Emery-Dreifuss muscular dystrophy (redirected from Benign scapuloperoneal muscular dystrophy with early contractures)

dystrophy /dys·tro·phy/ (dis´trof-e) any disorder due to defective or faulty nutrition.dystroph´ic

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adiposogenital dystrophy  a condition marked by adiposity of the feminine type, genital hypoplasia, changes in secondary sex characters, and metabolic disturbances; seen with lesions of the hypothalamus.

Becker's muscular dystrophy , Becker type muscular dystrophy a form closely resembling pseudohypertrophic muscular dystrophy but having a late onset and slowly progressive course; transmitted as an X-linked recessive trait.

Duchenne's dystrophy , Duchenne's muscular dystrophy, Duchenne type muscular dystrophy the most common and severe type of pseudohypertrophic muscular dystrophy; it begins in early childhood, is chronic and progressive, and is characterized by increasing weakness in the pelvic and shoulder girdles, pseudohypertrophy of muscles followed by atrophy, lordosis, and a peculiar swinging gait with the legs kept wide apart.

Emery-Dreifuss muscular dystrophy  a rare X-linked form of muscular dystrophy beginning early in life and involving slowly progressive weakness of the upper arm and pelvic girdle muscles, with cardiomyopathy and flexion contractures of the elbows; muscles are not hypertrophied.

facioscapulohumeral muscular dystrophy  a relatively benign form of muscular dystrophy, with marked atrophy of the muscles of the face, shoulder girdle, and arm.

Fukuyama type congenital muscular dystrophy  a form of muscular dystrophy with muscle abnormalities resembling those of Duchenne's muscular dystrophy; characterized also by mental retardation with polymicrogyria and other cerebral abnormalities.

Landouzy dystrophy , Landouzy-Dejerine dystrophy, Landouzy-Dejerine muscular dystrophy facioscapulohumeral muscular d.

Leyden-Möbius muscular dystrophy , limb-girdle muscular dystrophy slowly progressive muscular dystrophy, usually beginning in childhood, marked by weakness and wasting in the pelvic girdle (pelvifemoral muscular dystrophy) or shoulder girdle (scapulohumeral muscular dystrophy) .

muscular dystrophy  a group of genetically determined, painless, degenerative myopathies marked by muscular weakness and atrophy without nervous system involvement. The three main types are pseudohypertrophic muscular d., facioscapulohumeral muscular d., and limb-girdle muscular d.

myotonic dystrophy  a rare, slowly progressive, hereditary disease, marked by myotonia followed by muscular atrophy (especially of the face and neck), cataracts, hypogonadism, frontal balding, and cardiac disorders.

oculopharyngeal dystrophy , oculopharyngeal muscular dystrophy a form with onset in adulthood, characterized by weakness of the external ocular and pharyngeal muscles that causes ptosis, ophthalmoplegia, and dysphagia.

pseudohypertrophic muscular dystrophy  a group of muscular dystrophies characterized by enlargement (pseudohypertrophy) of muscles, most commonly Duchenne's muscular d. or Becker's muscular d.

reflex sympathetic dystrophy  a series of changes caused by the sympathetic nervous system, marked by pallor or rubor, pain, sweating, edema, or osteoporosis, following muscle, bone, nerve, or blood vessel trauma.

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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.