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afya
Myasthenia gravis
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> autoimmune disorder resulting in insufficient functioning acetylcholine receptors Antibodies to acetylcholine receptors are seen in 85-90% of cases (less commonly seen in disease limited to ocular muscles) 2:1 women:men //muscle fatigability// - muscles become progressively weaker during periods of activity and slowly improve after periods of rest: extraocular muscle weakness: diplopia proximal muscle weakness: face, neck, limb girdle ptosis dysphagia ''Associations'' thymomas in 15% autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE thymic hyperplasia in 50-70% ''Investigations'' single fibre electromyography: high sensitivity (92-100%) CT thorax to exclude thymoma CK normal autoantibodies: around 85-90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used anymore due to the risk of cardiac arrhythmia ''Management'' long-acting anticholinesterase e.g. pyridostigmine immunosuppression: prednisolone initially thymectomy ''Management of myasthenic crisis'' plasmapheresis intravenous immunoglobulins
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Neurology