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2463807169150Management00Management15519407169150Mechanical ventilation in ?IVIG 2g/kg for 5/7 or plasma exchange / plasmapheresisAvoid: suxamethonium (associated with sudden death) – completely contraindicated00Mechanical ventilation in ?IVIG 2g/kg for 5/7 or plasma exchange / plasmapheresisAvoid: suxamethonium (associated with sudden death) – completely contraindicated45523157168515Acetylcholinesterase inhibitors: Pyridostigmine: 60-90mg PO Q4h (if dose missed, give double dose) Neostigmine: 0.5-2mg IV for crisisEdrophonium: for crisis; 1-1-2mg IV slow pushOthers: prednisone 100mg/day; cytotoxics effective in 80%; thymectomy in all; plasmapheresis/immunoglobulin if severe respiratory problemsAvoid: gentamicin, metronidazole, fluroquinolones, penicillamine, beta blockers, calcium channel blockers, quinidine, neuromuscular blockers (unpredictable response to suxamethonium; resistant – so need to double dose), benzodiazepines, barbiturates, lithium, phenytoin, amitriptyline, haloperidol, chlorpromazine00Acetylcholinesterase inhibitors: Pyridostigmine: 60-90mg PO Q4h (if dose missed, give double dose) Neostigmine: 0.5-2mg IV for crisisEdrophonium: for crisis; 1-1-2mg IV slow pushOthers: prednisone 100mg/day; cytotoxics effective in 80%; thymectomy in all; plasmapheresis/immunoglobulin if severe respiratory problemsAvoid: gentamicin, metronidazole, fluroquinolones, penicillamine, beta blockers, calcium channel blockers, quinidine, neuromuscular blockers (unpredictable response to suxamethonium; resistant – so need to double dose), benzodiazepines, barbiturates, lithium, phenytoin, amitriptyline, haloperidol, chlorpromazine2444756143625Investigation00Investigation45504106143625Edrophonium test: prompt improvement (caution in cardiac disease eg. AVB, AF, bradycardia)Electrophysiological tests00Edrophonium test: prompt improvement (caution in cardiac disease eg. AVB, AF, bradycardia)Electrophysiological tests15500356143625CSF: protein >0.4 (in 70% by 3/7, 90% by 1/52); normal CSF cell countBloods: ABGOther: respiratory function tests; electrophysiological studies00CSF: protein >0.4 (in 70% by 3/7, 90% by 1/52); normal CSF cell countBloods: ABGOther: respiratory function tests; electrophysiological studies2451104225290Assessment00Assessment45504104225925History: worse in evenings; gradual onset; weakness especially arms, facial bulbar, proximal limbs; 20% in extraocular only (ptosis, strabismus); CN III, IV, VI, INO; difficulty chewing, loss of facial expression; inducible fatigue; usually mild-modNo sensory / reflex / cerebellar symptoms00History: worse in evenings; gradual onset; weakness especially arms, facial bulbar, proximal limbs; 20% in extraocular only (ptosis, strabismus); CN III, IV, VI, INO; difficulty chewing, loss of facial expression; inducible fatigue; usually mild-modNo sensory / reflex / cerebellar symptoms15500354225926History: paraesthesia of hands and feet progressive ascending symmetrical motor weakness of >1 limb developing over days-weeks; recovery over 2-4/52 after cessation of progressionExamination: minor glove stocking sensory loss; areflexia (may take a few days to appear); limb pain on passive movement; CN involved in 50% (VII most common), extraocular muscles in MFS (ataxia, areflexia, external opthalmoplegia); autonomic dysfunction (poor prognosis; associated with SIADH, ileus, retention)00History: paraesthesia of hands and feet progressive ascending symmetrical motor weakness of >1 limb developing over days-weeks; recovery over 2-4/52 after cessation of progressionExamination: minor glove stocking sensory loss; areflexia (may take a few days to appear); limb pain on passive movement; CN involved in 50% (VII most common), extraocular muscles in MFS (ataxia, areflexia, external opthalmoplegia); autonomic dysfunction (poor prognosis; associated with SIADH, ileus, retention)15519402746375Occurs 1-4/52 after precipitating event<5% follow surgical proceduresInfection: campylobacter jejuni (associated with Miller Fischer Syndrome (MFS); found in 25% cases; confirmed by serological testing as stool cultures negative by time of presentation), CMV, EBV, HIV, vacciniaOthers: lymphoma, SLE00Occurs 1-4/52 after precipitating event<5% follow surgical proceduresInfection: campylobacter jejuni (associated with Miller Fischer Syndrome (MFS); found in 25% cases; confirmed by serological testing as stool cultures negative by time of presentation), CMV, EBV, HIV, vacciniaOthers: lymphoma, SLE4552315274700975% have thymic hyperplasia; 10% have thymomaMay be post-infectiousMyasthenic crisis: disease exacerbation / insufficient treatment; symptoms resolve with edrophonium0075% have thymic hyperplasia; 10% have thymomaMay be post-infectiousMyasthenic crisis: disease exacerbation / insufficient treatment; symptoms resolve with edrophonium2451102747010Aetiology00Aetiology2451101948180Patho-physiology00Patho-physiology45504101948180AutoimmuneAntibodies to acetylcholine receptors in skeletal muscle in 90%00AutoimmuneAntibodies to acetylcholine receptors in skeletal muscle in 90%15500351948180Acute demyelinating polyneuropathyImmune mediated attack on myelin sheath of peripheral nerves may cause secondary axonal degeneration with more prolonged recovery00Acute demyelinating polyneuropathyImmune mediated attack on myelin sheath of peripheral nerves may cause secondary axonal degeneration with more prolonged recovery1550035330200GBS00GBS4550410330200Myasthenia Gravis00Myasthenia Gravis1550035964565Most common cause of rapid onset paralysis in healthy people1/100,0000/month85% make complete / nearly complete recovery3-4% mortality rate00Most common cause of rapid onset paralysis in healthy people1/100,0000/month85% make complete / nearly complete recovery3-4% mortality rate4551045964565Incidence 1:20,000Female > malePeak onset 20’s for women, 60’s for men00Incidence 1:20,000Female > malePeak onset 20’s for women, 60’s for men245110965200Epidemiology00Epidemiology369697012068810Symptoms: monocular visual loss (amourosis fugax)00Symptoms: monocular visual loss (amourosis fugax)316357012068810Opthalmic00Opthalmic ................
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