dysftinction at onset or its peialstence thereon 3 Presence of polymorphonuclear leucocytes or >50 mononuclear leucocytea per cmm of CSF 4 Stiarp sensory level D Features excluding the diagnosis 1 Diagnosis of acute iniermittent porphyrla or recent diphtheria infection 2 Diagnosis of bolulisrn. poliomyeHis, hyslenca! paralyals, loxlc neuropathy 3 Purely sensory aynaoma COURSE - Within a period of 3 weeks, ie prog/asses la rra-lmjrrp dlaabilfly, often with complete quadriparesis and resplraiory paralysis Recovery without sighnifecant disabilliy occurs In aboul 80%. subsequent relapses occur in about 5% of patlenfs Management - No apeclfc therapy known 1 ITU - as long as any weakness of trunk muscles persists Vital capacity should be measured every 2-4 hours in earlysiages 2 Analgesics -Aspirin or paracesamol. 3 Hot packs - as in Ireatrrenf ol polio-myelitis, may be useful 4 Conlcosteroicfe -lorpalients wllhbuttiar sympioms or severe generalised weakness. 5 Antiblotics - to prevent secondary Infection S Supportive treaiment - IV or Iniragastric feeding may be necessary 7 Physiotherapy - Early active and passive movements Spllniaio prevent foot and wrist drop 8 Asaeled regpiralion -if respiratory or bulbar paralyse 9 Plasma exchange! - Indtcatlong -inafaitilyto walk unaided, rapid and signHicanl reduction in aenal vllal capacity measuramanla and onset d bulbar paraJysE Usually 4 lo 5 sessions ol PE are carried oul over 14 days, at each ol whtch about 50 ml/kg Is exchanged 10 IV immunoglobulin - may be used as alternative to plasma exchangs Dosa 04g/kg/day for 5 days Effective In reducing the disability in the disease 11 Combinaiton therapy - In contrast to PE. IV Ig therapy has the advantage of other madications being givan simultaneously (In PE cd-medications are removed jointly with (he exchange). Hence IVIg therapy has been combined with high dose IV methylprednfeolone(500mg daily for 5 days) 12 Llquorpheresis (CSF fillration) - Is a technique to purify CSF Irom pathological laciora (probably responsible for manifestations of GBS) II has been postulated that pathological cellular or humorat factors are corcflntrated in the CSF and these block sodium channels and hence liquorpheresis is attempted as a direct therapeutic intervention MiHer-Fisher syndrome - te characterised by ophthalmoplegia. ataxia and areflexia without weakness II Is considered lo be a variani of GE syndrome Anlibodles to ganglioslfb GQ 1b are seen it 90% of patients Chronic inflammafory demyellnatingi pofyneuro0alhy (GlDP) - e a subacute orcnront progressive dernyelinaiina neuropatny Prograssion over 2 months distinguishes it from G-B syndrome The disorder te associated with Increased frequercy of HLA DR3. indicating that the disease may result from an aberrant Immune response leading to a chronic form of G-B syndrome (iii) Vascutic; neuropathies - In systemic disorders such as RA and PAN whish produce a number oC different lesions within the nerve and are hence termed multifocal Because of the multifocal onset. the cornbinaiion of Individual pertpheral n invotvement is called mononeumis multiplex' Diagnosls - Hiqh ESR or positive
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