Which conditions have onset in adolescence or early adulthood with normal or slightly reduced lifespan and progressive weakness of the face, shoulders, and upper arms?

• A. EDMD
• B. Becker MD
• C. FSHD & LGMD
• D. Myotonic Dystrophy

Answer: (C)

The pattern here is weakness starting in adolescence or early adulthood that prominently affects the face and the shoulder girdle (face, shoulders, upper arms) with a near-normal lifespan. That combination points to diseases that specifically hit the facial muscles and scapulohumeral region. Facioscapulohumeral muscular dystrophy does exactly this: facial weakness paired with scapular winging and shoulder girdle weakness that begins in the teen years or early adulthood, and life expectancy is typically close to normal. Limb-girdle muscular dystrophy includes several subtypes that produce proximal weakness around the shoulders and hips and often begin in adolescence as well; many of these patients live normal lifespans. Together, these two conditions best fit the described onset pattern and distribution of weakness.

In contrast, Emery-Dreifuss MD centers on early contractures and elbow/neck involvement; Becker MD features proximal weakness with calf pseudohypertrophy rather than prominent early facial weakness; myotonic dystrophy involves myotonia and multisystem features rather than a primary facial-shoulder girdle pattern in adolescence.