ABSTRACT

In 1885, Landouzy and Déjérine coined the adjective facioscapulohumeral to describe the hallmarks of the more advanced stage of the muscular dystrophy (FSHD) they had observed in several families and to contrast this disease with the only known muscle disease at that time named after Duchenne (Landouzy and Déjérine, 1885). Patients with FSHD had been described and even photographed before 1885 (Padberg, 1982). However, it is likely that the adjective FSH later precluded recognition of the disease in some families with lower limb involvement (Oransky, 1927; Kazakov et al., 1975), while mild facial weakness in other families prompted the use of the term scapuloperoneal muscular dystrophy (Davidenkow, 1939). In any case, a discussion was necessary to get early foot-extensor weakness accepted as part of the natural course of FSHD (Tyler and Stephens, 1950; Padberg, 1982).