Facioscapulohumeral muscular dystrophy (FSHD) is a neuromuscular disease, 
characterized by an autosomal dominant mode of inheritance, facial involvement, 
and selectivity and asymmetry of muscle involvement. In general, FSHD typically 
presents before age 20 years. Usually, FSHD muscle involvement starts in the 
face and then progresses to the shoulder girdle, the humeral muscles and the 
abdominal muscles, and then the anterolateral compartment of the leg. Disease 
severity is highly variable and progression is very slow. About 20% of FSHD 
patients become wheelchair-bound. Lifespan is not shortened. The diagnosis of 
FSHD is based on a genetic test by which a deletion of 3.3kb DNA repeats (named 
D4Z4 and mapping to the subtelomeric region of chromosome 4q35) is identified. 
The progressive pattern of FSHD requires that the severity of symptoms as well 
as their physical, social and psychological impact be evaluated on a regular 
basis. A yearly assessment is recommended. Multidisciplinary management of 
FSHD--consisting of a combination of genetic counselling, functional assessment, 
an assessment by a physical therapist, prescription of symptomatic therapies and 
prevention of known complications of this disease--is required. Prescription of 
physical therapy sessions and orthopedic appliances are to be adapted to the 
patient's deficiencies and contractures.