About The Medical Condition Emery-Dreifuss Syndrome
Find the definition of the medical term Emery-Dreifuss Syndrome. Emery-Dreifuss Syndrome defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Emery-Dreifuss Syndrome
| Emery-Dreifuss Syndrome Medical Condition |
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What's The Definition Of The Medical Condition Emery-Dreifuss Syndrome?
An X-linked recessive muscular dystrophy that may present in children or adults. The disease is characterized by a clinical triad: (1) CONTRACTURE of the elbows, ACHILLES TENDON, and posterior cervical muscles; (2) slowly progressive muscle atrophy and weakness; and (3) a cardiomyopathy usually presenting as an atrioventricular block (HEART BLOCK). The weakness initially affects the upper arm and chest muscles, followed by pelvic and leg muscle paresis. Sudden death (DEATH, SUDDEN, CARDIAC) may occur as the result of the cardiac conduction defect. (Adams et al., Principles of Neurology, 6th ed, p1419; Curr Opin Neurol 1997;10(5):421-425)
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