A neurogenic component in muscular dystrophy

Abstract

Evidence for a neurogenic component in mouse and human muscular dystrophy is briefly reviewed. Such evidence comes from certain clinical observations, electrophysiological studies, muscle pathology, nervous system pathology, transplantation experiments in animals, and tissue culture studies. The evidence is at present rather conflicting though the results of recent tissue culture experiments are more convincing. If there is a neurogenic component in dystrophy then the basic defect may have to be sought in the central nervous system rather than in the muscle itself. It is argued, however, that a neurogenic component in dystrophy cannot be simply a defect in the anterior horn cells of the spinal cord since the clinical features and the laboratory and pathological findings are quite different from those in spinal muscular atrophy.