Motor neuron disease (amyotrophic lateral sclerosis)

Abstract

Amyotrophic lateral sclerosis is an insidiously developing, adult-onset, progressive anterior horn cell degeneration with associated degeneration of descending motor pathways. It has been recognized as an important clinical syndrome since the middle of the 19th century. Despite increasing clinical and research interest in this condition, its cause remains obscure, even in the broadest terms. Epidemiologic characteristics of the disease have been interpreted as evidence of both genetic and environmental causes. A major change in the view of this disease is the widely developing perception that it is a disease of elderly persons more than of middle-aged adults as was previously taught. Etiologic hypotheses encompass a broad range of postulated pathophysiologic mechanisms, and we review these in detail. The clinical limits of the disease can now be better defined by using modern diagnostic techniques. Although interest in supportive symptomatic therapy is growing, no intervention has yet been shown to modify the biologically determined motor system degeneration.