Fluctuating muscle weakness (proximal limb, trunk, ocular, e.g. ptosis/diplopia or bulbar) with fatigability, respiratory muscles may also be involved
Steroids indicated (oral or i.v.)
Pyridostigmine initial dose 30 mg
Neurological consult
If no improvement, or worsening, plasmapheresis or IVIG may be considered
Avoid certain medications, that may precipitate cholinergic crisis (e.g. ciprofloxacin, beta blockers, amikacin, benzodiazepines and above all curares during general anesthesia)’ )
Check for ocular muscle and proximal muscle fatigability
AChr and MuSK antibodies
Besides tests, e.g. Tensilon test or ice pack test with neurological input
Repetitive nerve stimulation and single fibre EMG
Exclude myocarditis with cardiac enzymes (and cardiac MRI in case of doubt) (cave pseudo myasthenic myositis)
Rhumato-onco taskforce KBVR/SRBR (Yves Piette, Ellen Delanghe) Gauthier Remiche, ULB Erasme
Olivier Lambotte, AP-HP, Hôpital Bicêtre, Service de Médecine
Interne et Immunologie Clinique, Paris, France,
Dimitri Psimaras, Practicien Hospitalier
Département de Neurologie Mazarin, GHPS, Paris, France
Abbrevations