The Limb Girdle Types of Muscular Dystrophy

The muscular dystrophies are inherited progressive disorders which cause breakdown of muscle fibres. In the early days of muscular dystrophy research four main types of muscular dystrophy were known - Duchenne, facioscapulohumeral, myotonic and a final group which was given the name of limb-girdle muscular dystrophy (LGD).

The main features of the limb-girdle type were that the muscles of the upper or "proximal" parts of the limbs were mainly involved, the muscles of the face were spared and the symptoms of the weakness were usually first noted in late childhood, adolescence or adult life (thus differing from the Duchenne type). Both males and females might be affected and their brothers or sisters were much more often affected than their parents or children.

It is now known that a number of conditions with different causes and different genetic implications may present with symptoms similar to this. It is evident, therefore, that the term "limb-girdle muscular dystrophy" may be applied loosely to many quite different disorders. This is important because these disorders differ in their rate of progression, outcome and especially in their mode of inheritance so that misleading genetic advice may be given if a more precise diagnosis is not made. Some doctors now consider that the name "limb-girdle muscular dystrophy" should no longer be used at all, and it seems clear that people who have been given this diagnosis should receive up to date advice about the particular type that is affecting them or members of their family.