.

√ In pain clinics a multidisciplinary team (including pain nurses,

physiotherapists, and psychologists) is often involved in the treatment of

neuropathic pain.

Ü Key points


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181



182


183

IndexA~ÅÅÉëëçêó=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=ufF==T~ÅèìáêÉÇ=áåÑä~ãã~íçêó=ÇÉãóÉäáå~íáîÉ=éçäóåÉìêçé~íÜó==NNQ~Åíáçå=éçíÉåíá~ä==NN~ÅìíÉ=~êÉÑäÉñáÅ=èì~Çêáé~êÉëáë==QP~ÅìíÉ=ãçíçê=~åÇ=ëÉåëçêó=~ñçå~ä=åÉìêçé~íÜó=E^jp^kF==QO~ÅìíÉ=ãçíçê=~ñçå~ä=åÉìêçé~íÜó=E^j^kF==QO~ÅìíÉ=éçäáçãóÉäáíáë==áñ^fap==VT~äÅçÜçäáÅ=éçäóåÉìêçé~íÜó==PPÑI=PQI=NTP~ääçÇóåá~==áñ^j^k=E~ÅìíÉ=ãçíçê=~ñçå~ä=åÉìêçé~íÜóF==QO^ãÉêáÅ~å=^Å~ÇÉãó=çÑ=kÉìêçäçÖó=^Ç=eçÅ=pìÄÅçããáííÉÉ==RS~ãáíêóéíóäáåÉ==NNTI=NTNI=NTS~ãéáÅáääáå==VP^jp^k=E~ÅìíÉ=ãçíçê=~åÇ=ëÉåëçêó=~ñçå~ä=åÉìêçé~íÜóF==QO~ãóäçáÇçëáë==OMI=NPMI=NRQ~ãóçíêçéÜó==áñI=UP~å~íçãó

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index:index.qxd 10/21/2014 1:13 PM Page 183

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184


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ÄÉåáÖå=ãçåçÅäçå~ä=Ö~ããçé~íÜó=~åÇ==NPMI=NPNÑÅäáåáÅ~ä=éêÉëÉåí~íáçåë==RRJSMÇá~ÖåçëíáÅ=íÉëíë==SNJSQÇáÑÑÉêÉåíá~ä=Çá~Öåçëáë==SQJSRÉéáÇÉãáçäçÖó==RRÜáëíçäçÖó==NTRÑçîÉêîáÉï==RRéêçÖåçëáë==SMJSNíêÉ~íãÉåí==SR

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Index

185


`js=ëÉÉ ÅóíçãÉÖ~äçîáêìëÅçÇÉáåÉ==NNTÅçåÇìÅíáçå=îÉäçÅáíó==NOJNPÅçååÉÅíáîÉ=íáëëìÉ=ÇáëçêÇÉêë==ñÅçêíá=çêÖ~å==ñÅçêíáÅçëíÉêçáÇë==PMI=SRI=TRI=UUI=NTOÅê~åá~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜó==NMVJNNMÅê~åá~ä=åÉêîÉë==RJTI=NSVJNTMòm=E`JêÉ~ÅíáîÉ=éêçíÉáåF==TRÅêóçÖäìÄìäáå~Éãá~==ñÅìíáë=ä~ñ~==ñÅóÅäçéÜçëéÜ~ãáÇÉ==SRÅóíçãÉÖ~äçîáêìë=E`jsF==ñI=STÑI=VRJVS

DÇ~Åíáäáíáë==ñaaf=EOIÛPÛJÇáÇÉçñóáåçëáåÉF==VTaÉàÉêáåÉJpçíí~ë=ëóåÇêçãÉ==NQTÇÉä~óÉÇJíóéÉ=ÜóéÉêëÉåëáíáîáíó=êÉ~Åíáçå==ñÇÉãóÉäáå~íáçå==QOJQPI=SOI=SPÑÇÉåÉêî~íáçå=ãìëÅäÉ=~íêçéÜó==ORI=ORÑÇÉêã~íçãÉ==ñÇá~ÄÉíáÅ=åÉìêçé~íÜó

~ìíçåçãáÅ==NMSJNMTI=NMUÑ`fam=áå==SMÅê~åá~ä==NMVJNNMÇáÑÑÉêÉåíá~ä=Çá~Öåçëáë==NNRappm==NMPJNMQI=NMRI=NMUÑI=NNTÉéáÇÉãáçäçÖó==NMOÑçÅ~äLãìäíáÑçÅ~ä==NMVI=NNOÑI=NNPI=NOOä~íÉJçåëÉí=ëÉåëçêó==NONiaam==PQI=NNTI=NOMI=NTOJNTQåçåJÇá~ÄÉíáÅ= åÉìêçé~íÜáÉë= ãçêÉ= Åçããçå= áå= Çá~ÄÉíáÅ= é~íáÉåíëNNPJNNQçîÉêîáÉï==NMNJNMOé~áåÑìä=ëóããÉíêáÅ~ä=éçäóåÉìêçé~íÜó==NMQJNMRé~íÜçäçÖó==NVI=PQI=NMTI=NMTÑI=NMUÑI=NNS


186


mak==NNMI=NNNÑI=NNOJNNPI=NOOI=NTOéêÉîÉåíáçå==NNSJNNTêÉå~ä=Ñ~áäìêÉ=~åÇ==NNQJNNRëÉåëçêó=~í~ñá~=~åÇ==NNUJNNVíÜçê~ÅáÅ==NNPíêÉ~íãÉåí==NNSJNOOíêçéÜáÅ=ÅÜ~åÖÉë==NMRìê~Éãá~=~åÇ==NOR

Çá~ÖåçëíáÅ=íÉëíë==PRJPVÇáÑäìåáë~ä==NSNÇáéÜÉåóäÜóÇ~åíçáå==NNTÇáëí~ä=ëóããÉíêáÅ~ä=~ñçå~ä=éçäóåÉìêçé~íÜó=Eáå=efsF==VRÇáëí~ä=ëóããÉíêáÅ~ä=ëÉåëçêó=éçäóåÉìêçé~íÜó=EappmF==NMPJNMQI=NMRI=NMUÑINNTÇáòòáåÉëë==PMJPNak^=íÉëíáåÖ==PUÇçãéÉêáÇçåÉ==NNTÇçñóÅóÅäáåÉ==VPappm=ëÉÉ Çáëí~ä=ëóããÉíêáÅ~ä=ëÉåëçêó=éçäóåÉìêçé~íÜóÇìäçñÉíáåÉ==NNTI=NTSI=NTUÇóáåÖJÄ~Åâ=~ñçå~ä=ÇÉÖÉåÉê~íáçå==NTJNVÑÇóë~ÉëíÜÉëá~==ñáÇóë~ìíçåçãá~==ñáI=QQI=QRI=NMRJNMSI=NMUÑÇóëÅê~ëá~==ñá

EÉäÉÅíêçéÜóëáçäçÖáÅ~ä=íÉëíáåÖ==PRJPSÉåÇçÅêáåçé~íÜó==NPNÉåÇçåÉìêáìã==ñáI=NMÑÉåòóãÉ=êÉéä~ÅÉãÉåí=íÜÉê~éó==NSOÉçëáåçéÜáäá~==ñáÉéáåÉìêáìã==ñáÉéáëÅäÉêáíáë==ñáÉêóíÜÉã~=ãáÖê~åë==ñáÉêóíÜêçÅóíÉ=ëÉÇáãÉåí~íáçå=ê~íÉ=EbpoF==TR

Index

187


Fc~ÄêóÛë=ÇáëÉ~ëÉ==NSNJNSOI=NSOÑÑ~Åá~ä=ÇáéäÉÖá~==QPÑ~Åá~ä=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=sfffF==SÑ~Åá~ä=é~äëó==UPÑ~ãáäá~ä=~ãóäçáÇ=éçäóåÉìêçé~íÜáÉë==NRQJNSNÑ~ëÅáÅìä~íáçå==OSÑ~íáÖìÉ==RMÑáÄêáää~íáçå==OSÑäìçêçÜóÇêçÅçêíáëçåÉ==NNTI=NOMÑçÅ~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜó==NNPÑçÅ~ä=åÉìêçé~íÜáÉë==PNJPOI=POÑI=RUI=NNP

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ÅäáåáÅ~ä=éêÉëÉåí~íáçå==QPJQRÇá~Öåçëáë==QRJQSáå=efs==VQJVRåÉìêçé~íÜáÅ=é~áå==NTQçîÉêîáÉï==QNJQOé~íÜçäçÖó==QSJQUI=QTÑI=QUÑI=QVÑé~íÜçéÜóëáçäçÖó==QOJQPéêçÖåçëáë==QUI=RMíêÉ~íãÉåí==RN

ÖÉäëçäáå=~ãóäçáÇçëáë==NSNÖÉåÉ=íÜÉê~éó==NSNÖÉåÉê~äáëÉÇ=åÉìêçé~íÜó==PPJPQI=PPÑÖá~åí=~ñçå~ä=åÉìêçé~íÜó==NSOJNSPI=NSPÑÖäçëëçéÜ~êóåÖÉ~ä=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=fuF==SÖäìÅçëÉ=íçäÉê~åÅÉ=íÉëí==ñáÖê~åìäçã~íçëáë=ïáíÜ=éçäó~åÖááíáë==ñáI=SVI=TOJTP

HÜ~ÉãçÇá~äóëáë==NOMI=NOQI=NORÜÉ~Ç~ÅÜÉI=éçëíJäìãÄ~ê=éìåÅíìêÉ==PU


188


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eÉêéÉë=òçëíÉê NTMJNTNefs==OMI=RSI=TQI=VQJVRekmm=EÜÉêÉÇáí~êó=åÉìêçé~íÜó=ïáíÜ=~=äá~Äáäáíó=íç=éêÉëëìêÉ=é~äëóF==NQSÑI=NQTepkLep^k=EÜÉêÉÇáí~êó=ëÉåëçêó=~åÇ=~ìíçåçãáÅ=åÉìêçé~íÜáÉëF

íóéÉ=f==NQVJNRMíóéÉ=ff==NRMJNRNI=NROÑíóéÉ=fff==NROJNRPíóéÉ=fs==NRPI=NRPÑ

Üìã~å=qJäóãéÜçíêçéáÅ=îáêìë=íóéÉ=f=EeqisJNF==OMI=TQI=VQÜóéÉê~ÉëíÜÉëá~==ñááÜóéÉê~äÖÉëá~==ñááÜóéÉêé~íÜá~==ñááÜóéçÖäçëë~ä=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=uffF==TÜóéçÖäóÅ~Éãá~==NMSÜóéçå~íêÉãá~==ñááÜóéçíçåá~==ñáá

IfÖd==NPMI=NPNfÖj==NPMI=NPNáã~ÖáåÖ==PSI=SOáãáéê~ãáåÉ==NNTI=NTSáããìåçÜáëíçÅÜÉãáëíêó==PTáåÇçãÉíÜ~Åáå==TR

Index

189


áåÑÉÅíáçìëLáåÑä~ãã~íçêó=åÉìêçé~íÜáÉë`js=åÉìêçé~íÜó==STÑI=VRJVSÇáëí~ä=ëóããÉíêáÅ~ä=~ñçå~ä=éçäóåÉìêçé~íÜó==VRáå=d_p==VQáå=efs==VQJVRäÉéêçëó==TUJUUióãÉ=ÇáëÉ~ëÉ==UUJVPã~äáÖå~åí=äóãéÜçã~ë==VTçîÉêîáÉï==TTëìÄ~ÅìíÉ=ãìäíáÑçÅ~ä=åÉìêçé~íÜó==VRíçñáÅ=åÉìêçé~íÜó=áå=^fap==VT

fåíÉêå~íáçå~ä=aá~ÄÉíÉë=cçìåÇ~íáçå==NMOáåíê~JÉéáÇÉêã~ä=åÉêîÉ=ÑáÄêÉ=~å~äóëáë==PSJPTáåíê~îÉåçìë=áããìåçÖäçÄìäáå=EfsfdF==ñááI=PMI=RNI=RSI=SRI=NNVáêáÇçÅóÅäáíáë==ñáááëÅÜ~ÉãáÅ=åÉìêçé~íÜó==TMáëçä~íÉÇ=åÉìêçé~íÜó==TPfsfd=ëÉÉ áåíê~îÉåçìë=áããìåçÖäçÄìäáåfñçÇÉë=ëééK==ñááI=UV

Lä~ÖçéíÜ~äãìë==ñááI=UPä~êÖÉ=ÑáÄêÉ=åÉìêçé~íÜó==PQI=NTPJNTQiaam=EäÉåÖíÜJÇÉéÉåÇÉåí=Çá~ÄÉíáÅ=éçäóåÉìêçé~íÜóF==PQI=NNTI=NOMI=NTOJNTQäÉéêçëó

ÅäáåáÅ~ä=ã~åáÑÉëí~íáçåë==UNJUTÅìí~åÉçìë=äÉëáçåë==UOÇá~Öåçëáë==PVI=UTJUUäÉéêçã~íçìë==UQJURãáÅêçÄáçäçÖó==UNãçíçê=ÇáëíìêÄ~åÅÉ==UPåÉÅêçíáëáåÖ=î~ëÅìäáíáë=~åÇ==TQçîÉêîáÉï==TUI=UMé~íÜçéÜóëáçäçÖó==TVÑI=UMêÉ~Åíáçå~ä=ëí~íÉë==USëÉåëçêó=äçëë==UOJUPíê~åëãáëëáçå==UN


190


íêçéÜáÅ=ìäÅÉêë==UQíìÄÉêÅìäçáÇ==URJUS

äÉìÅçéÉåá~==ñááäÉìâçÅóíçÅä~ëá~==ñááiÜÉêãáííÉÛë=éÜÉåçãÉåçå==ñááäáÇçÅ~áåÉ==NTTI=NTUäáÖÜíJÅÜ~áå=áããìåçÖäçÄìäáå==ñááäáîÉê=íê~åëéä~åí~íáçå==NSMJNSNäçïÉê=äáãÄ=åÉêîÉë==QJRäìãÄ~ê=éìåÅíìêÉ==PTJPUäìãÄçë~Åê~ä=éäÉñìë==PJQäìãÄçë~Åê~ä=ê~ÇáÅìäçéäÉñçé~íÜó==NPSäìåÖ=Å~åÅÉê==NPPJNPQI=NPRióãÉ=ÇáëÉ~ëÉ

ÅäáåáÅ~ä=ã~åáÑÉëí~íáçåë==UVJVOÇá~Öåçëáë==VPçîÉêîáÉï==UUJUVíêÉ~íãÉåí==VP

äóãéÜ~ÇÉåáíáë==ñááäóãéÜçÅóíÉë==ñáI=ñîáäóãéÜçÉÇÉã~==ñááäóãéÜçã~==VTJVU

Mã~ÅêçéÜ~ÖÉë==ñáááã~Åìä~É==ñáááã~ÖåÉíáÅ=êÉëçå~åÅÉ=áã~ÖáåÖ=EjofF==ñîáI=PSã~äÉ=áãéçíÉåÅÉ==PNI=NNTã~äáÖå~åÅó=ëÉÉ Å~åÅÉêã~äåìíêáíáçå==NTPãÉÇá~å=åÉêîÉ==PNjÉÇáÅ~ä=`çìåÅáä=oÉëÉ~êÅÜ=pÅ~äÉ=Ñçê=ãìëÅäÉ=éçïÉê==ORíãÉåáåÖáíáë==VMJVNI=VPãÉåáåÖçê~ÇáÅìäáíáë==ñáááãÉíÜóäéêÉÇåáëçäçåÉ==NNVãÉíçÅäçéê~ãáÇÉ==NNTjdrp=EãçåçÅäçå~ä=Ö~ããçé~íÜó=çÑ=ìåâåçïå=ëáÖåáÑáÅ~åÅÉF==SQ

Index

191


jáääÉêJcáëÜÉê=ëóåÇêçãÉ==QRãáíçÅÜçåÇêáçé~íÜáÉë==ñáááãçåçÅäçå~ä=Ö~ããçé~íÜó

ÇÉÑáåÉÇ==ñáááäáÖÜí=ÅÜ~áå=~ãóäçáÇçëáë=~åÇ==NPMjdrp==SQI=NOVåÉìêçé~íÜó=~åÇ==NPMJNPOçîÉêîáÉï==NOVJNPMmlbjp=ëóåÇêçãÉ==NPNJNPOI=NPOÑ

ãçåçåÉìêáíáë=ãìäíáéäÉñ==ñáááI=PVãçêéÜáåÉ==NTTãçêíçåÛë=åÉìêçã~==ñáááãçíçê=ã~åáÑÉëí~íáçåë

ÇÉÑáÅáíë==OPJORéçëáíáîÉ==OS

ãçíçê=åÉìêçåë==VãìÅçêãóÅçëáë==NNQãìäíáÄ~Åáää~êó=äÉéêçëó==UQJURãìäíáÑçÅ~ä=åÉìêçé~íÜáÉë==PNJPOI=POÑãìäíáåìÅäÉ~íÉÇ=Öá~åí=ÅÉääë==ñáááãìäíáéäÉ=ëÅäÉêçëáë==ñáááI=SMI=NTMãìëÅäÉ=Åê~ãéë==OSãìëÅäÉ=éçïÉê==OQI=ORíãìëÅäÉ=ëéáåÇäÉë==ñáááãìëÅäÉ=ï~ëíáåÖ==ORI=ORÑjóÅçÄ~ÅíÉêáìã=äÉéê~É TUJUUãóÅçéÜÉåçä~íÉ==SRãóÇêá~ëáë==ñáááãóÉäáå=ëÜÉ~íÜ==NMI=NNÑI=NOI=NSJNTãóÉäáåJ~ëëçÅá~íÉÇ=ÖäóÅçéêçíÉáå=Ej^dF==NOãóçâóãá~==OS

NåÉÅêçëáë==ñáááåÉÅêçíáëáåÖ=~êíÉêáíáë=ëÉÉ î~ëÅìäáíáÅ=åÉìêçé~íÜáÉëåÉêîÉ=Äáçéëó==PUJPVåÉêîÉ=ÜóéÉêíêçéÜó==NQPÑI=NQQJNQR


192


åÉìêáíáë==ñáááåÉìêçÑáÄêçã~==ñáááåÉìêçÖÉåáÅ=Ää~ÇÇÉê==PNI=NMSåÉìêçäóãéÜçã~íçëáë==ñáîåÉìêçé~íÜáÅ=çëíÉç~êíÜêçé~íÜó==NMRåÉìêçé~íÜáÅ=é~áå

Äê~ÅÜá~ä=éäÉñìë==NTNJNTOÅÜ~ê~ÅíÉêáëíáÅë==OVJPMI=NSTJNSUáå=ÖÉåÉê~äáëÉÇ=éçäóåÉìêçé~íÜó==NTOJNTRã~å~ÖÉãÉåí==NTRJNTVåçåJéÜ~êã~ÅçäçÖáÅ~ä=íêÉ~íãÉåíë==NTTJNTUçîÉêîáÉï==NSTçñ~äáéä~íáå=~åÇ==NTPáå=mak==NTOéÜ~êã~ÅçäçÖáÅ~ä=íêÉ~íãÉåíë==NTRJNTTmek==NTMJNTNéêçÖåçëáë==NTVê~ÇáÅìä~ê=é~áåë==NTNíÜ~äáÇçãáÇÉ=~åÇ==NTQíêáÖÉãáå~ä=åÉìê~äÖá~==NSVJNTM

åáÅçíáåáÅ=êÉÅÉéíçêë==ñáîåçÇÉë=çÑ=o~åîáÉê==ñáîI=NNI=NNÑåçåJäÉåÖíÜJÇÉéÉåÇÉåí=éçäóåÉìêçé~íÜó==PQåìÅäÉìë=éìäéçëìë==ñáî

OçÅíêÉçíáÇÉ==NNTçÅìäçãçíçê=åÉêîÉë=EÅê~åá~ä=åÉêîÉ=fffI=fs=~åÇ=sfF==RçåÅçåÉìê~ä=~åíáÄçÇáÉë==ñáîI=NPQJNPRçéÜíÜ~äãçéäÉÖá~==ñáîI=QPJQQI=QRçêÅÜáíáë==ñáîçêÖ~åçãÉÖ~äó==NPNçêíÜçëí~íáÅ=ÜóéçíÉåëáçå=ëÉÉ éçëíìê~ä=ÜóéçíÉåëáçåçëíÉç~êíÜêçé~íÜáÉë==ñáîI=OUJOVI=OVÑçëíÉçäóëáë==ñáîçëíÉçãóÉäáíáë==NMRçëíÉçéÜóíçëáë==ñáî

Index

193


çñ~äáéä~íáå==NPTI=NTPçñóÅçÇçåÉ==NTT

Pé~Åäáí~ñÉä==NPUé~áå=ëÉÉ åÉìêçé~íÜáÅ=é~áåé~áå=ëÉåë~íáçå=íÉëíáåÖ==OTé~áåÑìä=ëóããÉíêáÅ~ä=éçäóåÉìêçé~íÜó==NMQJNMRm^k=ëÉÉ éçäó~êíÉêáíáë=åçÇçë~m~åÅç~ëí=íìãçìêë==ñáîI=NPPé~åÇóë~ìíçåçãá~==ñáîé~éáääçÉÇÉã~==ñáîé~ê~ÅÉí~ãçä==NNTé~ê~ÉëíÜÉëá~==ñáîé~ê~äóëáë==OQé~ê~åÉçéä~ëíáÅ=åÉìêçé~íÜáÉë==TQJTRI=NPQJNPRé~ê~ëóãé~íÜÉíáÅ=åÉêîçìë=ëóëíÉã==TJUmak=Eéêçñáã~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜóF==NNMI=NNNÑI=NNOJNNPI=NOOI=NTOéÉåáÅáääáå==VPéÉêáÅ~êÇáíáë==ñáîéÉêáåÉìêáìã==ñáîéÉêáéÜÉê~ä=åÉêîÉëI=ãáÅêçëÅçéáÅ=~å~íçãó==UJVI=VÑI=NPÑéÉêáéÜÉê~ä=åÉìêçé~íÜóI=ÇÉÑáåÉÇ==ñáîéÉêáî~ëÅìä~ê=ÅìÑÑáåÖ==ñáîéÉë=Å~îìë==NQOI=NQRmdm=EéêçíÉáå=ÖÉåÉ=éêçÇìÅíF==PSéÜÉåóíçáå==NTMmek=EéçëíJÜÉêéÉíáÅ=åÉìê~äÖá~F==NTMJNTNmÜóÅçãóÅÉí~=ëééK NNQéä~åí~ê=ìäÅÉêë==OUI=OUÑI=UQI=NMRéä~ëã~=ÅÉää=äÉìâ~Éãá~==NPPÑéä~ëã~=ÉñÅÜ~åÖÉ==RNI=SRéä~ëã~Åóíçã~ë==ñîéä~ëãáÇë==ñîéä~íáåìã=ÅçãéçìåÇë==NPTéäÉçÅóíçëáë==ñî


194


mlbjp= ëóåÇêçãÉ= EéçäóåÉìêçé~íÜóI= çêÖ~åçãÉÖ~äóI= ÉåÇçÅêáåçé~íÜóIãçåçÅäçå~ä=Ö~ããçé~íÜóI=ëâáå=ÅÜ~åÖÉëF==SQI=NPNJNPOI=NPOÑéçáâáäçÇÉêãá~==ñîéçäó~êíÉêáíáë=åçÇçë~=Em^kF

`ÜìêÖJpíê~ìëë=ëóåÇêçãÉ==TNÇÉÑáåÉÇ==ñîÇá~Öåçëáë==STÑI=SUçîÉêîáÉï==TMJTN

éçäóÅóíÜ~Éãá~==ñîéçäóåÉìêçé~íÜáÉë==PPJPQI=PPÑéçëíJÜÉêéÉíáÅ=åÉìê~äÖá~=EmekF==NTMJNTNéçëíìê~ä=ÜóéçíÉåëáçå==PMI=NNTI=NOMI=NTSéêÉÇåáëçäçåÉ==SRI=TRI=NNVéêÉÖ~Ä~äáå==NNTI=NTTI=NTUéêáã~êó=î~ëÅìäáíáë==SUJSVéêçéêáçÅÉéíáçå==ñîéêçñáã~ä=Çá~ÄÉíáÅ=åÉìêçé~íÜó=EmakF==NNMI=NNNÑI=NNOJNNPI=NOOI=NTOéëÉìÇç~íÜÉíçíáÅ=ãçîÉãÉåíë==ñîéíçëáë==ñî

Rê~Çá~ä=åÉêîÉ==PNê~Çá~íáçåJáåÇìÅÉÇ=Äê~ÅÜá~ä=éäÉñçé~íÜó==NPSê~ÇáÅìä~ê=é~áåë==NTNêÉÅÉëëáîÉ=ÇÉãóÉäáå~íáåÖ=åÉìêçé~íÜó==NQTJNQUêÉå~ä=Ñ~áäìêÉ==NNQJNNRI=NOPJNORêÉå~ä=íê~åëéä~åí~íáçå==NORêÉëéáê~íçêó=Ñ~áäìêÉ==OQI=QQêÉëíäÉëë=äÉÖ=ëóåÇêçãÉ==NOPêÉíáåáíáë==ñîêÜÉìã~íçáÇ=~êíÜêáíáë==ñîI=TNJTOêáÄçëçãÉë==ñîêáÑ~ãéáÅáå==UUoáäÉóJa~ó=ëóåÇêçãÉ==NROJNRPoçãÄÉêÖÛë=ëáÖå==ñî

Index

195


Së~äí~íçêó=ÅçåÇìÅíáçå==NNë~êÅçáÇçëáë==ñîI=TQpÅÜãáÇíJi~åíÉêã~å=áåÅáëìêÉë==ñîpÅÜï~åå=ÅÉääë==ñîáI=NMJNNI=NNÑI=NPÑI=OMëÅÜï~ååçã~ë==ñîáëÅäÉêçíáÅ=ÄçåÉ=äÉëáçåë==NPNI=NPOÑëÉÅçåÇ~êó=î~ëÅìäáíáë==SVI=TQëÉÖãÉåí~ä=ÇÉÖÉåÉê~íáçå==NVëÉÖãÉåí~ä=ãóçÅäçåìë==OSëÉåëçêó=ã~åáÑÉëí~íáçåë

ëÉåëçêó=äçëë==OTJOVI=UOJUPëéçåí~åÉçìë=é~áåë==OVJPM=ëÉÉ ~äëç=åÉìêçé~íÜáÅ=é~áå

ëÉåëçêó=åÉêîÉ=~Åíáçå=éçíÉåíá~ä=Epk^mF==PSëÉåëçêó=åÉìêçåë==VJNMI=NMÑëÉêçíçåáå=~åÇ=åçê~ÇêÉå~äáåÉ=êÉìéí~âÉ=áåÜáÄáíçêë=EpkofëF==NTSI=NTUëâÉäÉí~ä=Ñççí=ÇÉÑçêãáíáÉë==NQOI=NQPÑëâáå=Äáçéëó==PSJPTI=TMëâáå=ÅÜ~åÖÉë==NPNëã~ää=ÑáÄêÉ=åÉìêçé~íÜó==PQI=NTOJNTPëé~ëíáÅ=é~ê~é~êÉëáë==ñîáëéáå~ä=ÅçêÇ=ëíáãìä~íáçå==NTUëéáêçÅÜ~ÉíÉë==ñîáëéçåí~åÉçìë=é~áåë==OVJPM=ëÉÉ ~äëç åÉìêçé~íÜáÅ=é~áåëìÄ~ÅìíÉ=ãìäíáÑçÅ~ä=åÉìêçé~íÜó==VRëìÄ~ÅìíÉ=ëÉåëçêó=åÉìêçåçé~íÜó=Eé~ê~åÉçéä~ëíáÅF==NPQJNPRëóãé~íÜÉíáÅ=åÉêîçìë=ëóëíÉã==UëóåÅçéÉ==PMJPNI=NMSëóêáåÖçãóÉäá~==ñîáI=OVëóëíÉãáÅ=äìéìë=ÉêóíÜÉã~íçëìë=EpibF==ñîá

TqOJïÉáÖÜíÉÇ=áã~ÖÉë==ñîáí~Ñ~ãáÇáë==NSNí~ñ~åÉë==NPUíÉíê~ÅóÅäáåÉ==VPíÜ~äáÇçãáÇÉ==NPU


196


íÜçê~ÅáÅ=åÉêîÉë==PíÜçê~ÅáÅ=åÉìêçé~íÜó==NNPíÜêçãÄçÅóíçëáë==ñîáíáÅâë==ñááI=UVíçñáÅ=åÉìêçé~íÜó==VTI=NPSJNPUíê~ã~Ççä==NTTíê~åëíÜóêÉíáåJÑ~ãáäá~ä=~ãóäçáÇ=éçäóåÉìêçé~íÜó=EqqoJc^mF

ÅäáåáÅ~ä=éêÉëÉåí~íáçåë==NRQJNRUÇá~Öåçëáë==NRUJNRVI=NRVÑÖÉåÉíáÅë==NRVJNSMíêÉ~íãÉåí==NSMJNSN

íê~åëîÉêëÉ=ãóÉäáíáë==ñîáíê~ìã~==NSíêÉãçê==OQI=OSI=SMíêáÅóÅäáÅ=~åíáÇÉéêÉëë~åíë==NNTI=NTSI=NTUíêáÖÉãáå~ä=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=sF==RJSíêáÖÉãáå~ä=åÉìê~äÖá~==NSVJNTMíêçéÜáÅ=ìäÅÉêë==OUI=OUÑI=UQI=NMRíêçéáëã==ñîáqqoJc^m=ëÉÉ íê~åëíÜóêÉíáåJÑ~ãáäá~ä=~ãóäçáÇ=éçäóåÉìêçé~íÜóíìÄÉêÅìäçáÇ=äÉéêçëó==URJUSíóéÉ=NLíóéÉ=O=Çá~ÄÉíÉë=ãÉääáíìë==ñîáJñîáá=ëÉÉ=~äëç Çá~ÄÉíáÅ=åÉìêçé~íÜóíóéÉ=fs=ÜóéÉêëÉåëáíáîáíó=êÉ~Åíáçå==ñ

Uìäíê~ëçìåÇ=áã~ÖáåÖ==PSìééÉê=äáãÄ=åÉêîÉë==OJPìê~ÉãáÅ=åÉìêçé~íÜó==NNQJNNRI=NOPJNORìîÉáíáë==ñîáá

Vî~Öìë=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=uF==Tî~ëÅìä~ê=ÉåÇçíÜÉäá~ä=ÖêçïíÜ=Ñ~Åíçê=EsbdcF==ñîááI=SQI=NPOî~ëÅìäáíáÅ=åÉìêçé~íÜáÉë

Çá~Öåçëáë==TMåÉÅêçíáëáåÖ=î~ëÅìäáíáë==SVJTNåÉìêçé~íÜáÅ=é~áå==NTQI=NTR

Index

197


áå=çäÇÉê=éÉçéäÉ==TPm^k==SUI=SUÑI=TMJTNé~íÜçéÜóëáçäçÖó==STJSVI=TMêÜÉìã~íçáÇ=~êíÜêáíáë=~åÇ==TNJTOíêÉ~íãÉåí==TRîáê~ä=áåÑÉÅíáçå=~åÇ==TQ

î~ëÅìäáíáë==ñîááI=OMI=PVI=STJSVîÉåä~Ñ~ñáåÉ==NTSîÉëíáÄìäçÅçÅÜäÉ~ê=åÉêîÉ=EÅê~åá~ä=åÉêîÉ=sfffF==SîáåÅ~=~äâ~äçáÇë==NPTîçäí~ÖÉJÖ~íÉÇ=ÅÜ~ååÉäë==ñîáá

Wt~ääÉêá~å=ÇÉÖÉåÉê~íáçå==NSJNTïÉ~âåÉëë==OQI=QPI=QQ


198


tfm

Written by one of the world's leading experts on neuropathy, Professor Gérard Said, this

book is a ‘must read’ and also a handy reference book for doctors, nurses, physiotherapists,

chiropodists/podiatrists, other health professionals, and, importantly, for patients who wish

to be more informed.

As well as covering the anatomy of the nervous system and the basic pathological processes

that may affect the peripheral nerves, this book covers a whole range of neuropathic

conditions. These include, for example, Guillain‐Barré syndrome, chronic inflammatory

demyelinating polyneuropathy, vasculitic neuropathies, infectious neuropathies, diabetic and

other metabolic neuropathies, hereditary neuropathies and neuropathies in patients with

cancer.

Given the almost explosive increase in diabetes predicted over the coming years and the

high incidence of HIV infections alone, not to mention all the other possible causes of

peripheral neuropathy, no self‐respecting medical unit should be without a copy of this new

book on their shelves.

Peripheral Neuropathy & N

europathic Pain — Into the Light


Gérard Said


The author, Professor Gérard Said, is based in the Department of Neurology atthe prestigious Hôpital de la Salpêtrière in Paris. He has devoted a lifetime tothe study of peripheral neuropathy and — alongside other great neurologicalnames — added much to the world's ever‐growing store of knowledge on thiscomplex but fascinating condition which affects so many individuals.

Into the Light

PN cover.qxd 04/08/2014 14:40 Page 1

Documents

Peripheral Neuropathy and Neuropathic Pain Into The Light