Guillain-Barre Syndrome

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Guillain-Barre Syndrome (GBS)/Acute inflammatory demyelinating polyneuropathy

Acute onset severe progressive symmetrical inflammatory polyneuropathy; (Pronounced “ghee-lain” or “ge-yan”); in 70% - develops 1-4 weeks after respiratory infection or diarrhea. More common <30 years. Incidence of 1-2/100 000/year.Pathologically – considered an autoimmune response to viral infection  multifocal inflammatory segmental demyelinationClinical features: History of viral respiratory or gastrointestinal infection (usually clears before onset of neurological symptoms). Rapidly progressive ascending symmetric muscle weakness (progresses to paralysis) – usually starts in lower limbs with numbness or tingling  then upper. Typically develops over a few days; Maximum weakness after about 3 to 4 weeks. Distal pain and parathesia (feet and hands) may precede weakness. No or mild sensory loss. Reflexes lost (normal for first few days). Muscles may be painful on palpation. 20-30% develop respiratory weakness – can develop very rapidly. CSF protein is increased from, usually, 2nd week.Four clinical variants generally recognised – Ascending GBS; Descending GBS; Miller-Fisher variant (may not be a form of GBS, but is related to brain stem encephalitis); and pure motor GBSDifferential diagnosis – poliomyelitis, botulism, heavy metal poisoning, polymyositisTreatment: Most need hospitalisation. Respiratory function monitoring  often needs intubation and ventilatory support; prevention of DVT; Plasma exchanges helpful if given early; intravenous immunoglobulin; Steroids are not indicated; Physiotherapy and occupational therapy for rehabilitation 3-6% have a relapse or second episode

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