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Nani Kurniani

Guillain Barre Syndrome

Department of Neurology

Dr. Hasan Sadikin General Hospital

Bandung

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Guillain Barre Syndrome ( GBS ) is the eponym

used to refer a group of immune mediated

peripheral nerves system disorder

The most frequent ause of a ute generali!edparalysis

Sin e the virtual elimination of poliomyelitis" GBS

has #e ome the leading ause of a ute fla id

paralysis in $estern ountry and development

ountry

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%daya &''' in iden e GBS * + , ''.''' population in-urope" %S and ustralia. /eak in young adult andeldery

llan" 001 in iden e .2 ases per ''.'''" and +3patients $ith respiratory failure" mortality * 3 4

Hughes" 005 6espiratory failure requiring artifi ialventilation o ur in a#out 1 4 patients

6SHS &''' &1 patient 1 patient died $ith respiratory

failure

&'' & patients + patient died" patient in 78%

Epidemiology

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9otor dysfun tion

Symmetri al lim# $eakness pro:imal" distal" or glo#al

Ne k mus le $eakness

6espiratory mus le $eakness

8ranial nerve palsies 777;

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Sensory dysfun tion

/ain

Num#ness" paraesthesiae

?oss of @oint position sense" vi#ration" tou h and paindistally

ta:ia

Clinical features of Guillain Barre Syndrome( contd )

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utonomi dysfun tion

Sinus ta hy ardia and #rady ardia

Ather ardia arrhytmias ( #oth ta hy and #rady )

Hypertension and postural hypotension

>ide flu tuations of pulse and #lood pressure

Toni pupils

Hypersalivation

nhydrosis or e: essive s$eating

%rinary sphin ter distur#an es8onstipation

Gastri dysmotility

#normal vasomotor tone ausing venous pooling and fa ialflushing

Clinical features of Guillain Barre Syndrome( contd )

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Ather

/apilloedema

Clinical features of Guillain Barre Syndrome( contd )

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Guillain Barre Syndrome and variants

Weakness Is Predominant

ute inflammatory demyelinating /olyradi uloneuropathy

( 7D/ ) ute motor a:onal neuropathy ( 9 N )

ute motor sensory a:onal neuropathy ( 9S N )

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Guillain Barre Syndrome and variants ( contd )

Weakness Is Not Predominant

isher syndrome

ute panautonomi neuropathy

/ure sensory neuropathy

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Features of Acute Inflammatory DemyelinatingPolyradiculoneuropathy ( AIDP

CLINICALCLINICAL

Two-thirds of patients have antecedent infection or another Two-thirds of patients have antecedent infection or anotherprovocative eventprovocative eventSymptoms begin with paresthesias and pain (50%) followedSymptoms begin with paresthesias and pain (50%) followedby muscles weakness in the legs about !0 % begin withby muscles weakness in the legs about !0 % begin with

arm weakness rarely weakness begins in the face"arm weakness rarely weakness begins in the face"#omplete ophthalmoplegia in $ % - 5 % partial in !5 %#omplete ophthalmoplegia in $ % - 5 % partial in !5 %utonomic manifestations include labile blood pressure&utonomic manifestations include labile blood pressure&cardiac arrhythmias& bladder dysfunction& constipation&cardiac arrhythmias& bladder dysfunction& constipation&abdominal distension and bolatingabdominal distension and bolating

'isease progresses for days to weeks" The average time'isease progresses for days to weeks" The average timeto onset of recovery is weeksto onset of recovery is weeks0 % of patients recovery within * months0 % of patients recovery within * months!5 % have severe residual disability!5 % have severe residual disability+ortality rate $ % - 5 %+ortality rate $ % - 5 %

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Features of Acute Inflammatory DemyelinatingPolyradiculoneuropathy ( AIDP ( contd )

CEREBROSPINAL FLUIDCEREBROSPINAL FLUID,rotein is elevated in 0 % of cases by the nadir of illness",rotein is elevated in 0 % of cases by the nadir of illness",leocytosis,leocytosis >> !0 cells.mm!0 cells.mm $$ in 5 %in 5 % >> 50 cell . mm50 cell . mm $$ suggestssuggests/ 1 infection/ 1 infection

ELECTRODIAGNOSTIC STUDIESELECTRODIAGNOSTIC STUDIES,artial motor conduction block is found in 25 %& with study,artial motor conduction block is found in 25 %& with studyof pro3imal nerves and nerve rootsof pro3imal nerves and nerve roots4outine studies demonstrate partial motor conduction block4outine studies demonstrate partial motor conduction blockin only $0 % - 0 % of casesin only $0 % - 0 % of cases

,rolonged distal and -wave latencies are found in the,rolonged distal and -wave latencies are found in thema6ority of casesma6ority of cases'emyelinating conduction velocities appear in the third and'emyelinating conduction velocities appear in the third andfourth weekfourth week

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Features of Acute Inflammatory DemyelinatingPolyradiculoneuropathy ( AIDP ( contd )

IMMUNOPATHOLOGY / PATHOLOGY IMMUNOPATHOLOGY / PATHOLOGY

Sural nerve biopsy is not recommended for diagnosis& as itSural nerve biopsy is not recommended for diagnosis& as it

may be normal or nondiagnostic and the diagnosis can bemay be normal or nondiagnostic and the diagnosis can be

made con7dently through other meansmade con7dently through other means

mmune attack is directed at the Schwann cellmmune attack is directed at the Schwann cell

plasmalemma& especially in the nerve roots& with g8 andplasmalemma& especially in the nerve roots& with g8 and

complement deposits preceding demyelination"complement deposits preceding demyelination"

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Features of Acute !otor Sensory A"onal #europathy(A!SA#

CLINICAL FEATURESCLINICAL FEATURES

#ommonly preceded by diarrhea& especially related to#ommonly preceded by diarrhea& especially related tocompylobacter 6e6uni infection"compylobacter 6e6uni infection"

brupt onset of weakness& with rapid progression tobrupt onset of weakness& with rapid progression to9uadriplegia and often early respiratory insu:ciency"9uadriplegia and often early respiratory insu:ciency"

,atients may have facial weakness& ophthalmoparesis& and,atients may have facial weakness& ophthalmoparesis& andautonomic instabilityautonomic instability

,atients have longer recovery periods with signi7cant,atients have longer recovery periods with signi7cantresidual de7cits than in ',residual de7cits than in ',

+ortality rate 5 % - !0 %+ortality rate 5 % - !0 %

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Features of Acute !otor Sensory A"onal #europathy(A!SA# ( contd )

CEREBROSPINAL FLUID CHANGESCEREBROSPINAL FLUID CHANGES

,rotein is elevated without signi7cant cellular response,rotein is elevated without signi7cant cellular response

ELECTRODIAGNOSTIC STUDIESELECTRODIAGNOSTIC STUDIES

There is marked reduction in compound muscle action There is marked reduction in compound muscle actionpotential amplitudes or electrical ine3citability of motorpotential amplitudes or electrical ine3citability of motornerves and absent sensory nerve action potentialsnerves and absent sensory nerve action potentials

bundant 7brillations are usually present onbundant 7brillations are usually present onelectromyographic e3amination at ; < $ weekselectromyographic e3amination at ; < $ weeks

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Features of Acute !otor Sensory A"onal #europathy(A!SA# ( contd )

IMMUNOPATHOLOGY / PATHOLOGY IMMUNOPATHOLOGY / PATHOLOGY

mmune attack is directed at a3on plasmalemma with g8mmune attack is directed at a3on plasmalemma with g8

and complement deposits at nodes of 4anvier" +acrophagesand complement deposits at nodes of 4anvier" +acrophages

are recruited& which penetrate the basal lamina and directlyare recruited& which penetrate the basal lamina and directlyattack the a3on"attack the a3on"

=3tensive >allerian-like degeneration is seen in the nerve=3tensive >allerian-like degeneration is seen in the nerve

roots"roots"

,eripheral nerves become involved at later time points",eripheral nerves become involved at later time points"

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Features of Acute !otor A"onal #europathy ( A!A#

CLINICAL FEATURESCLINICAL FEATURES The condition is often preceded by a history of diarrhea& The condition is often preceded by a history of diarrhea&especially #ampylobacter 6e6uniespecially #ampylobacter 6e6uni

?ects children and young adults in @orthern #hina?ects children and young adults in @orthern #hina

primarily& and elsewhere it occurs sporadically in @orthprimarily& and elsewhere it occurs sporadically in @orthmericamerica

+ortality rate+ortality rate

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Features of Fisher Syndrome

CLINICAL FEATURESCLINICAL FEATURESSyndrome usually begins with diplopia& followed inSyndrome usually begins with diplopia& followed in$ < days by limb and gait ata3ia$ < days by limb and gait ata3ia

#omplete ophthalmoplegia evolves over several#omplete ophthalmoplegia evolves over severaldaysdaysreAe3ia is typicalreAe3ia is typicalSensory loss is usually mild in the distal limbsSensory loss is usually mild in the distal limbs

mild degree of muscle weakness may be mild degree of muscle weakness may bepresentpresent There is an e3cellent prognosis for full recovery There is an e3cellent prognosis for full recovery

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Features of Fisher Syndrome ( contd )

CEREBROSPINAL FLUIDCEREBROSPINAL FLUID The protein is usually elevated after 2 < !0 days of The protein is usually elevated after 2 < !0 days ofillnessillness

@o signi7cant pleocytosis occurs@o signi7cant pleocytosis occursELECTRODIAGNOSTIC STUDIESELECTRODIAGNOSTIC STUDIES

'ecreased amplitude of sensory nerve action'ecreased amplitude of sensory nerve actionpotentials that return with time is commonpotentials that return with time is common

Sensory conduction velocities are normal& as areSensory conduction velocities are normal& as aremotor nerve conduction studiesmotor nerve conduction studies

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Features of Fisher Syndrome ( contd )

PATHOLOGY PATHOLOGY ,athology is poorly characteriBed,athology is poorly characteriBedcompared to other patterns of 8CS"compared to other patterns of 8CS"

#hanges described in nerve roots& cranial#hanges described in nerve roots& cranialnerves& and peripheral nerves& withnerves& and peripheral nerves& withpatchydemyelinationpatchydemyelination

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Features of Acute Panautonomic #europathy

CLINICAL FEATURESCLINICAL FEATURES

Dnset occurs over ! < ; weeks in most patientsDnset occurs over ! < ; weeks in most patientsbut may be subacute ( over weeks )but may be subacute ( over weeks )+anifestations include E lightheadedness&+anifestations include E lightheadedness&diBBiness& orthostatic hypotension& nausea&diBBiness& orthostatic hypotension& nausea&

vomiting& diarrhea& constipation& and postprandialvomiting& diarrhea& constipation& and postprandialbloating"bloating"Dther 7ndings include E heat intolerance&Dther 7ndings include E heat intolerance&decreased sweating& blurred vision& dry eyes&decreased sweating& blurred vision& dry eyes&voiding problems& and impotencevoiding problems& and impotence+uscle strech reAe3es are lost in one third of+uscle strech reAe3es are lost in one third ofpatients& and distal sensory loss in one < fourthpatients& and distal sensory loss in one < fourth

CEREBROSPINAL FLUIDCEREBROSPINAL FLUID

=levated protein without pleocytosis occurs in the=levated protein without pleocytosis occurs in the

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Features of Acute Panautonomic #europathy( contd )

ELECTRODIAGNOSTIC STUDIESELECTRODIAGNOSTIC STUDIES@erve conduction studies are usually normal@erve conduction studies are usually normalutonomic reAe3 tests E sinus arrhythmia lost toutonomic reAe3 tests E sinus arrhythmia lost todeep breathing 1alsalva maneuver showsdeep breathing 1alsalva maneuver showse3aggerated fall in early phase & absence ofe3aggerated fall in early phase & absence ofrecovery in late phase & and reduced or absentrecovery in late phase & and reduced or absentovershoot in phase 1 1alsalva ratio diminishedovershoot in phase 1 1alsalva ratio diminishedreduced sudomotor functionreduced sudomotor function

PATHOLOGY PATHOLOGY Sural nerve may be normal or show mild changesSural nerve may be normal or show mild changesincluding loss of myelinated and unmyelinatedincluding loss of myelinated and unmyelinated7bers& mild a3onal degeneration& and scattered7bers& mild a3onal degeneration& and scattereddemyelinationdemyelination

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Clinical features $

Small num#er of ases GBS

9anifestation in lude ata:ia" arefle:i" sensoryneuropathy" little or no motor involvement.

Severe ases may have of the fa e and trunk"

have an ommon

Features of Pure Sensory #europathy

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Antecedent Events for Guillain % Barre Syndrome

INFECTIONSINFECTIONS

1iral1iral=pstein < Carr virus=pstein < Carr virus#ytomegalovirus#ytomegalovirus/uman immunode7ciency virus/uman immunode7ciency virusnAuenBa virusesnAuenBa viruses#o3sackie viruses#o3sackie viruses/erpes simple3/erpes simple3

/epatitis and # viruses/epatitis and # virusesDthersFDthersF

* Isolated reports of various individual viruses or bacteria

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Antecedent Events for Guillain % Barre Syndrome ( contd )

INFECTIONSINFECTIONS

Cacterial infectionsCacterial infections#ampylobacter 6e6uni#ampylobacter 6e6uni+ycoplasma pneumoniae+ycoplasma pneumoniae=scherichia coli=scherichia coliDtherFDtherF

,arasitic,arasitic

+alaria+alaria To3oplasmosis To3oplasmosis

* Isolated reports of various individual viruses or bacteria

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Antecedent Events for Guillain % Barre Syndrome ( contd )

SISTEMIC ILLNESSSISTEMIC ILLNESS

/odgkinGs disease/odgkinGs disease

#hronic lymphocytic leukemia#hronic lymphocytic leukemia

/yperthyroidism/yperthyroidism#ollagen vascular diseases#ollagen vascular diseases

SarcoidosisSarcoidosis

4enal disease4enal disease

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Antecedent Events for Guillain % BarreSyndrome ( contd )

OTHER MEDICAL CONDITIONSOTHER MEDICAL CONDITIONS,regnancy,regnancy

Surgical proceduresSurgical proceduresCone marrow transplantationCone marrow transplantationmmuniBations ( e"g"&swine Au )mmuniBations ( e"g"&swine Au )=nvenomiBation=nvenomiBation'rug ingestion'rug ingestion

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Criteria for admitting GBS patients to IC&

1ital capacity less than !; ml . kg1ital capacity less than !; ml . kg'eteriorating vital capacity less than ! to ;0'eteriorating vital capacity less than ! to ;0mH.kg clinical signs of diaphragmatic fatiguemH.kg clinical signs of diaphragmatic fatigueincluding tachypnea& diaphoresis& parado3icalincluding tachypnea& diaphoresis& parado3icalbreathingbreathing

,oor cough& accumulating secretions& aspiration,oor cough& accumulating secretions& aspirationpneumoniapneumonia,rogressive weakness associated with di:culty,rogressive weakness associated with di:cultyswallowingswallowing

+a6or dysautonomic features ( wide blood pressure+a6or dysautonomic features ( wide blood pressureand pulse Auctuations E arrhythmias& heart block&and pulse Auctuations E arrhythmias& heart block&pulmonary edema& profound ileus with risk ofpulmonary edema& profound ileus with risk ofvisceral rupture )visceral rupture )

/ypotension precipitated by plasma e3change& or/ypotension precipitated by plasma e3change& orplasma e3change planned in a ventilated orplasma e3change planned in a ventilated or

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'a n )**+

!echanical ventilation

6apid disease progression

Bul#ar dysfun tion

Bilateral fa ial $eakness

Dysautonomia

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Autonomic dysfunction

DysautonomiaDysautonomia No. as!sNo. as!s P!" !ntP!" !nt

Sinus tachycardiaSinus tachycardiaHabile heart rateHabile heart rateDrthostatic hypotensionDrthostatic hypotensionSustained hypertensionSustained hypertension,aro3ysmal hypertension,aro3ysmal hypertension

II 1agal spells I1agal spells IDther arrhythmiasDther arrhythmiasbnormal drug responsesbnormal drug responsesJrinary retentionJrinary retentionJrinary incontinenceJrinary incontinencempotence ( males )mpotence ( males )#onstipation#onstipationleusleusecal incontinenceecal incontinence

*;*;!!$;$;5500

!$!$

;;**

;;;;!5!5;;

$2$2

!!$$

;;

55!!

;2;2;;;;

!!

!!

espiratory Failure

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,espiratory Failure-

#ormal

./ ml01g

2/ ml01g

3* ml01g

)/ ml01g

)* ml01g

+/ ml01g

+* ml01g

/ ml01g

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IC& Complication

9ortality * 3 4Tra heostomy pneumoni

%rinary infe tion/hle#ilitis/ulmonary em#oli

Depression

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T4= T+=@T (conKt) T4= T+=@T (conKt)

F ,revent the multiple medicalF ,revent the multiple medicalcomplication as result prolongedcomplication as result prolongedmmobilitymmobility

F 4ehabilitation e?ort during the acuteF 4ehabilitation e?ort during the acutephasesphases

F ,ain controlF ,ain control

F ,sychological suport for patient andF ,sychological suport for patient andfamiliesfamilies

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5utcome and prognosis

%daya ; +5 4 improvement the first $eek

; 3 4 improvement fourth $eek

Death rate + 4 #e ome 3 4 in 78%

8ause of death ; ardia arest &3 4

; respiratory failure 23 4

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Poor Prognostic Featuresfor Guillain Barre Syndrome

Dlder ageDlder age4apid onset prior to presentation (4apid onset prior to presentation (

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Factor associated ith poor outcome

etiology/revious gastrointestinal infe tion8ytomegalovirus

8lini al featuresAlder ageShorter laten y to nadir ?onger time to lini al improvementNeed for me hani al ventilationGreater disa#ility and disease severity

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Factor associated ith poor outcome ( contd )

-le trophysiology #sent or redu ed 89 / ( mean distal 89 /

amplitude < &' 4 of the lo$er limit normal )

7ne: ita#le nerves

Bio hemi al markers

nti;G9 anti#odies

Neurone spe ifi enolase and S; ''# proteins

in 8S

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