Emery-Dreifuss muscular dystrophy

(redirected from Benign scapuloperoneal muscular dystrophy with early contractures)
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Em·er·y-Drei·fuss mus·cu·lar dys·tro·phy

(em'ĕr-ē drī'fŭs), [MIM*310300]
a generally benign type of muscular dystrophy, with onset in childhood or early adulthood. Weakness begins with the pectoral girdle and proximal upper extremity muscles and spreads to the pelvic girdle and distal lower extremity muscles. Contractures of the elbow, flexors, neck flexors, and calf muscles often occur; muscle pseudohypertrophy and mental retardation do not occur. A cardiomyopathy is common. An X-linked inherited disorder, nonallelic to Duchenne muscular dystrophy.
[Alan E. H. Emery, Fritz Dreifuss]

Emery-Dreifuss muscular dystrophy

Scapulohumeral dystrophy Molecular medicine A form of muscular dystrophy characterized by contractions of elbow, Achilles tendon, and postcervical muscles in childhood, with slowly progressive wasting and weakness of humeroperoneal muscles; by adulthood, Pts with EDMD have conduction system disease, usually, heart block. See Dilated cardiomyopathy, Emerin, Lamin A/C.

Em·er·y-Drei·fuss mus·cu·lar dys·tro·phy

(em'ĕr-ē drī'fŭs mŭskyū-lăr distrŏ-fē)
Usually benign type of muscular dystrophy, with onset in childhood or early adulthood. Weakness begins with the pectoral girdle and proximal upper extremity muscles and spreads to the pelvic girdle and distal lower extremity muscles. Contractures of the elbow, flexors, neck flexors, and calf muscles often occur; muscle pseudohypertrophy and mental retardation do not occur. A cardiomyopathy is common.

Emery-Dreifuss muscular dystrophy

(em′ĕ-rē-drī′fŭs)
[Alan E. H. Emery, British geneticist, b. 1928; Fritz Emanuel Dreifuss, German-born Brit. neurologist, 1926–1997]
,

EDMD

One of several rare forms of muscular dystrophy, characterized by muscular degeneration principally in the shoulders, arms, and calves. Cardiac conduction abnormalities resulting in heart block and joint contractures are common complications.
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