SEVERE SENSORY CHANGES, AND TROPHIC DISORDER, IN PERONEAL MUSCULAR ATROPHY (CHARCOT-MARIE-TOOTH TYPE)

Abstract

THE EARLY descriptions of peroneal muscular atrophy failed to draw any clear distinction between this disease and the amyotrophic spinal disease called progressive spinal muscular atrophy, of which it was at first assumed to represent a familial form. The publication in 1886 of the classic commentaries of Charcot and Marie¹ and of Tooth² drew attention to the onset of the hereditary disease in childhood, its commencement in the feet and legs, the sparing of proximal muscles, and the occasional occurrence of mild sensory changes as distinguishing features. These features led Tooth to propose that peroneal muscular atrophy is an affection of the peripheral nerves, as opposed to primary affection of the anterior horn cells in progressive neural muscular atrophy. Charcot and Marie favored the possibility of a myelopathy. Hoffmann,³ in presenting further cases, also emphasized the slight sensory changes and drew attention to the autopsy in a