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Neuropediatrics 1977; 8(3): 274-285
DOI: 10.1055/s-0028-1091523
Original article

© 1977 by Thieme Medical Publishers, Inc.

The Unusual Features of Traumatic Neurogenic Muscular Atrophy in the Infant: An Anatomic Study1

H. H. Goebel, J. Muller
  • Division of Neuropathology, Indiana University Medical Center, Indianapolis, U.S.A.
1Supported by USPHS grants NS-04607 and NS-09797, and a grant from The Children's Brain Diseases Foundation, San Francisco.
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Publication History

1977

1977

Publication Date:
18 November 2008 (online)

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Abstract

A biopsy from the deltoid muscle of an 8-week old girl who had sustained a bilateral traumatic Erb's brachial plexus paresis at birth revealed the neurogenic atrophy to be different from that of adult muscle in that small, round fibers, similar to those observed in Werdnig-Hoffmann disease, were found. Detailed histochemistry of the fibers is submitted. On electron microscopy, there was focal degeneration of sarcomeres; a loss of myofibrils and myofilaments accompanied by increased autophagocytosis and increased amounts of glycogen; occasionally, preferential drop out of thick myofilaments and a peculiar displacement and deformation of the T-system, triads. In spite of the fact that the injury was sustained at one well defined point of time, the degree to which the muscle fibers were affected was quite variable from area to area.

It is proposed that denervation in infantile muscle results in typical small rounded fibers and that this process, so different from that of adult neurogenic atrophy, is age-dependent.

Zusammenfassung

Nach geburtsbedingter beidseitiger Plexus-Brachialis-Schädigung mit schlaffer Lähmung beider Arme zeigte eine Biopsie des M. deltoideus bei einem 8 Wochen alten Mädchen zahlreiche atrophische abgerundete Muskelfasern. Diese Querschnittsform der Muskelfasern nach Denervation ist — wie auch bei der Werdnig-Hoffmann-Krankheit — im Gegensatz zu den polygonalen angulären Fasern nach Denervation beim Erwachsenen charakteristisch für das frühe Kindesalter. Elektronenmikroskopisch waren die pathologischen Befunde an den einzelnen Muskelfasern trotz der akuten zeitlich bestimmbaren Läsion unterschiedlich stark ausgeprägt: Verlust von Myofilamenten, vornehmlich des Myosins, Autophagozytose, Reduplikation der Basalmembran und Veränderungen am sarkoplasmatischen Retikulum und T-System.

Keyword

Brachial plexus lesion - infantile denervation - neurogenic muscular atrophy - enzyme histochemistry - ultrastructure

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