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facioscapulohumeral muscular dystrophy |
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dystrophy /dys·tro·phy/ (dis´trof-e) any disorder due to defective or faulty nutrition.dystroph´ic 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.
Facioscapulohumeral muscular dystrophy (FSH) This form of muscular dystrophy, also known as Landouzy-Dejerine disease, begins in late childhood to early adulthood and affects both men and women, causing weakness in the muscles of the face, shoulders, and upper arms. Mentioned in: Muscular Dystrophy facioscapulohumeral muscular dystrophy Landouzy-Dejerine disease Neurology A benign AD type of muscular dystrophy characterized by marked atrophy of the muscles of the upper limb girdle and face, resulting in the so-called myopathic face. Cf
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Phenotypic and pathologic evaluation of the myd mouse: a candidate model for facioscapulohumeral dystrophy. |
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