The name Facio (face) – Scapulo (shoulder blades) – Humeral (upper arm) indicates the particular distribution of affected muscles that characterizes early FSHD. Over time and depending on how an individual is affected, the muscles of the leg can become affected as well. This pattern of decreasing growth of the face and shoulders to the legs is typical for most individuals.
The majority of individuals with FSHD usually start noticing symptoms in their teens, but the range of the age at which symptoms begin varies widely from early childhood, also called infantile FSHD, in late adulthood. In fact, a fraction of people who have very mild weakness to which they may have compensated never notice any symptoms.
The typical initial complaint relates to the weakness of shoulder blades and improvise with difficulty raising the arms above. Sometimes the initial symptom is tripped or slapping of the foot due to foot drop.