Motor neurone disease.

Abstract

THE story of motor neurone disease goes back more than a hundred years, to the latter half of the igth century, and the days of the great clinical neurologists of France. Of the many famous names linked to this story three are pre-eminent: Charcot-physician and neuropathologist, and great teacher, who became even more renowned for his studies of hysteria (Fig. i); Duchenne, the one from Boulogne, who came from a family of fishermen and seafarers (Fig. 2); and Dejerine, great clinical neurologist, and pioneer in the study of localization of function in the brain (Fig. 3). Progressive degenerative lesions affecting both upper and lower motor neurones of the spinal cord and brain stem were described as separate diseases, distinct one from another, under the titles of progressive muscular atrophy, a spinal disease of lower motor neurones (Aran, I850; Duchenne, I858); progressive bulbar palsy, a disease of bulbar lower motor neurones (Wachsmuth, I864); and amyotrophic lateral sclerosis (Charcot and Jqffroy, I869), a disease of both lower and upper motor neurones. Progressive ophthalmoplegia (Hutchinson, I879; Gowers, I879; Brissaud, I895) was formerly considered to be a nuclear disease analogous to the cases of progressive bulbar palsy originally described by Duchenne (i858) as 'primary labio-glosso-laryngeal paralysis'. The nuclear origin of progressive bulbar palsy was demonstrated by Charcot, and the association with amyotrophic lateral sclerosis by Dejerine (I883). Chronic poliomyelitis, nuclear amyotrophy and progressive spinal muscular atrophy are other synonyms which have been used to describe the diseases now grouped by many authorities under the name of motor neurone disease. The Werdnig-Hoffmann paralysis of infantile progressive spinal muscular atrophy is usually added to this grouping, the original cases being described in I89I and I893. The unity of these several conditions has been criticized by Wilson (1954), who considers the term motor neurone disease 'etiologically vague and pathologically unspecific'. The clinical picture and progress of these diseases often remain distinct throughout life, and there is a tendency for this clarity of outline to become blurred by nosological fusion into a single disease entity. Despite the probable pathological unity there is much to be said for retaining within the overall heading of motor neurone disease, the original individual names for diseases which have many clinical differences and differing prognoses.