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July 1986

Autosomal Dominant Humeroperoneal Myopathy

Author Affiliations

From the Department of Neurology, Medical College of Virginia, Richmond. Dr Gilchrist is now with Duke University Medical Center, Durham, NC.

Arch Neurol. 1986;43(7):734-735. doi:10.1001/archneur.1986.00520070088026

• Emery-Dreifuss muscular dystrophy is a syndrome with five salient features: (1) early and unusual contractures; (2) humeroperoneal muscle wasting; (3) the slow progression of weakness, beginning in childhood; (4) cardiac conduction defects; and (5) X-linked inheritance. We present two cases and detail other reports with a similar constellation of findings with apparent autosomal dominant inheritance. We postulate separate genetic disorders with similar phenotypic expression.

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