Pre‐ and postsynaptic neuromuscular junction abnormalities in musk myasthenia

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...

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Bibliographic Details
Published in:Muscle & nerve 2010-08, Vol.42 (2), p.283-288
Main Authors: Niks, Erik H., Kuks, Jan B.M., Wokke, John H.J., Veldman, Henk, Bakker, Egbert, Verschuuren, Jan J.G.M., Plomp, Jaap J.
Format: Article
Language:English
Subjects:
Adult
Autoantibodies - immunology
Autoimmune diseases
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Electrophysiology
Female
Fundamental and applied biological sciences. Psychology
histology
Humans
in vitro electrophysiology
Medical sciences
miniature end plate potential
Motor Endplate - immunology
Motor Endplate - physiopathology
Muscle Weakness - immunology
Muscle Weakness - physiopathology
Muscle, Skeletal - immunology
Muscle, Skeletal - physiopathology
Muscular system
MuSK, myasthenia gravis
Myasthenia Gravis - immunology
Myasthenia Gravis - physiopathology
Neurology
neuromuscular junction
Neuromuscular Junction - immunology
Neuromuscular Junction - physiopathology
Peripheral nervous system. Autonomic nervous system. Neuromuscular transmission. Ganglionic transmission. Electric organ
quantal content
Receptor Protein-Tyrosine Kinases - immunology
Receptors, Cholinergic - immunology
Vertebrates: nervous system and sense organs
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Summary: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, 2010
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.21642