Classification of Neuromuscular Diseases

Abstract

A large number of different neuromuscular diseases are recognised. Classification is important for purposes of nomenclature and standardised diagnosis, but classification itself depends on knowledge of the underlying pathogenesis of the different diseases and, as new advances in knowledge develop, the classification of diseases alters. For example, the impact of molecular biology and genetics on neuromuscular diseases has had a particularly profound impact on classification of the genetically determined neuropathies. As the relationship between acquired and genetic syndromes in the progressive proximal neurogenic disorders becomes better understood there will probably also be profound changes in classification of these disorders. Even as this book has been in preparation, the limb-girdle dystrophy syndromes have begun to be unravelled. The World Federation of Neurology Committee on Neuromuscular Diseases modified its classification of Neuromuscular Diseases in 1994 in response to the changes necessary since its previous classifications of 1968 and 1988, and this internationally accepted classification of the neuromuscular diseases forms the basis of the organisation of chapters in this book.