Guillain Barre Syndrome

Guillain Barre Syndrome ( GBS ) a group of immune mediated peripheral nerves system disorderThe most frequent cause of acute generalized paralysisSince the virtual elimination of poliomyelitis, GBS has become the leading cause of acute flacid paralysis in western country and development country

Udaya 2000 : incidence GBS 1 3 / 100.000 population in Europe, USA and Australia. Peak in young adult and elderyAllan, 1994 : incidence 1.7 cases per 100.000, and 35 patients with respiratory failure, mortality 1 5 %RSHS 2000 : 24 patient 4 patient died with respiratory failure 2001 : 21 patients : 3 patient died, 1 patient in ICUEpidemiology

Motor dysfunctionSymmetrical limb weakness : proximal, distal, or globalNeck muscle weaknessRespiratory muscle weaknessCranial nerve palsies : III-VII, IX-XIIAreflexiaWasting of limb musclesClinical features of Guillain Barre Syndrome

Sensory dysfunctionPainNumbness, paraesthesiaeLoss of joint position sense, vibration, touch and pain distallyAtaxia

Autonomic dysfunctionSinus tachycardia and bradycardiaOther cardiac arrhytmias ( both tachy and brady )Hypertension and postural hypotensionWide fluctuations of pulse and blood pressureHypersalivationAnhydrosis or excessive sweatingUrinary sphincter disturbancesConstipationGastric dysmotilityAbnormal vasomotor tone causing venous pooling and facial flushing

Guillain Barre Syndrome and variantsWeakness Is PredominantAcute inflammatory demyelinating Polyradiculoneuropathy ( AIDP )Acute motor axonal neuropathy ( AMAN )Acute motor sensory axonal neuropathy ( AMSAN )

Weakness Is Not PredominantFisher syndromeAcute panautonomic neuropathyPure sensory neuropathy

Features of Acute Inflammatory Demyelinating Polyradiculoneuropathy ( AIDP )

CLINICALTwo-thirds of patients have antecedent infection or another provocative eventSymptoms begin with paresthesias and pain (50%) followed by muscles weakness in the legs; about 10 % begin with arm weakness; rarely weakness begins in the face.Complete ophthalmoplegia in 3 % - 5 %; partial in 15 %Autonomic manifestations include labile blood pressure, cardiac arrhythmias, bladder dysfunction, constipation, abdominal distension and bolatingDisease progresses for days to 4 weeks. The average time to onset of recovery is 4 weeks80 % of patients recovery within 6 months15 % have severe residual disabilityMortality rate 3 % - 5 %

Features of Acute Motor Sensory Axonal Neuropathy (AMSAN)

CLINICAL FEATURESCommonly preceded by diarrhea, especially related to compylobacter jejuni infection. Abrupt onset of weakness, with rapid progression to quadriplegia and often early respiratory insufficiency.Patients may have facial weakness, ophthalmoparesis, and autonomic instabilityPatients have longer recovery periods with significant residual deficits than in AIDPMortality rate 5 % - 10 %

Features of Acute Motor Axonal Neuropathy ( AMAN )

CLINICAL FEATURESThe condition is often preceded by a history of diarrhea, especially Campylobacter jejuniAffects children and young adults in Northern China primarily, and elsewhere; it occurs sporadically in North AmericaMortality rate 5 %CEREBROSPINAL FLUID CHANGESProtein is elevated in most cases after the first weekSpinal fluid cell counts are normal

Features of Fisher Syndrome

CLINICAL FEATURESSyndrome usually begins with diplopia, followed in 3 4 days by limb and gait ataxiaComplete ophthalmoplegia evolves over several daysAreflexia is typicalSensory loss is usually mild in the distal limbsA mild degree of muscle weakness may be presentThere is an excellent prognosis for full recovery

Features of Fisher Syndrome ( contd )

CEREBROSPINAL FLUIDThe protein is usually elevated after 7 10 days of illnessNo significant pleocytosis occursELECTRODIAGNOSTIC STUDIESDecreased amplitude of sensory nerve action potentials that return with time is commonSensory conduction velocities are normal, as are motor nerve conduction studies

Features of Acute Panautonomic Neuropathy

CLINICAL FEATURESOnset occurs over 1 2 weeks in most patients but may be subacute ( over 8 weeks )Manifestations include : lightheadedness, dizziness, orthostatic hypotension, nausea, vomiting, diarrhea, constipation, and postprandial bloating.Other findings include : heat intolerance, decreased sweating, blurred vision, dry eyes, voiding problems, and impotenceMuscle strech reflexes are lost in one third of patients, and distal sensory loss in one fourthCEREBROSPINAL FLUIDElevated protein without pleocytosis occurs in the majority of, but not all, patients

Clinical features :Small number of cases GBSManifestation include : ataxia, areflexi, sensory neuropathy, little or no motor involvement.Severe cases may have of the face and trunk, have an commonFeatures of Pure Sensory Neuropathy

Antecedent Events for Guillain Barre Syndrome* Isolated reports of various individual viruses or bacteria

INFECTIONSViralEpstein Barr virusCytomegalovirusHuman immunodeficiency virusInfluenza virusesCoxsackie virusesHerpes simplexHepatitis A and C virusesOthers*

* Isolated reports of various individual viruses or bacteria

INFECTIONSBacterial infectionsCampylobacter jejuniMycoplasma pneumoniaeEscherichia coliOther*

ParasiticMalariaToxoplasmosis

SISTEMIC ILLNESSHodgkins diseaseChronic lymphocytic leukemiaHyperthyroidismCollagen vascular diseasesSarcoidosisRenal disease

OTHER MEDICAL CONDITIONSPregnancySurgical proceduresBone marrow transplantationImmunizations ( e.g.,swine flu )EnvenomizationDrug ingestion

Criteria for admitting GBS patients to ICU

Vital capacity less than 12 ml / kgDeteriorating vital capacity less than 18 to 20 mL/kg; clinical signs of diaphragmatic fatigue including tachypnea, diaphoresis, paradoxical breathingPoor cough, accumulating secretions, aspiration pneumoniaProgressive weakness associated with difficulty swallowingMajor dysautonomic features ( wide blood pressure and pulse fluctuations : arrhythmias, heart block, pulmonary edema, profound ileus with risk of visceral rupture )Hypotension precipitated by plasma exchange, or plasma exchange planned in a ventilated or unstable patientSepsis or pneumoniaChest pain

Autonomic dysfunction

DysautonomiaNo. casesPercentSinus tachycardiaLabile heart rateOrthostatic hypotensionSustained hypertensionParoxysmal hypertension Vagal spells Other arrhythmiasAbnormal drug responsesUrinary retentionUrinary incontinenceImpotence ( males )ConstipationIleusFecal incontinence62143254013824642241523781932485127221491

ICU Complication

Mortality 1 5 %Tracheostomy pneumoniUrinary infectionPhlebilitisPulmonary emboliDepression

Treatment

Supportive care remains unequivocally the most important component of treatmentEssentially all patient should be observed in hospital for at least 2 3 weekPatient with severe disease especially there with respiratory insufficien requiring intubation. And with autonomic instability need closed observation and manually in an Intensive Care Unit

* Prevent the multiple medical complication as result prolonged Immobility * Rehabilitation effort during the acute phases * Pain control * Psychological suport for patient and families

Outcome and prognosis

Udaya : - 36 % improvement the first week - 85 % improvement fourth weekDeath rate 13 % become 5 % in ICUCause of death : - cardiac arest 25 % - respiratory failure 75 %

Poor Prognostic Featuresfor Guillain Barre Syndrome

Older ageRapid onset prior to presentation ( 7 days )Ventilator dependencyInexcitable or reduced amplitude motor evoked responsesNo treatment ( plasma exchange or intravenous immunoglobulin )Preceding diarrheal illness

Factor associated with poor outcome

AetiologyPrevious gastrointestinal infectionCytomegalovirusClinical featuresOlder ageShorter latency to nadirLonger time to clinical improvementNeed for mechanical ventilationGreater disability and disease severity

ElectrophysiologyAbsent or reduced CMAP ( mean distal CMAP amplitude 20 % of the lower limit normal )Inexcitable nervesBiochemical markersAnti-GM1 antibodiesNeurone specific enolase and S-100b proteins in CSF