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Electromyographic findings in the so-called non-progressive myopathies

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Summary

Electrophysiological findings in 40 cases of non-progressive myopathies are reported, and compared with a group of 20 cases of Duchenne progressive muscular dystrophy and a control group. In all cases the electrophysiological changes were of the mild s.c. myogenic type.

The involvement of proximal and distal muscles was equal without prevalence in proximal muscles as is typical for Duchenne's dystrophy.

EMG reexaminations showed a slight progression of the diseases. A peculiar feature of myotubular myopathy was spontaneous activity (fibrillation) in 70% of muscles.

A myogenic character of the process of congenital deficiency of muscle innervation with preserved number of motor units is suggested.

Zusammenfassung

Es werden die elektrophysiologischen Befunde in 40 Fällen von nichtprogredienten Myopathien mitgeteilt und mit denjenigen bei einer Gruppe von 20 Fällen von Duchennescher progressiver Muskeldystrophie und einer Kontrollgruppe verglichen. In allen 40 Fällen fanden sich elektrophysiologisch Befunde im Sinne eines diskreten myogenen Befalles.

Der Befall von proximalen und distalen Muskeln war etwa gleich ausgeprägt ohne die für einen Duchenne typische Bevorzugung proximaler Muskeln.

Bei einem später durchgeführten Kontroll-EMG zeigte sich ein leichtes Fortschreiten der Erkrankungsfälle. Eine Besonderheit der „myotubular myopathy“ war das Auftreten von Spontanaktivität (Fibrillationen) in 70% der Muskeln.

Ein myogener Charakter des Prozesses bei der kongenitalen Insuffizienz der Muskelinnervation mit erhaltener Anzahl der motorischen Einheiten wird vermutet.

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Authors and Affiliations

  1. Department of Neurology, Medical Academy, Warsaw

    Irena Hausmanowa-Petrusewicz & Barbara Ryniewicz

Authors
  1. Irena Hausmanowa-Petrusewicz
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  2. Barbara Ryniewicz
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The study was supported by agreement No. 05-002-1 with N.I.H. Bethesda.

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Hausmanowa-Petrusewicz, I., Ryniewicz, B. Electromyographic findings in the so-called non-progressive myopathies. J. Neurol. 211, 241–251 (1976). https://doi.org/10.1007/BF00313234

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  • DOI: https://doi.org/10.1007/BF00313234

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