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  Vol. 33 No. 9, September 1976 TABLE OF CONTENTS
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Regeneration in Duchenne Muscular Dystrophy

Electromyographic Evidence

John E. Desmedt, MD; Samuel Borenstein, MD

Arch Neurol. 1976;33(9):642-650.


Abstract

• Eight patients with Duchenne muscular dystrophy (DMD) and seven normal children of similar age were studied with a new electromyographic method for coherent displays of potentials. Five hundred motor unit potentials (MUPs) were analyzed in 20 proximal and distal muscles representing a wide spectrum of dystrophy. The progressive MUP disintegration by dropping out of muscle fibers was documented, as well as a high incidence of spontaneous fibrillation. A total of 386 late component (LC) potentials followed the 500 MUPs at consistent latencies. No LC occurred in normal children. The LCs result from motor axon sprouts innervating muscle fibers that are newly formed either by segmentation of existing muscle fibers (focal necrosis and membrane repair) or by muscle regeneration. Axons in DMD can thus collaterally innervate additional muscle fibers. These processes must delay the onset and progression of clinical weakness in DMD patients.



Author Affiliations

From the Brain Research Unit of the University of Brussels.


Footnotes

Accepted for publication Nov 11, 1975.

Reprint requests to Brain Research Unit, 115 Bd de Waterloo, B 1000 Brussels, Belgium (Prof Desmedt).



THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Open-Biopsy Electromyography: Direct Correlation of a Pattern of Excessively Recruited, Pathologically Small Motor Unit Potentials With Histologic Evidence of Neuropathy
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Arch Neurol 1979;36:406-409.
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Morphological Changes of Motor Units in Duchenne's Muscular Dystrophy
Coers and Telerman-Toppet
Arch Neurol 1977;34:396-402.
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