title: Quadriceps myopathy: forme fruste of Becker muscular dystrophy.
abstract: We examined dystrophin, the protein product of the Duchenne muscular dystrophy gene, in muscle biopsy specimens from 4 male patients with quadriceps myopathy, all of whom showed a mild and slowly progressive myopathy confined to the quadriceps muscles. All 4 patients had clear abnormalities of dystrophin, and were diagnosed as having Becker muscular dystrophy by both immunofluorescence and immunoblot examinations; that is, dystrophin of an abnormal molecular mass was visualized in muscle cryosections as "patchy" or discontinuous immunostaining at the surface membrane of the muscle fibers. One patient had a brother who showed widespread myopathic changes consistent with typical Becker muscular dystrophy. We conclude that the syndrome called quadriceps myopathy includes a group of forme fruste Becker muscular dystrophy.
authors: Department of Neurology, National Center Hospital, Tokyo, Japan.
authors: N
authors: N
authors: Sunohara
authors: K
authors: K
authors: Arahata
authors: E P
authors: EP
authors: Hoffman
authors: H
authors: H
authors: Yamada
authors: J
authors: J
authors: Nishimiya
authors: E
authors: E
authors: Arikawa
authors: M
authors: M
authors: Kaido
authors: I
authors: I
authors: Nonaka
authors: H
authors: H
authors: Sugita
mesh_terms: D000328:Adult; D000368:Aged; D016189:Dystrophin / Q000032:analysis*; D004576:Electromyography; D005455:Fluorescent Antibody Technique; D006801:Humans; D006984:Hypertrophy; D015151:Immunoblotting; D007866:Leg; D008297:Male; D009132:Muscles / Q000473:pathology / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D010375:Pedigree; D013848:Thigh
chemical_list: D016189:Dystrophin