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Guillain-Barre’ Syndrome

Guillain-Barre’ Syndrome

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Guillain-Barre’ Syndrome

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  1. Guillain-Barre’ Syndrome

  2. 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 Guillaine Barre Syndrome

  3. Epidemiology • 1-3/100,000 - Europe, USA, Australia • -occurs in any age group • -bimodal age distribution • -increase incidence with age • -males > females • -seasonal variations • -lower during pregnancy, increases after delivery Guillaine Barre Syndrome

  4. Clinical Features • -antecedent event • respiratory infection 40% • gastroenteritis 20% • -common manifestations • proximal limb weakness • facial palsy • sensory symptoms 80% • pain 90% • autonomic dysfunction 66% Guillaine Barre Syndrome

  5. Motor Dysfunction symmetrical limb weakness neck muscle weakness respiratory weakness CN III-VII, IX-XII palsies areflexia wasting Sensory dysfunction pain numbness, paresthesias loss of position sense, vibration, touch, pain ataxia Autonomic dysfunction sinus tachycardia/bradycardia hypertension postural hypotension fluctuations, PR and BP tonic pupils hypersalivation anhidrosis or excess sweating urinary sphincter disturbance constipation gastric dysmotility abnormal vasomotor tone Other papilloedema Clinical Features Guillaine Barre Syndrome

  6. Clinical Features • -onset is acute or subacute • nadir 12 days • improvement 28 days • recovery 200 days • -plateau phase reached 4 weeks from onset in 98% of patients • -plateau duration 12 days Guillaine Barre Syndrome

  7. Antecedent Events • Campylobacter jejuni • -most common pathogen • 26-30% USA and Europe • 45% Japan • -AMAN and AMSAN more common • -common preceding infection in Miller Fisher Syndrome • Pathogenesis • ”molecular mimicry” • GM1 ganglioside Guillaine Barre Syndrome

  8. Antecedent Events • Cytomegalovirus • -second most common • 5% Japan • 11-13% Europe • -more common in females and young age groups • -severe course, with respiratory difficulties • -reduced SNAPs are more common • Pathogenesis • ”molecular mimicry” • GM2 ganglioside Guillaine Barre Syndrome

  9. Antecedent Events • Associated infections • Epstein Barr (10%) • Mycoplasma pneumoniae (5%) • HIV • Lyme disease • Possibly Associated infections • hepatitis A, B, C, and D • typhoid • falciparum malaria Guillaine Barre Syndrome

  10. Antecedent Events • Vaccines? • -influenza vaccine • -polio vaccination • -measles • -MMR • -hepatitis B • presently no evidence for association with GBS Guillaine Barre Syndrome

  11. -surgery -epidural anesthesia -renal transplantation -bone marrow transplantation -SLE -sarcoidosis -lymphoma -snake bite -some drugs Antecedent Events Other anectodal associations Guillaine Barre Syndrome

  12. Clinicopathological Types • Acute Inflammatory Demyelinating Polyradiculopathy • most common • Pathology • lymphocytic infiltration of peripheral nerves • macrophage mediated segmental demyelination • secondary axonal change • humoral and cellular immunity implicated • Electrophysiology • segmental deymyelination • Recovery with remyelination Guillaine Barre Syndrome

  13. Clinicopathological Types • Acute Motor Axonal Neuropathy • GBS in China (1991, 1992) • 55-65% pure motor axonal neuropathy • 76% positive for C jejuni • 10-20% of sporadic cases • Pathology • anti-GM1, GD1a, GD1b • Wallerian-like degeneration of motor axons • lengthening Nodes of Ranvier • distortion of paranodal myelin • periaxonal macrophages Guillaine Barre Syndrome

  14. Clinicopathological Types • Acute Motor Axonal Neuropathy • Electrophysiology • CMAP amplitudes reduced • tendon reflexes, preserved, exaggerated • Rapidly progressive weakness, respiratory failure • Usually good recovery Guillaine Barre Syndrome

  15. Clinicopathological Types • Acute Motor Sensory Axonal Neuropathy • Feasby 1986 described 7 patients • severe sensory motor dysfunction • marked wasting, poor recovery • Pathology • Wallerian-like degeneration, motor sensory axons • little demyelination, lymphocytic infiltration • periaxonal macrophages Guillaine Barre Syndrome

  16. Clinicopathological Types • Acute Motor Sensory Axonal Neuropathy • Electrophysiology • sensory and motor axonal dysfunction • Fulminant disease • Slow incomplete recovery • Most severe form of immune mediated axonal damage Guillaine Barre Syndrome

  17. Clinicopathological Types • Miller Fisher Syndrome • Fisher 1956 described 3 patients • ataxia, areflexia, ophtalmoplegia • 5% of patients with GBS • associated with C jejuni infection • Pathology • anti-GQ1b • demyelination CN III, VI, spinal ganglia, peripheral nerves • Electrophysiology • reduced/absent SNAPs Guillaine Barre Syndrome

  18. Clinicopathological Types • Other Variants • pure sensory • pure dysautonomic • pharyngeal-brachial-cervical • paraparetic • 10% of GBS Guillaine Barre Syndrome

  19. Electrophysiological Features • AIDP • reduced conduction velocity • conduction block or temporal dispersion • prolonged terminal latency • absent or prolonged F-wave • AMAN • absent or reduced CMAP • normal motor latency and NCV • normal SNAP • AMSAN • absent or reduced SNAP • absent or reduced CMAP • normal motor latency and NCV Guillaine Barre Syndrome

  20. Criteria for Diagnosis • Required Criteria • progressive weakness > two limbs • areflexia • progression < 4 weeks • other causes excluded • lead poisoning, vasculitis, botulism, porphyria • Supportive Criteria • mild sensory signs • albuminocytological dissociation • conduction block Guillaine Barre Syndrome

  21. Investigations • cerebrospinal fluid • antiganglioside antibodies • stool culture for C jejuni • antibodies to C jejuni, cytomegalovirus, EBV, HSV, HIV, M. pneumoniae • biochemical screening: urea, electrolytes, liver enzymes • full blood count • ESR • autonomic function tests • electrophysiology Guillaine Barre Syndrome

  22. Treatment • Good intensive care • Respiratory support • Prophylaxis for dvt • Rehabilitation Guillaine Barre Syndrome

  23. Treatment • Plasma Exchange • Brettle 1978 • done within 2 weeks • reduced period of hospital stay, duration of mechanical ventilation, time to reach ambulation • North American Trial 200-250 ml/kg body weight, 7-14 days • French Cooperative Group • 2 exchanges for mild • 4 exchanges for moderate to severe Guillaine Barre Syndrome

  24. Treatment • Plasma Exchange • Complications • hypotension • septicemia • hypocalcemia • abnormal clotting Guillaine Barre Syndrome

  25. Treatment • Intravenous Immunoglobulin • tx for immunologically mediated disorders • anti-idiotypic suppression of autoantibodies • Kleyweg et al 1988 • given within 2 weeks • as effective as plasma exchange • 0.4 g/kg body weight for five days Guillaine Barre Syndrome

  26. Contraindications selective IgA deficiency Anaphylaxis with previous IVIg Relative contraindications severe congestive cardiac failure renal insufficiency Adverse Effects malaise, myalgia, fever, chills vasomotor symptoms, headache nausea, vomiting increase in liver enzymes renal tubular necrosis, acute renal failure aseptic meningitis hypercoagulable state anaphylaxis rashes encephalopathy Treatment Intravenous Immunoglobulin Guillaine Barre Syndrome

  27. Etiology previous GI infection C jejuni infection cytomegalovirus Clinical Features older age shorter latency to nadir longer time to clinical improvement need for mechanical ventilation greater disability and disease severity Electrophysiology absent or reduced CMAP inexcitable nerves Biochemical Markers Anti-GM1 antibodies Neurone specific enolase and S100b proteins in CSF Factors Associated with Poor Outcome Guillaine Barre Syndrome