Guillain-Barr syndrome

Gullain-Barr syndrome (GBS) (in French pronounced [il bae]

[1]
, in English
pronounced / gi ln b re/
[2]
, /gi n b re /,
[3]
etc.
[4]
) is an acue, auoi!!une,
pol"radiculoneuropah" a##ecing he peripheral ner$ous s"se!, usuall" riggered b"
an acue in#ecious process% & is included in he 'ider group o# peripheral
neuropahies% (here are se$eral "pes o# )*+, bu unless oher'ise saed, )*+ re#ers
o he !os co!!on #or!, acue in#la!!aor" de!"elinaing pol"neuropah"
(,&-.)% & is #re/uenl" se$ere and usuall" e0hibis as an ascending paral"sis noed b"
'ea1ness in he legs ha spreads o he upper li!bs and he #ace along 'ih co!plee
loss o# deep endon re#le0es% 2ih pro!p rea!en o# plas!apheresis #ollo'ed b"
i!!unoglobulins and suppori$e care, he !a3ori" o# paiens 'ill regain #ull
#uncional capaci"% 4o'e$er, deah !a" occur i# se$ere pul!onar" co!plicaions
and d"sauono!ia are presen%
P A T HOP HY S I O L O G Y
,ll #or!s o# )uillain5*arr6 s"ndro!e are due o an i!!une response o #oreign
anigens (such as in#ecious agens or $accines) bu !isargeed o hos ner$e issues
insead (a #or! o# anigenic !i!icr")% (he arges o# such i!!une aac1 are hough
o be gangliosides, 'hich are co!ple0 gl"cosphingolipids presen in large /uaniies
on hu!an ner$e issues, especiall" in he nodes o# 7an$ier% ,n e0a!ple is he )81
ganglioside, 'hich can be a##eced in as !an" as 295:9; o# cases, especiall" in hose
preceded b" Campylobacter jejuni in#ecions% ,noher e0a!ple is he )<1b
ganglioside, 'hich is he arge in he 8iller Fisher s"ndro!e $arian (see belo')%
(he end resul o# such auoi!!une aac1 on he peripheral ner$es is in#la!!aion o#
!"elin and conducion bloc1, leading o a !uscle paral"sis ha !a" be acco!panied
b" sensor" or auono!ic disurbances%
4o'e$er, in !ild cases, a0onal #uncion re!ains inac and reco$er" can be rapid i#
re!"elinaion occurs% &n se$ere cases, such as in he ,8,= or ,8+,= $arians (see
belo'), a0onal degeneraion occurs, and reco$er" depends on a0onal regeneraion%
7eco$er" beco!es !uch slo'er, and here is a greaer degree o# residual da!age%
7ecen sudies on he disease ha$e de!onsraed ha appro0i!ael" >9; o# he
paiens ha$e !"elin loss, 'hereas, in he re!aining 29;, he pahologic hall!ar1 o#
he disease is indeed a0on loss%
S I G NS A N D S Y MP T O MS
(he disease is characeri?ed b" 'ea1ness 'hich a##ecs he lo'er li!bs #irs, and
rapidl" progresses in an ascending #ashion% .aiens generall" noice 'ea1ness in heir
legs, !ani#esing as @rubber" legs@ or legs ha end o buc1le, 'ih or 'ihou
d"shesias (nu!bness or ingling)% ,s he 'ea1ness progresses up'ard, usuall" o$er
periods o# hours o da"s, he ar!s and #acial !uscles also beco!e a##eced%
Fre/uenl", he lo'er cranial ner$es !a" be a##eced, leading o bulbar 'ea1ness,
(orophar"ngeal d"sphagia, ha is di##icul" 'ih s'allo'ing, drooling, and/or
!ainaining an open air'a") and respiraor" di##iculies% 8os paiens re/uire
hospiali?aion and abou 39; re/uire $enilaor" assisance% Facial 'ea1ness is also
co!!onl" a #eaure, bu e"e !o$e!en abnor!aliies are no co!!onl" seen in
ascending )*+, bu are a pro!inen #eaure in he 8iller5Fisher $arian (see belo'%)
+ensor" loss, i# presen, usuall" a1es he #or! o# loss o# propriocepion (posiion
sense) and are#le0ia (co!plee loss o# deep endon re#le0es), an i!poran #eaure o#
)*+% Aoss o# pain and e!peraure sensaion is usuall" !ild% &n #ac, pain is a
co!!on s"!po! in )*+, presening as deep aching pain usuall" in he 'ea1ened
!uscles, 'hich paiens co!pare o he pain #ro! o$ere0ercising% (hese pains are
sel#5li!ied and should be reaed 'ih sandard analgesics% *ladder d"s#uncion !a"
occur in se$ere cases bu should be ransien% &# se$ere, spinal cord disease should be
suspeced%
Fe$er should no be presen, and i# i is, anoher cause should be suspeced%
&n se$ere cases o# )*+, loss o# auono!ic #uncion is co!!on, !ani#esing as 'ide
#lucuaions in blood pressure, orhosaic h"poension, and cardiac arrh"h!ias%
(he s"!po!s are si!ilar o hose #or progressi$e in#la!!aor" neuropah"%
[:]
Clinical variants
,lhough ascending paral"sis is he !os co!!on #or! o# spread in )*+, oher
$arians also e0is%
Miller Fisher Syndrome (MFS) is a rare $arian o# )*+ and !ani#ess as a
descending paral"sis, proceeding in he re$erse order o# he !ore co!!on
#or! o# )*+% & usuall" a##ecs he ocular !uscles #irs and presens as
ophhal!oplegia, aa0ia, and are#le0ia% ,ni5)<1b anibodies are presen in
B9; o# cases%
Acute motor axonal neuropathy (AMAN)
!"
# a$a% Chinese &aralytic
Syndrome# aac1s !oor nodes o# 7an$ier and is pre$alen in China and
8e0ico% (he disease !a" be seasonal and reco$er" can be rapid% ,ni5)-1a
anibodies
[D]
are presen% ,ni5)-3 anibodies are #ound !ore #re/uenl" in
,8,=
Acute motor sensory axonal neuropathy (AMSAN) is si!ilar o ,8,= bu
also a##ecs sensor" ner$es 'ih se$ere a0onal da!age% 7eco$er" is slo' and
o#en inco!plee
[>]
%
DI A G N O S I S
(he diagnosis o# )*+ usuall" depends on #indings such as rapid de$elop!en o#
!uscle paral"sis, are#le0ia, absence o# #e$er, and a li1el" inciing e$en% C+F and
EC- is used al!os e$er" i!e o $eri#" s"!po!s, bu because o# he acue naure o#
he disease, he" !a" no beco!e abnor!al unil a#er he #irs 'ee1 o# onse o# signs
and s"!po!s%
CSF 5 "pical C+F #indings include an ele$aed proein le$el (199 5 1999
!g/dA) 'ihou an acco!pan"ing pleoc"osis (increased cell coun)% ,
susained pleoc"osis !a" indicae an alernai$e diagnosis such as in#ecion%
(he diagnosis is con#ir!ed b" he presence o# ,lbu!inoc"ological dissociaion in
he C+F
'lectrodia(nostics 5 elecro!"ograph" (E8)) and ner$e conducion sud"
(=C+) !a" sho' prolonged disal laencies, conducion slo'ing, conducion
bloc1, and e!poral dispersion o# co!pound acion poenial in de!"elinaing
cases% &n pri!ar" a0onal da!age, he #indings include reduced a!pliude o#
he acion poenials 'ihou conducion slo'ing%
)ia(nostic criteria
*e+uired
o &ro(ressive# relatively symmetrical ,ea$ness o- . or more lim/s
due to neuropathy
o Are-lexia
o )isease course 0 1 ,ee$s
o 'xclusion o- other causes (see /elo,)
Supportive
o relatively symmetric ,ea$ness accompanied /y num/ness and2or
tin(lin(
o mild sensory involvement
o -acial nerve or other cranial nerve involvement
o a/sence o- -ever
o typical CSF -indin(s o/tained -rom lum/ar puncture
o electrophysiolo(ic evidence o- demyelination -rom electromyo(ram
)i--erential dia(nosis
Acute myelopathies ,ith chronic /ac$ pain and sphincter dys-unction
/otulism ,ith early loss o- pupillary reactivity
diphtheria ,ith early oropharyn(eal dys-unction
3yme disease polyradiculitis and other tic$-/orne paralyses
porphyria ,ith a/dominal pain# sei4ures# psychosis
vasculitis neuropathy
poliomyelitis ,ith -ever and menin(eal si(ns
CM5 polyradiculitis in immunocompromised patients
critical illness neuropathy
myasthenia (ravis
poisonin(s ,ith or(anophosphate# poison hemloc$# thallium# or arsenic
paresis caused /y 6est Nile 5irus
spinal astrocytoma
T R E A T ME N T
+uppori$e care 'ih !onioring o# all $ial #uncions is he cornersone o# success#ul
!anage!en in he acue paien% E# greaes concern is respiraor" #ailure due o
paral"sis o# he diaphrag!% Earl" inubaion should be considered in an" paien 'ih
a $ial capaci" (FC) G29 !l/1g, a =egai$e &nspiraor" Force (=&F) G52: c!4
2
E,
!ore han 39; decrease in eiher FC or =&F 'ihin 24 hours, rapid progression o#
disease, or auono!ic insabili"%
Ence he paien is sabili?ed, rea!en o# he underl"ing condiion should be
iniiaed as soon as possible% Eiher high5dose inra$enous i!!unoglobulins (&F&g) a
499!g/1g #or : da"s or plas!apheresis can be ad!inisered, as he" are e/uall"
e##eci$e and a co!binaion o# he 'o is no signi#icanl" beer han eiher alone%
(herap" is no longer e##eci$e a#er 2 'ee1s a#er he #irs !oor s"!po!s appear, so
rea!en should be insiued as soon as possible% &F&g is usuall" used #irs because o#
is ease o# ad!inisraion and sa#e" pro#ile, 'ih a oal o# #i$e dail" in#usions #or a
oal dose o# 2 g/1g bod" 'eigh (%41g each da")% (he use o# inra$enous
i!!unoglobulins is no 'ihou ris1, occasionall" causing hepaiis, or in rare cases,
renal #ailure i# used #or longer han #i$e da"s% )lucocoricoids ha$e N78 been #ound
o be e##eci$e in )*+% &# plas!apheresis is chosen, a dose o# 495:9 !A/1g plas!a
e0change (.E) is ad!inisered #our i!es o$er a 'ee1%
Follo'ing he acue phase, he paien !a" also need rehabiliaion o regain los
#uncions% (his rea!en 'ill #ocus on i!pro$ing ,-A (aci$iies o# dail" li$ing)
#uncions such as brushing eeh, 'ashing and geing dressed% -epending on he local
srucuring on healh care, here 'ill be esablished a ea! o# di##eren herapiss and
nurses according o paien needs% ,n occupaional herapis can o##er e/uip!en
(such as 'heel chair and culer") o help he paien achie$e ,-A independence% ,
ph"sioherapis 'ould plan a progressi$e raining progra!!e, and guide he paien o
correc, #uncional !o$e!en, a$oiding har!#ul co!pensaions 'hich !igh ha$e a
negai$e e##ec in he long run% (here 'ould also be a docor, nurse and perhaps a
speech rainer in$ol$ed, depending on he needs o# he paien% (his ea! conribue
'ih heir 1no'ledge o guide he paien o'ards his or her goals, and i is i!poran
ha all goals se b" he separae ea! !e!bers are rele$an #or he paienHs o'n
prioriies% ,#er rehabiliaion he paien should be able o #uncion in his or her o'n
ho!e and aend necessar" raining as needed%
, #unda!enal par o# hospial rea!en should #all on he #a!il"% ,s hospials
reduce healhcare i beco!es i!possible o care #or paiens around he cloc1% .aiens
ha reach oal paral"sis are unable o signal or call #or help and his is 'here #a!il"
care beco!es so i!poran% Fa!il" !e!bers pro$ide care and suppor ha paiens
desperael" need and !edical sa## so!ei!es donH undersand or are unable o
pro$ide% -ue o inaci$i" he bod" loses one and #le0ibili"% &Hs suggesed ha
learning 7ange o# 8oion #ro! !edical sa## and using sreches and 1eeping he
3oins pliable 'ill aid he paien o reco$er sooner han leing he! la" in a
$egeai$e posiion% (his also helps 'ih circulaion and he onse o# bedsores%
*edsore pre$enion !aresses pro$ide co!#or o he paien i# he #a!il" is unable o
!ainain 24 hour care% (his is i!poran because hospial reco$er" #ro! )uillain5
*arre can las #ro! 'ee1s o !onhs%
P R OG N O S I S
8os o# he i!e recovery starts after 4th week #ro! he onse o# he disease%
,ppro0i!ael" 80% of patients have a complete recovery within a few months to a
year, alhough !inor #indings !a" persis, such as are#le0ia% ,bou :519; reco$er
'ih se$ere disabili", 'ih !os o# such cases in$ol$ing se$ere pro0i!al !oor and
sensor" a0onal da!age 'ih inabili" o# a0onal regeneraion% 4o'e$er, his is a gra$e
disease and despie all i!pro$e!ens in rea!en and suppori$e care, he deah rae
a!ong paiens 'ih his disease is sill abou 253; e$en in he bes inensi$e care
unis% 2orld'ide, he deah rae runs slighl" higher (4;), !osl" #ro! a lac1 o#
a$ailabili" o# li#e suppor e/uip!en during he lengh" plaeau lasing 4 o I 'ee1s,
and in so!e cases up o 1 "ear, 'hen a $enilaor is needed in he 'orse cases% ,bou
:519; o# paiens ha$e one or !ore lae relapses, in 'hich case he" are hen
classi#ied as ha$ing chronic in#la!!aor" de!"elinaing pol"neuropah" (C&-.)%
HI S T O R Y
(he disease 'as #irs described b" he French ph"sician Jean Aandr" in 1>:B% &n 1B1I,
)eorges )uillain, Jean ,le0andre *arr6, and ,ndre +rohl diagnosed 'o soldiers
'ih he illness and disco$ered he 1e" diagnosic abnor!ali" o# increased spinal
#luid proein producion, bu nor!al cell coun%
[B]
)*+ is also 1no'n as acute inflammatory demyelinating polyneuropathy, acute
idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and
Landry's ascending paralysis%
N O T A B L E P A T I E N T S
,nd" )ri##ih , acor on Andy Griffith Show, and atloc!% 4e conraced
)uillain5*arr6 in 1B>3% [4]
7achel Chagall , acress, conraced )*+ in 1B>2% &n 1B>D she porra"ed
)abriela *ri!!er, a noable disabiliies aci$is% [:]
Joseph 4eller , auhor, conraced )*+ in 1B>1% (his episode in his li#e is
recouned in he auobiographical "o Laughing atter, 'hich conains
alernaing chapers b" 4eller and his good #riend +peed Fogel%[I]
Fran1lin -% 7oose$el , K%+% presiden% &n 2993, a peer5re$ie'ed sud"
[19]
#ound
ha i 'as !ore li1el" ha 7oose$elHs paral"ic illness 'as acuall" )uillain5
*arr6 s"ndro!e, no polio!"eliis%
8ar1us *abbel , #or!er inernaional #ooballer, conraced )*+ in 2991,
#ollo'ing a period su##ering #ro! he Epsein5*arr $irus% 4e los al!os an
enire "ear o# his #ooballing career be'een he 'o illnesses and ne$er again
de!onsraed he sa!e le$el o# abili" ha 'on hi! o$er :9 caps #or
)er!an"% [D]
+co Llop#ensein , singer/song'rier/guiaris/ru!pe #or he K%+% s1a5pun1
band 7eel *ig Fish conraced )*+ and 'as diagnosed during a our in =e'
Mealand in +epe!ber 299:% ,#er re3oining he band #or a =e' Nears concer,
and a Europe our, he had a recurrence and 'as #lo'n bac1 o he Knied
+aes #ro! ,!serda!% 4e has since reurned o he band%
[)*+/C&-. Foundaion &nernaional 5 in#or!aion and suppor
)uillain5*arr6 +"ndro!e +uppor )roup (KL)
)*+ / C&-. +uppor )roup ,usralia
miller#fisher a =&=-+
Guillain$%arre syndrome and iller$Fisher syndrome a ).noeboo1