gbs kuliah pa

8/9/2019 GBS Kuliah PA

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PARYONO


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Objectives1. Defne Guillain Barre Syndrome GBS! and describeits "at#o"#ysiolo$y%causes% e"idemiolo$y% usual"resentation% and most common variants.

&. Describe t#e usual #istorical "resentation and"#ysical e'am fndin$s (or "atients )it# GBS.

*. Outline t#e )or+ u" o( a "atient )it#actual,sus"ected GBS.

-. State t#e treatment a""roac# (or GBS )it#em"#asis u"on di erences bet)een adults andc#ildren and common com"lications.

/. Outline (ollo) u" and "ro$nosis (or GBS "atients.


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Di erential dia$nosis 01. GBS&.Acute myelo"at#ies

*. Botulism-. Di"#t#eria/. yme disease2. Por"#yria3. 4asculitis neuro"at#y5. 6ritical illness

neuro"at#y

7.8yast#enia $ravis19. Poisonin$s

11. Poliomyelitis1&. 684 "olyradiculitis1*. :od$+in;s disease

1-. S <1/. Sarcoidosis


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Pathogenes

is1. Ingestion of campylobactercontaining food, water 2. Patients have infectious

disease(GI/ tract! in wee"s prior toonset.

#. immune mediated disease4, Lymphocytic infltration o(s"inal roots and "eri"#eralnerves

5. Macrophage mediated

multi(ocal stri""in$ o( myelin2. De(ects in nerve im"ulse

"ro"a$ation3. Results in conduction bloc+

and ?accid "aralysis o( some

de$ree GBS !


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4ariations = 1 .8iller >is#er Syndrome 4ariant / @o( cases!1. Ata'ia mostly in $ait and trun+!&. O"t#almo"le$ia*. Are?e'ia-. 8otor stren$t# usually s"ared./. Gradual and near (ull recovery over

)ee+s to mont#s2. O(ten involves oculomotor% abducens%

and troc#lear nerves


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4ariations=*A'onal >orm A8SAN Acute motorsensory a'onal neuro"at#y! 1. Esually "resents )it# ra"id% severe

"aralysis&. Delayed and incom"lete recovery*. Associated )it# "rior 6. jejuni diarr#ea-. Pat#olo$y s#o)s severe a'onal

de$eneration o( motor and sensory nervefbers )it# little demyelination


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4ariations=/

Acute "andysautonomia )it#outsi$nifcant motor or sensoryinvolvement= Rare!1. Postural #y"otension&. Bo)el and bladder retention*. An#idrosis

-. Decreased salivation and lacrimation/. Abnormal "u"illary res"onses


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4ariations=2

P#aryn$eal=6ervical=Brac#ial 4ariantisolated (acial% oro"#aryn$eal% cervical%and u""er limb )ea+ness


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6 N 6A >


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P:YS 6A


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P:YS 6A


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P:YS 6A


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6AES


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6auses=&

/. n in(ants% consider botulism=)ea+ness%es" o(


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or+ E"= ab

1. "rotein% no B6 elevation=(rom in?ammationJ6S> may be normal (or-5 #oursJ"rotein "ossibly normal (or 1)ee+.

&. 6B6%68P to r,o ot#er in(ectious or metaboliccauses o( )ea+ness

*. Peri"#eral neuro"at#y evalCt#yroid"anel%r#eum "rofle%B=1&%(olate%#$bA16%


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or+ u"= ma$in$% P>Fs

1. 8R nons"ecifc=can s#o) nerve rooten#ancement

&. P>Fs a! 8a'imal ins"iratory "ressure and vital

ca"acity=measure neuromuscular res"iratory(unction and "redict dia"#ra$m stren$t#J

b! 8a'. e'"iratory "ressures re?ect abdominalmuscle stren$t#J c#ec+ t#ese o(ten

c! Get res". assistance i( e'". 46 15 m ,+$ ordro" in O& saturation


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or+ E"=


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or+ E"= =)ave


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Diagnostic criteriaA. Re uired

1! Pro$ressive% relatively symmetrical )ea+ness o(t)o or more limbs due to neuro"at#y

&! Are?e'*! Disorder course - )ee+s-!


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Freatment= 11. -9@ "atients #os"italiLed )ill need in"atient

re#abilitation% 1,* need ventilatory su""ort&.


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Freatment=&

/.


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Freatment=*

1*. O"timiLe nutrition1-. 8onitor (or in(ectious com"lications

EF %"neumonia%se"sis!1/. Res"iratory t#era"y=serial eval o(

ventilatory status12. 6ardiac monitorin$13. Prevent D4F% "ressure

ulcers%contractures15. Bo)el and bladder (unction


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Freatment=-

15. Sur$ical trac#eotomy% "ercutaneous(eedin$ tubes

17. 6onsultationsNeurolo$y%Pulmonolo$y%6ardiolo$y%Psyc#iatry etc.

&9. 8edications 4 G% "lasma"#eresis%etc.&1. >ollo) u" care "ersistent (ati$ue%

c#an$es in )or+,leisure activity in 1,*"ts


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Freatment=/

&&. mmunomodulatory t#era"y1. Plasma"#eresis and intravenous

immuno$lobin 4 G!&. Bot# #ave e ual e cacy*. 6ombined treatment does not im"rove

disability-. Esed based on disease severity or in

ra"id "ro$ression


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Freatment=5

1. No role (or oral or 4 steroids&. Pain NSA Ds% acetamino"#en% narcs as

needed.*. Anticoa$ulation as )arranted-. mmunot#era"y in "re$nancy not

studiedJsa(ety not )ell +no)n


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>ollo) u"1. 8onitor care(ully (or com"lications&. Patients need out"atient care to im"rove

stren$t# and "ersistent (ati$ue*. 6#an$es in )or+ or leisure activity

needed in around 1,* o( cases-. 19@ "atients rela"se acutely a(ter initial

im"rovement=es". i( later onset o(

treatment%"rotracted,com"le' course


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Pro$nosis

1. &=1&@ die o( com"lications&. 3/=5/@ #ave $ood recovery*. 1/=&9@ #ave moderate residual defcits-. 1=19@ le(t )it# severe disability

/. orse outcomes i( older a$e% "oor E< stren$t#% need(or ventilator su""ort% ra"idly "ro$ressin$ )ea+ness%lo) mean 68AP am"litudes o( &9@ o( lo)er limit o(normal% "ersistent lo) 68APH 1 mont#% 6S> levels o(#i$# molecular )ei$#t monoflament "rotein

2. GBS is a li(e c#an$in$ event )it# "otential lon$lastin$ in?uence


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Pro$nosis in Qids1. 6#ild#ood mortality rate /@&. Deat# usually (rom res"iratory (ailure*. 8ost common serious com"lication is )ea+ness

o( res"iratory muscles and autonomic instability

-. 8ost 79=7/@! +ids recover in *=1& mont#s/. /=19@ #ave si$nifcant "ermanent disability2. Outcome usually better t#an (or adults

3. Recovery "eriods lasts lon$er t#an acute illness5. /@ recurrence rate


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$han" you for your

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gbs kuliah pa

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