Age of onset is variable. The disease tends to progress from the face downwards. Asymmetry and selective muscle group involvement distinguishes FSHD from other muscular dystrophies. Many authors describe stepwise deterioration with prolonged periods of apparent arrest. Extraocular muscles, bulbar muscles, deltoid, and respiratory muscles are usually spared.
Life expectancy is normal in most patients.
The muscular dystrophies. Evidence for anticipation and association of deletion size with severity in facioscapulohumeral muscular dystrophy. The FSH-DY Group . FSH Consortium . Medicine is a constantly changing science and not all therapies are clearly established.