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Emery-Dreifuss Humeroperoneal Muscular Dystrophy: Cardiac Manifestations
Canadian Journal of Cardiology, 2012Emery-Dreifuss muscular dystrophy (EDMD) is an inherited disorder affecting skeletal and cardiac muscles and characterized by muscular atrophy, contractures, and cardiomyopathy with conduction defects. It can be X-linked or autosomal. Not all patients with EDMD develop heart involvement, but heart disease associated with EDMD can be unpredictable and ...
Parmar, Malvinder S. +1 more
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Cardiac transplantation in female Emery-Dreifuss muscular dystrophy
Journal of Neurology, 1990A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities.
M P, Merchut, D, Zdonczyk, M, Gujrati
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Emery-Dreifuss muscular dystrophy and other related disorders
British Medical Bulletin, 1989There are some 30 or so different forms of muscular dystrophy which are conveniently classified according to the mode of inheritance. Emery-Dreifuss X-linked muscular dystrophy is characterized by the triad of: (1) early contractures of the elbows, Achilles tendons and postcervical muscles; (2) slowly progressive muscle wasting and weakness with a ...
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Emery-Dreifuss muscular dystrophy.
Genetic counseling (Geneva, Switzerland), 2004The muscular dystrophies are a group of disorders, genetically determined, with progressive degeneration of muscle(s), without central nervous nor peripheral nerve abnormalities. The Emery-Dreifuss muscular dystrophy is one of these. We report a case with typical features.
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Emery-Dreifuss muscular dystrophy
Journal of the Neurological Sciences, 1987Hideo Hara +4 more
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