<<forEachTiddler where 'tiddler.tags.contains(context.inTiddler.title)'
sortBy
tiddler.title
ascending
write '"*[["+tiddler.title+"]]\n"'>>
<<tiddler AutoRefresh with: force>>
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