ABSTRACT

Cause is autoimmune attack at the NMJ resulting in failure of the nerve ■ impulse to generate an effective postsynaptic response. Causative agents identifi ed are: ■

antibodies to the nicotinic ACh receptor at the NMJ ❚ antibodies to a muscle-specifi c tyrosine kinase (MuSK) which mediates ❚ AChR clustering*

antibodies to other proteins which mediate normal NMJ transmission. ❚ Diagnosis includes: ■

detection of circulating antibodies to AChR or MuSK ❚ detection of antibodies to other proteins, e.g. actin ❚ electrodiagnostic tests of NMJ function ❚ edrophonium, a short-acting anticholinesterase, which causes transient ❚ improvement.