En autosomalt dominant, degenerativ muskelsjukdom som kännetecknas av långsamt tilltagande svaghet i ansiktets, överarmens och skuldergördelns muskler. Symtomen debuterar vanligtvis under livets första eller andra årtionde. De drabbade uppvisar oftast försämrad förmåga att höja armarna. Dessa tecken brukar följas av ansiktsmuskelsvaghet, främst i munnens och ögats ringmuskler.
An 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)
Facioskapulohumeral muskeldystrofi — Landouzy-Dejerines syndrom
Dystrophies, Facioscapulohumeral Muscular — Dystrophy, Facioscapulohumeral Muscular — Facioscapulohumeral Muscular Dystrophies — Muscular Dystrophies, Facioscapulohumeral — Facioscapulohumeral Atrophy — Atrophies, Facioscapulohumeral — Atrophy, Facioscapulohumeral — Facioscapulohumeral Atrophies — Facioscapulohumeral Muscular Dystrophy — Facioscapulohumeral Type Progressive Muscular Dystrophy — FSH Muscular Dystrophy — Landouzy-Dejerine Dystrophy — Dystrophies, Landouzy-Dejerine — Dystrophy, Landouzy-Dejerine — Landouzy Dejerine Dystrophy — Landouzy-Dejerine Dystrophies — Muscular Dystrophy, Landouzy Dejerine — Progressive Muscular Dystrophy, Facioscapulohumeral Type — Facio-Scapulo-Humeral Dystrophy — Facioscapuloperoneal Muscular Dystrophy