Myastenia gravis

Symptom Grade

Fluctuating muscle weakness (proximal limb, trunk, ocular, e.g. ptosis/diplopia or bulbar) with fatigability, respiratory muscles may also be involved

Management escalation pathway

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)’ )

Assessment and Investigations

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)

In collaboration with

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



ACPA: Anti Citrullinated Antibody
ANF: Anti Nuclear Factor
C3/C4: Complement Factor 3 or 4
CK: Creatine Kinase
CK MB: Creatine Kinase Muscle
ECG: ElectroCardioGram
EMG: ElectroMyoGram
ICPi: Immune Checkpoint Blockade Inhibition
IVIg: IntraVeneous Immunoglobulins
MRI: Magnetic Resonance Imaging
PD: Per Day
PO: Per Os
Pro-BNP: Pro-Brain Natriuretic Peptide
RF: Rheuma Factor
ULN: Upper Limit of Normal