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  • Title: Quadriceps myopathy: forme fruste of Becker muscular dystrophy.
    Author: Sunohara N, Arahata K, Hoffman EP, Yamada H, Nishimiya J, Arikawa E, Kaido M, Nonaka I, Sugita H.
    Journal: Ann Neurol; 1990 Nov; 28(5):634-9. PubMed ID: 2260849.
    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.
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