facioscapulohumeral muscular dystrophy


dystrophy

 [dis´trah-fe] any disorder due to defective or faulty nutrition, especially muscular dystrophy. adj., adj dystroph´ic.adiposogenital dystrophy adiposity of the feminine type, genital hypoplasia, changes in secondary sex characters, and metabolic disturbances; seen with lesions of the hypothalamus; see also adiposogenital dystrophy.Becker's muscular dystrophy (Becker type muscular dystrophy) a form closely resembling Duchenne's muscular dystrophy, but having a later onset and milder course; transmitted as an X-linked recessive trait.distal muscular dystrophy distal myopathy.Duchenne's muscular dystrophy (Duchenne type muscular dystrophy) The childhood type of muscular dystrophy.facioscapulohumeral muscular dystrophy muscular dystrophy affecting the face, shoulder, and upper arm muscles; called also Landouzy-Dejerine muscular dystrophy.Landouzy-Dejerine dystrophy (Landouzy-Dejerine muscular dystrophy) facioscapulohumeral muscular dystrophy.muscular dystrophy see muscular dystrophy.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. Called also dystrophia myotonica, myotonia atrophica, and myotonia dystrophica.progressive muscular dystrophy muscular dystrophy.pseudohypertrophic muscular dystrophy muscular dystrophy affecting the shoulder and pelvic girdles, beginning in childhood and marked by increasing weakness, pseudohypertrophy of the muscles, followed by atrophy, and a peculiar swaying gait with the legs kept wide apart. Called also pseudohypertrophic muscular paralysis.reflex sympathetic dystrophy a syndrome of chronic pain that usually develops after a trauma or noxious stimulus, although the nerve injury cannot be immediately identified. The pain is not limited to the distribution of a single nerve and is often out of proportion to the precipitating event. It is most often described as a burning pain, and is accompanied by swelling, sweating, sensitivity to touch, and sometimes changes in tissue growth. Called also chronic or complex regional pain syndrome. Clinical practice guidelines have been published by the Reflex Sympathetic Dystrophy Syndrome Association of America and are available on their web site at http://www.rsds.org or by writing to Reflex Sympathetic Dystrophy Syndrome Association of America, P.O. Box 502, Milford CT 06460.

fa·ci·o·scap·u·lo·hu·mer·al mus·cu·lar dys·tro·phy

[MIM*158900] a highly variable hereditary disorder with onset in childhood or adolescence, characterized by weakness and wasting, sometimes asymmetric, mainly of the muscles of the face, shoulder girdle, arms, and later, pelvic girdle and legs; autosomal dominant inheritance. Synonym(s): facioscapulohumeral atrophy, Landouzy-Dejerine 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. See Muscular dystrophy, Myopathic face.

fa·ci·o·scap·u·lo·hu·mer·al mus·cu·lar dys·tro·phy

(fāshē-ō-skapyū-lō-hyūmĕr-ăl mŭs'kyū-lăr dis'trŏ-fē) Highly variable hereditary disorder with onset in childhood or adolescence, characterized by weakness and wasting, sometimes asymmetric, mainly of the muscles of the face, shoulder girdle, arms, and later, pelvic girdle and legs.

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

Landouzy,

Louis T.J., French neurologist, 1845-1917. Dejerine-Landouzy dystrophy - Synonym(s): Landouzy-Dejerine dystrophyDejerine-Landouzy myopathy - Synonym(s): Landouzy-Dejerine dystrophyLandouzy-Dejerine dystrophy - a relatively benign type of muscular dystrophy commencing in childhood and slowly progressive. Synonym(s): Dejerine-Landouzy dystrophy; Dejerine-Landouzy myopathy; facioscapulohumeral muscular dystrophyLandouzy-Grasset law - in lesions of one hemisphere, the patient's head is turned to the side of the affected muscles if there is spasticity and to that of the cerebral lesion if there is paralysis. Synonym(s): Grasset law

fa·ci·o·scap·u·lo·hu·mer·al mus·cu·lar dys·tro·phy

(fāshē-ō-skapyū-lō-hyūmĕr-ăl mŭs'kyū-lăr dis'trŏ-fē) [MIM*158900] Highly variable hereditary disorder with onset in childhood or adolescence, characterized by muscular weakness and wasting.