PRE- AND POSTSYNAPTIC NEUROMUSCULAR JUNCTION ABNORMALITIES IN MuSK MYASTHENIA

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

*Corresponding author for this work

    Research output: Contribution to journalArticleAcademicpeer-review

    45 Citations (Scopus)

    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

    Original languageEnglish
    Pages (from-to)283-288
    Number of pages6
    JournalMUSCLE & NERVE
    Volume42
    Issue number2
    DOIs
    Publication statusPublished - Aug-2010

    Keywords

    • 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

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