Anticholinesterase drugs should be given first in the management of
MG, but with some caution in patients with MuSK antibodies (good practice point).
Plasma exchange is recommended in severe cases to induce remission and in prepa-
ration for surgery (recommendation level B). IvIg and plasma exchange are effective
for the treatment of MG exacerbations (recommendation level A). For patients with
non-thymomatous MG, thymectomy is recommended as an option to increase the
probability of remission or improvement (recommendation level B). Once thymoma is
diagnosed, thymectomy is indicated irrespective of MG severity (recommendation
level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are
necessary (good practice point). When long-term immunosuppression is necessary,
azathioprine is recommended to allow tapering the steroids to the lowest possible dose
whilst maintaining azathioprine (recommendation level A). 3,4-Diaminopyridine is
recommended as symptomatic treatment and IvIG has a positive short-term effect in
LEMS (good practice point). Neuromyotonia patients should be treated with an an-
tiepileptic drug that reduces peripheral nerve hyperexcitability (good practice point).
For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying
tumour is essential (good practice point). Immunosuppressive treatment of LEMS and
neuromyotonia should be similar to MG (good practice point).