The Limb-Girdle Muscular Dystrophies

Matthew P. Wicklund, MDa,*, John T. Kissel, MDb


The Limb-Girdle Muscular Dystrophies

The most common presentation of muscle disease involves weakness in the hip girdle, thighs, shoulder girdle, and proximal arms. Many acquired and genetic muscle disor- ders present with this limb-girdle pattern (see the article by Barohn elsewhere in this issue). Of the 3 most common muscular dystrophies, the dystrophinopathies (see the article by Flanigan elsewhere in this issue) also have this proximal predominant limb- girdle pattern, whereas facioscapulohumeral muscular dystrophy (FSHD) (see the article by Statland and Tawil elsewhere in this issue) and myotonic dystrophy (see the article by Thornton elsewhere in this issue) have relatively unique phenotypes.