PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

Author(s):
Niks, E.H. (0000-0001-5892-5143); Kuks, J.B.M.; Wokke, J.H.J.; Veldman, H.; Bakker, E. (0000-0002-2843-7357); Verschuuren, J.J.G.M. (0000-0002-4572-1501); Plomp, J.J. (0000-0002-8427-9090)
Published
2010
Publication type
Article / Letter to editor

Abstract

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

histology in vitro electrophysiology MuSK, myasthenia gravis neuromuscular junction quantal content miniature end plate potential muscle-specific kinase acetylcholine-receptor antibodies amyotrophic-lateral-sclerosis gravis agrin lrp4 transmission release complex tid1
Date issued
2010-08-31
Language
English
Is part of
Muscle and Nerve (2010), Vol. 42, no. 2, p. 283-288
hdl
1887/100768
doi
10.1002/mus.21642