Journal
MUSCLE & NERVE
Volume 42, Issue 2, Pages 283-288Publisher
WILEY-BLACKWELL
DOI: 10.1002/mus.21642
Keywords
histology; in vitro electrophysiology; MuSK, myasthenia gravis; neuromuscular junction; quantal content; miniature end plate potential
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Funding
- Prinses Beatrix Fonds
- Stichting Willem H. kroger
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Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle nerve contact. Muscle Nerve 42:283-288, 2010
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