5. Levin KH. The Neurologist 2004;10: 61–74) Currently recognized forms of GB Syndrome AMSAN AMAN Axonal forms Miller Fischer syndrome Pure autonomic form Pure sensory form Facial diplegia with paresthesia Parapretic Pharyngo-cervico-brachial Regional presentations of AIDP Preserved reflexes Prominent sensory loss Pure motor Asymmetric Atypical forms of AIDP AIDP
9. Other Antecedent factors Haber P et al. JAMA 2004; 292: 2478–81. Souayah N et al. Vaccine 2007; 25: 5253–55. Pritchard J et al. J Neurol Neurosurg Psychiatry 2002; 73: 348–49. In a patient with h/o GB syndrome any future vaccination should be done with due deligence, for fear of relapse. Other physical stress SLE Surgery HIV seropositivity Thrombolysis Lymphoma Vaccines: Influenza, hepatitis, rabies (old type), tetanus
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25. AIDP AMAN Other axonal forms Schwann cell membrane Preterminal motor endings Axonal membrane Demyelination, Conduction block, Sec Axonal degen Degeneration of motor endings Axonal degeneration Slow Regeneration Rapid Regeneration Remyelination Rapid recovery Rapid recovery Slow Recovery
42. Diagnostic Criteria for GB Syndrome ºExcluding M. Fisher and other variant syndromes Modified from AK Asbury, DR Cornblath: Ann Neurol 1990; 27: S21, 1990. 6. Electrophysiologic evidence of demyelination 3. Facial or other cranial nerve involvement 5. Typical CSF profile (cytoalbumin dissociation) 2. Mild sensory involvement 4. Absence of fever 1. Relatively symmetrical weakness Supportive 4. Exclusion of other causes [e.g., vasculitis, toxins, botulism, diphtheria, porphyria, localized spinal cord or cauda equina syndrome] 3. Disease course < 4 weeks 2. Areflexia 1. Progressive weakness of 2 or more limbs due to neuropathyº Required
64. Recent trials Of IVIg No significant difference in the outcome. IVIg 0.4 g/kg/d X 5d vs PE total 200- 250ml/kg In upto 7 sessions over 4 Wks. N=47 Adults Nomura et al. 2000 Early relapse more in 2 d group. No other differences IVIg 1g/kg/day X 2 day Vs 0.4g/kg/day X 5 day. N=50. Children Able to walk without aid. Korinthenberg et al. 2005 No significant difference in the disability score. IVIg 0.5 g/kg/day X 2 days Vs supportive care. N=21. Children Able to walk without aid. Korinthenberg et al. 2005 Result Treatment Subjects Study
65. Recent trials Of IVIg IVIg and PE were more effective than steroids (Dexa). Steroids Vs Steroids + IVIg vs Steroids + PE N= 54 Children. Wang et al. 2001 6d group appeared to benefit, but not statistically significant. IVIg 0.4g/kg/day X 3 days Vs 6 days. N= 39. Adults with CI to PE. Raphael et al. 2001 IVIg and PE were equally effective. IVIg 0.4 g/kg/dX 5d vs PE 40-50ml/kg 5 times in 2 weeks vs immuno-absorption 5 times in 2 weeks. N= 67. Adults and children Diener et al. 2001 Result Treatment Subjects Study
66. Recent trials Of Steroids Steroids (oral, parenteral) alone are not beneficial in GBS. Hughes RA et al. Cochrane Database Syst Rev 2006; 2: CD001446. No difference in outcome. IVIg 0.4g/kg/d X 5d + IV Methylprednisolone 500mg/d for 5 day vs IVIg + placebo. N= 225 Van Koningsveld et al., 2004 No difference in outcome. Prednisone 60 mg/d X 4d, 45mg/d X 3d, 30 mg/d X 10d, then tapered and stopped Vs no treatment N= 20 Bansal et al. 2004 Result Treatment Subjects Study
82. Koeppen S et al. Neurocrit. Care 2006;05:235–242. 75% sensory 43% muscle weakness 13% orthostatic hypotension 48% residual neuropathy 1-14 40 de la Cour and Jakobsen, 2005 28% normal/ minor symptoms 24% unassisted gait 12% assisted gait 24% wheel chair/ bed bound 1 25 Cheng et al. 2004 31% residua 1 96 Cheng et al. 2003 48% muscle aches and cramps (38% in UL, 66% in LL) 69% sensory 3-6 yrs 122 Bernsen et al. 2001 4% moderate, 6% severe 42% mild residual symptoms 1 yr 53 Chang et al. 2000 Outcome Duration after onset No Author Long-term Neurological outcome in GB syndrome
83. GBS Disability Scale (modified) Most commonly used measure of levels of activity and participation. Plasma Exchange/Sandoglobulin GBS Trial Group, 1997 Death. 6 Requiring assisted ventilation ( for any part of the day or night). 5 Confined to bed or chair bound. 4 Able to walk with a stick, appliance or support (5m across an open space). 3 Able to walk without support of a stick (5m across an open space) but incapable of manual work/running. 2 Minor symptoms or signs of neuropathy but capable of manual work/capable of running. 1 Healthy. 0
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Editor's Notes
(B) Longitudinal section of the cauda equina. The nodes of Ranvier are stained selectively with protein G (arrowheads). (D) Wallerian-like degeneration of nerve fibers. Sciatic nerve cross section with toluidine blue stain. Myelin ovoids produced by Wallerian like degeneration of myelinated fibers are present. (B) Longitudinal section of the cauda equina. The nodes of Ranvier are stained selectively with protein G (arrowheads). (D) Wallerian-like degeneration of nerve fibers. Sciatic nerve cross section with toluidine blue stain. Myelin ovoids produced by Wallerian like degeneration of myelinated fibers are present.
(A) Longitudinal sections of rabbit ventral roots immunolabeled for voltage-gated Na (Nav) channels at nodes (red), contactin associated protein (Caspr) at paranodes (green), and the membrane attack complex (MAC) (blue). MAC staining appears at nodes in the acute phase. As MAC deposition spreads, Nav channels and Caspr become markedly disrupted, finally disappearing. (B) Nav channels located at the nodes form multiprotein complexes. Caspr forms axo-glial junctions at paranodes, which act as a diffusion barrier restricting the lateral mobility of nodal Nav channels. (C) Anti-GM1 IgG Ab cause complement-mediated attack with MAC at the nodal and paranodal axolemmas. Nav channel clusters are altered by the destruction of structures that mediate their stabilization.
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