guillain-barre’ syndrome
DESCRIPTION
Guillain-Barre’ Syndrome. Guillain Barre Syndrome. 1859 - Landry’s report on 10 patients with “ascending paralysis” 1916 - Guillain, Barre, Strohl described two French soldiers with motor weakness, areflexia, and “albuminocytological dissociation” in the CSF. Epidemiology. - PowerPoint PPT PresentationTRANSCRIPT
Guillain-Barre’ SyndromeGuillain-Barre’ Syndrome
Guillaine Barre SyndromeGuillaine Barre Syndrome
Guillain Barre SyndromeGuillain Barre Syndrome
1859 - Landry’s report on 10 patients with 1859 - Landry’s report on 10 patients with “ascending paralysis”“ascending paralysis”
1916 - Guillain, Barre, Strohl described two 1916 - Guillain, Barre, Strohl described two French soldiers with motor weakness, French soldiers with motor weakness, areflexia, and “albuminocytological areflexia, and “albuminocytological dissociation” in the CSFdissociation” in the CSF
Guillaine Barre SyndromeGuillaine Barre Syndrome
EpidemiologyEpidemiology
1-3/100,000 - Europe, USA, Australia1-3/100,000 - Europe, USA, Australia
-occurs in any age group-occurs in any age group
-bimodal age distribution-bimodal age distribution
-increase incidence with age-increase incidence with age
-males > females-males > females
-seasonal variations-seasonal variations
-lower during pregnancy, increases after -lower during pregnancy, increases after deliverydelivery
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinical FeaturesClinical Features
-antecedent event-antecedent event
respiratory infection 40%respiratory infection 40%
gastroenteritis 20%gastroenteritis 20%
-common manifestations-common manifestations
proximal limb weaknessproximal limb weakness
facial palsyfacial palsy
sensory symptoms 80%sensory symptoms 80%
pain 90%pain 90%
autonomic dysfunction autonomic dysfunction 66%66%
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinical FeaturesClinical Features•Motor DysfunctionMotor Dysfunction
symmetrical limb symmetrical limb weaknessweakness
neck muscle weaknessneck muscle weakness
respiratory weaknessrespiratory weakness
CN III-VII, IX-XII palsiesCN III-VII, IX-XII palsies
areflexiaareflexia
wastingwasting
•Sensory dysfunctionSensory dysfunction
painpain
numbness, paresthesiasnumbness, paresthesias
loss of position sense, loss of position sense, vibration, touch, painvibration, touch, pain
ataxiaataxia
•Autonomic dysfunctionAutonomic dysfunction
sinus sinus tachycardia/bradycardiatachycardia/bradycardia
hypertension hypertension
postural hypotensionpostural hypotension
fluctuations, PR and BPfluctuations, PR and BP
tonic pupilstonic pupils
hypersalivationhypersalivation
anhidrosis or excess anhidrosis or excess sweatingsweating
urinary sphincter urinary sphincter disturbancedisturbance
constipationconstipation
gastric dysmotilitygastric dysmotility
abnormal vasomotor toneabnormal vasomotor tone
•OtherOther
papilloedemapapilloedema
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinical FeaturesClinical Features
-onset is acute or subacute-onset is acute or subacute
nadir nadir 12 days 12 days
improvementimprovement 28 days 28 days
recoveryrecovery 200 days200 days
-plateau phase -plateau phase reached 4 weeks from reached 4 weeks from onset onset in 98% of patients in 98% of patients
-plateau duration -plateau duration 12 days12 days
Guillaine Barre SyndromeGuillaine Barre Syndrome
Antecedent EventsAntecedent Events
Campylobacter jejuniCampylobacter jejuni--most common pathogen most common pathogen
26-30% 26-30% USA and EuropeUSA and Europe
45% 45% JapanJapan
-AMAN and AMSAN more common-AMAN and AMSAN more common
-common preceding infection in Miller Fisher Syndrome-common preceding infection in Miller Fisher Syndrome
PathogenesisPathogenesis
””molecular mimicry”molecular mimicry”
GM1 ganglioside GM1 ganglioside
Guillaine Barre SyndromeGuillaine Barre Syndrome
Antecedent EventsAntecedent Events
CytomegalovirusCytomegalovirus-second most common -second most common
5% 5% Japan Japan
11-13% 11-13% EuropeEurope
-more common in females and young age groups-more common in females and young age groups
-severe course, with respiratory difficulties-severe course, with respiratory difficulties
-reduced SNAPs are more common-reduced SNAPs are more common
PathogenesisPathogenesis
””molecular mimicry”molecular mimicry”
GM2 gangliosideGM2 ganglioside
Guillaine Barre SyndromeGuillaine Barre Syndrome
Antecedent EventsAntecedent Events
Associated infectionsAssociated infections
Epstein Barr (10%) Epstein Barr (10%)
Mycoplasma pneumoniae Mycoplasma pneumoniae (5%)(5%)
HIVHIV
Lyme diseaseLyme disease
Possibly Associated infectionsPossibly Associated infections
hepatitis A, B, C, and Dhepatitis A, B, C, and D
typhoid typhoid
falciparum malariafalciparum malaria
Guillaine Barre SyndromeGuillaine Barre Syndrome
Antecedent EventsAntecedent Events
Vaccines?Vaccines?
-influenza vaccine-influenza vaccine
-polio vaccination-polio vaccination
-measles-measles
-MMR-MMR
-hepatitis B-hepatitis B
presently no evidence for association with GBSpresently no evidence for association with GBS
Guillaine Barre SyndromeGuillaine Barre Syndrome
Antecedent EventsAntecedent Events
-surgery-surgery
-epidural anesthesia-epidural anesthesia
-renal transplantation-renal transplantation
-bone marrow -bone marrow transplantationtransplantation
-SLE-SLE
-sarcoidosis-sarcoidosis
-lymphoma-lymphoma
-snake bite-snake bite
-some drugs-some drugs
Other anectodal associationsOther anectodal associations
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Acute Inflammatory Demyelinating PolyradiculopathyAcute Inflammatory Demyelinating Polyradiculopathy
most commonmost common
PathologyPathology
lymphocytic infiltration of peripheral nerveslymphocytic infiltration of peripheral nerves
macrophage mediated segmental demyelinationmacrophage mediated segmental demyelination
secondary axonal changesecondary axonal change
humoral and cellular immunity implicatedhumoral and cellular immunity implicated
ElectrophysiologyElectrophysiology
segmental deymyelinationsegmental deymyelination
Recovery with remyelinationRecovery with remyelination
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Acute Motor Axonal NeuropathyAcute Motor Axonal Neuropathy
GBS in China (1991, 1992) GBS in China (1991, 1992)
55-65% pure motor axonal neuropathy55-65% pure motor axonal neuropathy
76% positive for 76% positive for C jejuniC jejuni
10-20% of sporadic cases10-20% of sporadic cases
PathologyPathology
anti-GM1, GD1a, GD1banti-GM1, GD1a, GD1b
Wallerian-like degeneration of motor axonsWallerian-like degeneration of motor axons
lengthening Nodes of Ranvierlengthening Nodes of Ranvier
distortion of paranodal myelindistortion of paranodal myelin
periaxonal macrophages periaxonal macrophages
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Acute Motor Axonal NeuropathyAcute Motor Axonal Neuropathy
ElectrophysiologyElectrophysiology
CMAP amplitudes reducedCMAP amplitudes reduced
tendon reflexes, preserved, exaggeratedtendon reflexes, preserved, exaggerated
Rapidly progressive weakness, respiratory failureRapidly progressive weakness, respiratory failure
Usually good recoveryUsually good recovery
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Acute Motor Sensory Axonal NeuropathyAcute Motor Sensory Axonal Neuropathy
Feasby 1986 described 7 patientsFeasby 1986 described 7 patients
severe sensory motor dysfunctionsevere sensory motor dysfunction
marked wasting, poor recoverymarked wasting, poor recovery
PathologyPathology
Wallerian-like degeneration, motor sensory axonsWallerian-like degeneration, motor sensory axons
little demyelination, lymphocytic infiltrationlittle demyelination, lymphocytic infiltration
periaxonal macrophagesperiaxonal macrophages
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Acute Motor Sensory Axonal NeuropathyAcute Motor Sensory Axonal Neuropathy
ElectrophysiologyElectrophysiology
sensory and motor axonal dysfunctionsensory and motor axonal dysfunction
Fulminant diseaseFulminant disease
Slow incomplete recoverySlow incomplete recovery
Most severe form of immune mediated axonal damageMost severe form of immune mediated axonal damage
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Miller Fisher SyndromeMiller Fisher Syndrome
Fisher 1956 described 3 patientsFisher 1956 described 3 patients
ataxia, areflexia, ophtalmoplegiaataxia, areflexia, ophtalmoplegia
5% of patients with GBS5% of patients with GBS
associated with associated with C jejuniC jejuni infection infection
PathologyPathology
anti-GQ1banti-GQ1b
demyelination CN III, VI, spinal ganglia, peripheral demyelination CN III, VI, spinal ganglia, peripheral nervesnerves
ElectrophysiologyElectrophysiology
reduced/absent SNAPsreduced/absent SNAPs
Guillaine Barre SyndromeGuillaine Barre Syndrome
Clinicopathological TypesClinicopathological Types
Other VariantsOther Variants
pure sensorypure sensory
pure dysautonomicpure dysautonomic
pharyngeal-brachial-cervicalpharyngeal-brachial-cervical
parapareticparaparetic
10% of GBS10% of GBS
Guillaine Barre SyndromeGuillaine Barre Syndrome
Electrophysiological FeaturesElectrophysiological FeaturesAIDPAIDP
reduced conduction velocityreduced conduction velocity
conduction block or temporal dispersionconduction block or temporal dispersion
prolonged terminal latencyprolonged terminal latency
absent or prolonged F-waveabsent or prolonged F-wave
AMANAMANabsent or reduced CMAPabsent or reduced CMAP
normal motor latency and NCVnormal motor latency and NCV
normal SNAPnormal SNAP
AMSANAMSANabsent or reduced SNAPabsent or reduced SNAP
absent or reduced CMAPabsent or reduced CMAP
normal motor latency and NCVnormal motor latency and NCV
Guillaine Barre SyndromeGuillaine Barre Syndrome
Criteria for DiagnosisCriteria for Diagnosis
Required CriteriaRequired Criteria
progressive weakness > two limbsprogressive weakness > two limbs
areflexiaareflexia
progression < 4 weeksprogression < 4 weeks
other causes excludedother causes excluded
lead poisoning, vasculitis, botulism, porphyrialead poisoning, vasculitis, botulism, porphyria
Supportive CriteriaSupportive Criteria
mild sensory signsmild sensory signs
albuminocytological dissociationalbuminocytological dissociation
conduction blockconduction block
Guillaine Barre SyndromeGuillaine Barre Syndrome
InvestigationsInvestigations
• cerebrospinal fluidcerebrospinal fluid• antiganglioside antibodiesantiganglioside antibodies• stool culture for C jejunistool culture for C jejuni• antibodies to C jejuni, cytomegalovirus, EBV, HSV, antibodies to C jejuni, cytomegalovirus, EBV, HSV,
HIV, M. pneumoniaeHIV, M. pneumoniae• biochemical screening: urea, electrolytes, liver biochemical screening: urea, electrolytes, liver
enzymesenzymes• full blood countfull blood count• ESRESR• autonomic function testsautonomic function tests• electrophysiologyelectrophysiology
Guillaine Barre SyndromeGuillaine Barre Syndrome
TreatmentTreatment
Good intensive careGood intensive care
Respiratory support Respiratory support
Prophylaxis for dvtProphylaxis for dvt
RehabilitationRehabilitation
Guillaine Barre SyndromeGuillaine Barre Syndrome
TreatmentTreatment
Plasma ExchangePlasma ExchangeBrettle 1978Brettle 1978
done within 2 weeksdone within 2 weeks
reduced period of hospital stay, duration of mechanical reduced period of hospital stay, duration of mechanical ventilation, time to reach ambulationventilation, time to reach ambulation
North American Trial 200-250 ml/kg body weight, 7-14 North American Trial 200-250 ml/kg body weight, 7-14 days days
French Cooperative GroupFrench Cooperative Group
2 exchanges for mild2 exchanges for mild
4 exchanges for moderate to severe4 exchanges for moderate to severe
Guillaine Barre SyndromeGuillaine Barre Syndrome
TreatmentTreatment
Plasma ExchangePlasma Exchange
ComplicationsComplications
hypotensionhypotension
septicemiasepticemia
hypocalcemiahypocalcemia
abnormal clottingabnormal clotting
Guillaine Barre SyndromeGuillaine Barre Syndrome
TreatmentTreatment
Intravenous ImmunoglobulinIntravenous Immunoglobulintx for immunologically mediated disorderstx for immunologically mediated disorders
anti-idiotypic suppression of autoantibodies anti-idiotypic suppression of autoantibodies
Kleyweg et al 1988Kleyweg et al 1988
given within 2 weeksgiven within 2 weeks
as effective as plasma exchangeas effective as plasma exchange
0.4 g/kg body weight for five days0.4 g/kg body weight for five days
Guillaine Barre SyndromeGuillaine Barre Syndrome
TreatmentTreatment
ContraindicationsContraindications•selective IgA deficiencyselective IgA deficiency•Anaphylaxis with previous Anaphylaxis with previous IVIgIVIg
Relative contraindicationsRelative contraindications•severe congestive severe congestive cardiac failurecardiac failure•renal insufficiencyrenal insufficiency
Adverse EffectsAdverse Effects•malaise, myalgia, fever, chillsmalaise, myalgia, fever, chills•vasomotor symptoms, vasomotor symptoms, headacheheadache•nausea, vomitingnausea, vomiting•increase in liver enzymesincrease in liver enzymes•renal tubular necrosis, acute renal tubular necrosis, acute renal failurerenal failure•aseptic meningitisaseptic meningitis•hypercoagulable statehypercoagulable state•anaphylaxisanaphylaxis•rashesrashes•encephalopathyencephalopathy
Intravenous ImmunoglobulinIntravenous Immunoglobulin
Guillaine Barre SyndromeGuillaine Barre Syndrome
Factors Associated with Poor OutcomeFactors Associated with Poor Outcome
EtiologyEtiology
previous GI infectionprevious GI infection
C jejuni infectionC jejuni infection
cytomegaloviruscytomegalovirus
Clinical FeaturesClinical Features
older ageolder age
shorter latency to nadirshorter latency to nadir
longer time to clinical longer time to clinical improvementimprovement
need for mechanical need for mechanical ventilationventilation
greater disability and greater disability and disease severitydisease severity
ElectrophysiologyElectrophysiology
absent or reduced absent or reduced CMAPCMAP
inexcitable nervesinexcitable nerves
Biochemical MarkersBiochemical Markers
Anti-GM1 antibodiesAnti-GM1 antibodies
Neurone specific Neurone specific enolase and S100benolase and S100b
proteins in CSFproteins in CSF