Congenital muscular dystrophy: case reports and reappraisal

We report four cases of congenital muscular dystrophy; all demonstrated hypotonia and multiple contractures at birth. Strength remained stationary or improved, but the tendency for contracture formation persisted. Brief small amplitude polyphasic potentials were recorded on electromyography, and mus...

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Bibliographic Details
Published inMuscle & nerve Vol. 2; no. 5; p. 349
Main Authors Lazaro, R P, Fenichel, G M, Kilroy, A W
Format Journal Article
LanguageEnglish
Published United States 01.09.1979
Subjects
Child
Child, Preschool
Diagnosis, Differential
Female
Humans
Infant
Male
Muscles - pathology
Muscular Dystrophies - congenital
Muscular Dystrophies - diagnosis
Muscular Dystrophies - pathology
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Summary:We report four cases of congenital muscular dystrophy; all demonstrated hypotonia and multiple contractures at birth. Strength remained stationary or improved, but the tendency for contracture formation persisted. Brief small amplitude polyphasic potentials were recorded on electromyography, and muscle biopsy revealed extensive fat and/or collagen replacement, which was out of proportion to fiber necrosis or patient strength. The consistent clinical and pathologic features of these patients and others described in the literature justify considering this disorder to be a specific nosologic entity.
ISSN:0148-639X
DOI:10.1002/mus.880020505