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Pre‐ and postsynaptic neuromuscular junction abnormalities in musk myasthenia

2010; Wiley; Volume: 42; Issue: 2 Linguagem: Inglês

10.1002/mus.21642

1097-4598

Erik H. Niks, Jan B. M. Kuks, John H. J. Wokke, Henk Veldman, Egbert Bakker, Jan J.G.M. Verschuuren, Jaap J. Plomp,

Cancer Treatment and Pharmacology

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.