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dystrofia svalová facioskapulohumerálna

  1. Subject h.dystrofia svalová facioskapulohumerálna
    Subject h.Muscular Dystrophy, Facioscapulohumeral
    Entry termsLandouzyho-Déjerineova dystrofia
    English X referencesFacioscapulohumeral Muscular Dystrophy
    Landouzy-Dejerine Dystrophy
    Scope note in EnglishAn autosomal dominant degenerative muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder girdle. The onset of symptoms usually occurs in the first or second decade of life. Affected individuals usually present with impairment of upper extremity elevation. This tends to be followed by facial weakness, primarily involving the orbicularis oris and orbicularis oculi muscles. (Neuromuscul Disord 1997;7(1):55-62; Adams et al., Principles of Neurology, 6th ed, p1420)
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    (1) - CiBaMed
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