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3/17/16 1 GuillainBarré Syndrome de Angelis, Fahey & P agan BIONB 4910 March 16,2016 GuillainBarrè Syndrome—Overview & Symptoms Rare d iso rd er: 6 -4 0 in 1 millio n Bo d y ’s immu ne sy stem attack s Perip heral N ervo us System (PN S) U n k n o wn cause So metimes trig g ered b y an in fectio n , su rgery or vaccin atio n Symptoms: Weak n ess an d tin glin g in th e extremities Mu scle w eak n ess D ifficu lty w ith facial mo v emen ts Sev ere p ain H eart rate an d b lo o d pressure prob lems D ifficu lty b reath in g Sev ere cases: w h o le b o dy paraly sis Mo st p eo p le reco v er Sy mp to ms p eak at 2 -4 week s, th en p lateau s Recovery (weeks to years) So me ex p erien ce lin g ering effects (Ex : w eakness, n umbn ess, fatig ue) Ref er ences : Gui l ai n Bar r e Syndr om e ( 2016) . Mayo Cl i ni c. ht t p: //www. m ayocl i ni c.org/d i seasesco ndi t i o ns/gu i ll ai n bar r e s yndr om e/ bas i c s / s ym pt om s / c on 20 0258 32. Accessed 15 March 2016 Gui l ai n Bar r e Syndr om e ( 2013) . KAA Ar abi c Heal th Encycl opedi a. ht t ps : / / www. kaahe. or g/ heal t h/ en/ 530 g ui l ai n b ar r e s yndr om e. ht m l . Accessed 15 March 2016 Hughes , R. A. C. , Hadden, R. D. M . , Gr egson, N. A. and Sm i t h, K. J . ( 2000) . Pat hogenes i s Of Gui l ai n Bar r e Syndr om e. J our nal of t he Per i pher al Ner vous System, 5: 122–123. doi : 10. 1046/ j . 1529 8027. 2000. abs j u n 23. x Van Door n PA, Rut s L , J ac obs BC. ( 2008) . Cl i ni cal f eat ur es , pat hogenes i s, and t r eat m ent of Gui l ai n Bar r é s yndr om e. Lancet Neur ol ., 7( 10) : 939 50. Hughes , R. A C, Cor nbl at h, D. R. ( 2005) . Gui l ai n Bar r è Syndr om e. The Lancet Nov 5 Nov 11, 366, 9497, pg. 1653 GuillainBarrè Syndrome Pathophys iology of Guillain-Barré - Acute Inflammatory Demyelinating Polyradiculoneuropathy(AIDP) (M os t common) - Acute Motor Axonal Neuropathy (AMAN) - Acute Motor and Sensory Axonal Neuropathy (AMSAN) AIDP: due to macrophages penetrating myelin sheaths in the peripheral nervous system and “stripping” neurons bare. Location of inflammation correlates with motor deficits. Hyp: activated T-cells target macrophages to myelin sheaths. Activated macrophages release free radicals etc. AMAN-AMSAN: macrophages invade Nodes of Ranvier, leaving myelin wraps intact, but blocking conduction. Causes - About 2/3 of patient s have symptoms of infections before onset of weakness.Hypothesis i s that GBS caused by infection-induced immune response that becomes aberrant and resultsin damaging of PNS. Early stages very unpredictable. - Common precipit ating factors: C. jejuni and cytomegalovirus mos t correlated with GBS (1/1000), als o Eps tein-Barr virus , M. pneumoniae and other infections . Findings supporting theory: - Anti-ganglios ide antibodies + other antibodies agains t other glycolipids, T-cells for peripheral nerve proteins . - Manipulations of ganglioside antibodies can induce disease in rabbits. GuillainBarrè Syndrome: Treatment REFERENCES: Gui l i an Bar r e Syndr om e Fact Sheet ( 2015) . NI H: Nat i onal I ns t i t ut e of Neur ol ogi cal Di s or der s and Stroke. ht t p: //www. ni nds . ni h. gov/ di s orders/gbs/ detai l _g bs . ht m. Accessed 15 March 2016. Diagnosis: Lumbar puncture, Electromyography No known c ure 2 Treatment types : Intravenous immunoglobin or plasmapheresis Equally effective, but not additive Becomes ineffective 2 weeks after ons et of s ymptoms Treatment of potential complications Medicine for blood clots (Anticoagulants) Pain Control Potentially fatal breathing problems ht t p: / / t exas onc ol ogysanant oni o. c om / t r eat ment s servi ces/servi ces/pl as m apher es i s 1/ ht t p: //www. ecpl az a. net / pr oduc t / hum an i m m unogl obul i n ph4 f or i nt r avenous 200753 1245755. ht ml ht t p: //130. 237. 83. 53/medi cal devi ces/al bum/ C h%206%20M eas ur em ent %20m et hods %20&% 20val ues/sl i des / F%206 20%20El ec t r om yogr aphy. ht m l

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Page 1: Guillain-BarrédeAngelis,Fahey,Pagan (Read-Only) · 2016-03-17 · 3/17/16 1 Guillain’Barré Syndrome deAngelis,( Fahey( &( Pagan BIONB4910 March(16,(2016 Guillain ’Barrè Syndrome

3/17/16

1

Guillain-­‐Barré Syndrome

de  Angelis,   Fahey   &   PaganBIONB  4910

March  16,  2016

Guillain-­‐Barrè Syndrome—Overview  &  Symptoms

• Rare disorder: 6-40 in 1 million

• Body’s immune system attacks Peripheral Nervous System (PNS)• Unknown cause• Sometimes triggered by an infection, surgery or vaccination

• Symptoms:• Weakness and tingling in the extremities• Muscle weakness• Difficulty with facial movements• Severe pain• Heart rate and blood pressure problems• Difficulty breathing• Severe cases: whole body paralysis• Most people recover

• Symptoms peak at 2-4 weeks, then plateaus• Recovery (weeks to years)• Some experience lingering effects (Ex: weakness, numbness, fatigue)

Ref er ences :-­‐ Guillain-­‐ Bar r e   Syndr om e   ( 2016) .   Mayo   Clinic.   ht t p: / /www. m ayoclinic. or g/d iseases-­‐ co ndit io ns/gu il lai n-­‐ bar r e-­‐ syndr om e/bas ics/sym pt om s/c on-­‐ 20 0258 32.   Accessed   15   Mar ch   2016-­‐ Guillain-­‐ Bar r e   Syndr om e   ( 2013) .   KAA   Ar abic   Healt h   Encyclopedia.   ht t ps : / /www. kaahe. or g/healt h/en/530-­‐ g ui ll ain-­‐ b ar r e-­‐ syndr om e. htm l.   Accessed   15   Mar ch   2016

-­‐ Hughes ,   R. A. C. ,   Hadden,   R. D. M . ,   Gr egson,   N. A.   and   Sm it h,   K. J .  ( 2000) .   Pat hogenes is   Of   Guillain-­‐ Bar r e   Syndr om e.   J our nal   of  t he   Per ipher al   Ner vous   Syst em ,   5: 122–123.   doi: 10. 1046/ j. 1529-­‐ 8027. 2000. abs ju n-­‐ 23. x-­‐ Van   Door n PA,   Rut s   L,   J acobs   BC.   ( 2008) .  Clinical   f eat ur es ,   pat hogenes is ,   and   t r eatm ent   of  Guillain-­‐ Bar r é syndr om e.   Lancet   Neur ol. ,   7( 10) : 939-­‐ 50.-­‐ Hughes ,   R. A   C,Cor nblat h,   D. R.   ( 2005) .  Guillain-­‐ Bar r è Syndr om e.   The   Lancet   Nov   5-­‐ Nov   11,   366,   9497,   pg.   1653

Guillain-­‐Barrè SyndromePathophys iology of Guillain-Barré

- Acute Inflammatory Demyelinating Polyradiculoneuropathy(AIDP) (Most common)- Acute Motor Axonal Neuropathy (AMAN)- Acute Motor and Sensory Axonal Neuropathy (AMSAN)

AIDP: due to macrophages penetrating myelin sheaths in the peripheral nervous sys tem and “s tripping” neurons bare. Location of inflammation correlates with motor deficits . Hyp: activated T-cells target macrophages to myelin sheaths . Activated macrophages release free radicals etc.

AMAN-AMSAN: macrophages invade Nodes of Ranvier, leaving myelin wraps intact, but blocking conduction.

Causes- About 2/3 of patients have symptoms of infections before onset of weakness .Hypothes is is that GBS caused by infection-induced immune response that

becomes aberrant and results in damaging of PNS. Early stages very unpredictable.- Common precipitating factors : C. jejuni and cytomegalovirus most correlated with GBS (1/1000), also Eps tein-Barr virus , M. pneumoniae and other

infections .

Findings supporting theory: - Anti-ganglios ide antibodies + other antibodies agains t other glycolipids, T-cells for peripheral nerve proteins . - Manipulations of ganglioside antibodies can induce disease in rabbits .

Guillain-­‐Barrè Syndrome:  Treatment

REFERENCES:  • Guillian-­‐ Bar r e Syndr om e   Fact   Sheet   ( 2015) .  NI H:   Nat ional   I nst it ut e   of  Neur ological   Disor der s   and   St r oke.   ht t p: / /www. ninds . nih. gov/dis or der s/gbs/ det ai l_g bs . htm .   Accessed   15   Mar ch   2016.  

• Diagnos is :   Lumbar   puncture,   Electromyography  

• No  known   cure

• 2  Treatment   types :   Intravenous   immunoglobin or   plasmapheres is• Equally   effective,   but   not   additive  • Becomes   ineffective   2  weeks  after   onset   of  symptoms  

• Treatment   of   potential   complications  • Medicine   for   blood   clots  (Anticoagulants )• Pain   Control• Potentially   fatal   breathing   problems  

ht t p: // t exasoncologysanant onio. com /t reatments -­‐ser vices/ser vices/plasm apher es is -­‐ 1/

ht t p: / /www. ecplaza. net /pr oduct /hum an-­‐ imm unoglobulin-­‐ ph4-­‐f or -­‐ int r avenous-­‐ -­‐ 200753-­‐ 1245755. html

ht t p: / /130. 237. 83. 53/medicaldevices/album/Ch%206%20Measur em ent%20m et hods%20&%20values/s lides/F%206-­‐20%20Elect r om yogr aphy. htm l