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Adult onset centronuclear myopathy with peripheral nerve involvement

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Summary

A case of centronuclear myopathy is presented, showing a rapid course in a 47-year-old male. Clinical and muscular findings were consistent with the diagnosis of centronuclear myopathy. On nerve biopsy, there was evidence of a regenerative process. Electron microscopy showed proliferation of Schwann cells with numerous Büngner's bands and other anomalies. This rare case with peripheral nerve changes may provide evidence to support the hypothesis of peripheral nerve involvement in centronuclear myopathy.

Zusammenfassung

Es wird ein Fall von zentronukleärer Myopathie, die bei einem 47jährigen Patienten einen raschen Krankheitsverlauf nahm, beschrieben. Klinische und muskelbioptische Befunde waren identisch mit denen bei bisher veröffentlichten Fällen. Die Nervenbiopsie ergab das Vorhandensein eines Regenerationsprozesses. Die Elektronenmikroskopie zeigte eine Vermehrung der Schwannzellen mit zahlreichen Büngnerschen Bändern und andere Anomalien. Ein solcher Fall mit Degeneration der peripheren Nerven ist selten in der Literatur und kann als Stütze für die Hypothese gelten, daß bei zentronukleärer Myopathie eine Störung des peripheren Nervensystems vorliegt.

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Author notes

  1. N. Moriyama

    Present address: Dept. of Neuropsychiatry, School of Medicine, Kuyshu University, 812, Fukuoka, Japan

Authors and Affiliations

  1. Service des Maladies Nerveuses, Hôpital Sainte-Marguerite, 270, Boulevard de Sainte-Marguerite, 13009, Marseille, France

    P. Mouren, Y. Poinso, N. Moriyama & A. Mouren

  2. Laboratoire de Neuropathologie, Faculté de Médecine, Boulevard Jean Moulin, 13006, Marseille, France

    J.-F. Pellissier

Authors
  1. P. Mouren
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  2. Y. Poinso
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  3. J.-F. Pellissier
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  4. N. Moriyama
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  5. A. Mouren
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Mouren, P., Poinso, Y., Pellissier, JF. et al. Adult onset centronuclear myopathy with peripheral nerve involvement. J Neurol 228, 147–159 (1982). https://doi.org/10.1007/BF00313727

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  • DOI: https://doi.org/10.1007/BF00313727

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