Emery-Dreifuss muscular dystrophy


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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]
Farlex Partner Medical Dictionary © Farlex 2012

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.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

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.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
References in periodicals archive ?
Emery-Dreifuss muscular dystrophy 1, X-linked; EDMD1.
Properties of lamin A mutants found in Emery-Dreifuss muscular dystrophy, cardiomyopathy.
Other, less severe forms of muscular dystrophy include Becker muscular dystrophy (BMD), Emery-Dreifuss muscular dystrophy (EDMD), facioscapulohumeral muscular dystrophy (FMD), limb-girdle muscular dystrophy (LGMD) and myotonic dystrophy (MMD).
A cardiac pacemaker is frequently needed in Emery-Dreifuss muscular dystrophy. Medications can also be used to treat some cardiac problems.