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SATURDAY FORUM SATURDAY FORUM 6 6 th th AUGUEST 2005 AUGUEST 2005 A .S HENNAYAKE A .S HENNAYAKE

Guillain –barre syndrome

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Page 1: Guillain –barre syndrome

SATURDAY FORUM SATURDAY FORUM 66thth AUGUEST 2005 AUGUEST 2005

A .S HENNAYAKEA .S HENNAYAKE

Page 2: Guillain –barre syndrome

PC PC Facial Numbness 1 dayFacial Numbness 1 dayNumbness of both UL & LL Numbness of both UL & LL HPCHPCHe He waswas Well 10 days back and Developed fever Well 10 days back and Developed fever

which was associated with chills and subsided 5 which was associated with chills and subsided 5 days back .He was free of fever for Next 5 days days back .He was free of fever for Next 5 days except for a Non productive coughexcept for a Non productive cough

Then he Developed numbness of both LL & UL . Then he Developed numbness of both LL & UL . And developed weakness of both LL & ascended And developed weakness of both LL & ascended both UL , Meanwhile he noticed BL facial both UL , Meanwhile he noticed BL facial numbness & weakness.numbness & weakness.

He had Immunization for poliomyelitis ,No Hx of He had Immunization for poliomyelitis ,No Hx of food poisoning . Bowel habits & Urinary habits food poisoning . Bowel habits & Urinary habits normal normal

Page 3: Guillain –barre syndrome

PMH-No Hx of similar illness , DM or PMH-No Hx of similar illness , DM or Connective tissue diseaseConnective tissue disease

FH-No Hx of similar illness , autoimmune FH-No Hx of similar illness , autoimmune disease , , connective tissue disease,DM disease , , connective tissue disease,DM -Father-Father

DH - NADDH - NAD

SH – School boy, living with Mother & Father SH – School boy, living with Mother & Father .no social problems..no social problems.

Page 4: Guillain –barre syndrome

SummerySummery

14 year old school boy presented with 14 year old school boy presented with Numbness & Weakness of Both UL & LL Numbness & Weakness of Both UL & LL & Facial Weakness following a febrile & Facial Weakness following a febrile Illness. Bowel habits & Urinary habits Illness. Bowel habits & Urinary habits Normal .Father is having DM.Normal .Father is having DM.

FH of DM ,Immunization for poliomyelitis FH of DM ,Immunization for poliomyelitis donedone

Page 5: Guillain –barre syndrome

OE – ObeseOE – Obese

AfebrileAfebrile

CVSCVS

PR - 80 minPR - 80 min

BP – 130 /70 mmhgBP – 130 /70 mmhg

Ht S 1 ,S2 NormalHt S 1 ,S2 Normal

RSRS

RR -35 min RR -35 min

Chest movements symmetricalChest movements symmetrical

Breath holding counting 30 Breath holding counting 30

VB presentVB present

Page 6: Guillain –barre syndrome

MotorMotor

Power BL UL Grade 4Power BL UL Grade 4

BL LL Grade 2BL LL Grade 2

Tone BL UL reducedTone BL UL reduced

BL LL reducedBL LL reduced

Reflexes BL UL ReducedReflexes BL UL Reduced

BL LL absentBL LL absent

Plantar BL down Plantar BL down

Joint position sense absentJoint position sense absent

Gait- difficult to check Gait- difficult to check

Page 7: Guillain –barre syndrome

ABD- Soft ABD- Soft Liver ,spleen not palpableLiver ,spleen not palpable Kidneys not BalatableKidneys not Balatable NO FF ,BS+NO FF ,BS+CNS-Conscious ,RationalCNS-Conscious ,Rational BL facial weakness +BL facial weakness + No Neck stiffnessNo Neck stiffness Fundi –NormalFundi –Normal No cerebellar signsNo cerebellar signs No Sensory Loss.No Sensory Loss.

Page 8: Guillain –barre syndrome

IxIx

Hb 10 . 3 gdlHb 10 . 3 gdl Pcv 31 %Pcv 31 % WBC 11 x10 9WBC 11 x10 9

N 75 %N 75 %

L 21 %L 21 %

E 2%E 2%

M 2%SSSM 2%SSS

Page 9: Guillain –barre syndrome

SummerySummery14 year old school boy presented with 14 year old school boy presented with

Numbness & Weakness of Both UL & LL Numbness & Weakness of Both UL & LL & Facial Weakness following a febrile & Facial Weakness following a febrile Illness. Bowel habits & Urinary habits Illness. Bowel habits & Urinary habits Normal .Father is having DM.Normal .Father is having DM.

FH of DM ,Immunization for poliomyelitis FH of DM ,Immunization for poliomyelitis donedone

OE –Obese, BL facial weakness and flaccid OE –Obese, BL facial weakness and flaccid paralysis of both Upper & Lower limbs paralysis of both Upper & Lower limbs with absent reflexes and joint position with absent reflexes and joint position sensessenses

Page 10: Guillain –barre syndrome

ESR 10 mmhESR 10 mmh

UFR- NADUFR- NAD

BU- 4.2 mmolBU- 4.2 mmol

SE –Na 144meqlSE –Na 144meql

K 4.5 meqlK 4.5 meql

S. Protein -6 gdlS. Protein -6 gdl

A -3.9gdlA -3.9gdl

G -2.1gdlG -2.1gdl

CXR - NADCXR - NAD

ECG -NAD ECG -NAD

Page 11: Guillain –barre syndrome

RBS-100mgdlRBS-100mgdl

CSF CSF

Sugar 77.7gdlSugar 77.7gdl

Protein 160 mg dlProtein 160 mg dl

Cells L 5Cells L 5

RBC 3 RBC 3

Nerve conduction -Nerve conduction -

Page 12: Guillain –barre syndrome

GUILLAIN –BARRE SYNDROMEGUILLAIN –BARRE SYNDROME

Incidence 8.5 cases per millionIncidence 8.5 cases per million 3/10000 per y3/10000 per y Monophasic illness Monophasic illness Demyelinating neuropathy ,Rarely axonal Demyelinating neuropathy ,Rarely axonal Does not recurDoes not recur Probably has auto allergic process to myelin Probably has auto allergic process to myelin

sheathssheaths

Page 13: Guillain –barre syndrome

Usually follows Upper respiratory tract Usually follows Upper respiratory tract infections ,Entero virus ,EB virus infections ,Entero virus ,EB virus Camphylobactor jejuni ,Mycoplasma ,CMV , Camphylobactor jejuni ,Mycoplasma ,CMV , HIV HIV

Clinical featuresClinical features Ascending paralysis lasting for few days to Ascending paralysis lasting for few days to

4 weeks ( subacute 8 weeks)4 weeks ( subacute 8 weeks)Can present with lumbar or spinal painCan present with lumbar or spinal painMotor paralysis distal , proximal or Motor paralysis distal , proximal or

Respiratory musclesRespiratory musclesSome sensory ,tactile , vibratory sensation Some sensory ,tactile , vibratory sensation

and postural sensibility and postural sensibility

Page 14: Guillain –barre syndrome

Cranial nerves can affect ( facial)Cranial nerves can affect ( facial)

Bulbor involvement can occur Bulbor involvement can occur

Can present as complete Locked in stateCan present as complete Locked in state

Autonomic symptoms like atonic Bladder Autonomic symptoms like atonic Bladder ,illeus Hypertension , orthostatic ,illeus Hypertension , orthostatic HypotensionHypotension

Papilloedema ( CMV)Papilloedema ( CMV)

Combination of external opthalmoplagia Combination of external opthalmoplagia ,ataxia & tendon areflexia ( Miller Fisher ,ataxia & tendon areflexia ( Miller Fisher syndrome )syndrome )

Page 15: Guillain –barre syndrome

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Histology –Demeyelination maximal spinal Histology –Demeyelination maximal spinal roots , peripheral nerves roots , peripheral nerves

CSF-Protein elevated , no pleocytosisCSF-Protein elevated , no pleocytosis

Electrophysiological changes ( marked slowing Electrophysiological changes ( marked slowing of conduction velocities ,delayed later of conduction velocities ,delayed later responses ,prolong distal latencies ,dispersion responses ,prolong distal latencies ,dispersion of evoked responses and evidence of of evoked responses and evidence of conduction block)conduction block)

Page 16: Guillain –barre syndrome

Diagnosis Diagnosis

Clinical groundsClinical grounds

Confirmed by nerve conduction studies and CSFConfirmed by nerve conduction studies and CSF

Course and managementCourse and management

Paralysis progress rapidlyParalysis progress rapidly

May require ventilatory supportMay require ventilatory support

Subcutaneous Heparin ( venous thrombosis)Subcutaneous Heparin ( venous thrombosis)

High dose gamma globulin (IGA deficiency should High dose gamma globulin (IGA deficiency should be checked )be checked )

Plasmapheresis Plasmapheresis

Corticosteroids no valueCorticosteroids no value

Page 17: Guillain –barre syndrome

Spontaneous recovery begins in few days Spontaneous recovery begins in few days to 3 weeks from the onset with without to 3 weeks from the onset with without treatmenttreatment

Prolong ventilation may be necessaryProlong ventilation may be necessary

Improve over few months Improve over few months

Improvement may be complete or Improvement may be complete or incompleteincomplete

Page 18: Guillain –barre syndrome

DDDD Poliomyelitis Poliomyelitis BotulinismBotulinism Cord compressionCord compression Primary muscle diseasePrimary muscle disease Transverse MayelitisTransverse Mayelitis