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Chronic distal spinal amyotrophy or spinal forms of charcot marie disease a report on 6 sporadic adult cases


, : Chronic distal spinal amyotrophy or spinal forms of charcot marie disease a report on 6 sporadic adult cases. Revue Neurologique (Paris) 135(11): 815-826

Based on a personal series of 6 cases, and about 20 cases reported in the published literature, the principal characteristics of chronic distal amyotrophy of spinal origin are described: early onset or in young adults, initial disorder in lower limbs, especially anterolateral region of the legs, with secondary lesions in small muscles of the hands, absence of sensory disturbance, usually very slow progression, electrophysiological appearance of chronic denervation with normal conduction velocities and neurogenic muscle lesions with unaffected peripheral nerve. These forms can be compared to the spinal forms of Charcot-Marie-Tooth disease and appear to be degenerative in origin even though proof by autopsy is lacking. In spite of their common characteristics their genetic heterogenicity has to be noted; sporadic cases are seen most frequantly but some cases are related to recessive autosomal transmission and others to dominant autosomal transmission. From the clinical point of view, some cases are distinctive in that there is early or predominent atrophy in the lower limbs, while others have lesions in muscles of bulbar innervation, which is against their origin from spinal amyotrophy. Electrical signs at a distance from the atrophied muscles suggest extension of the pathological process. The clinical concept of the distal form of spinal amyotrophy should be retained together with the proximal and scapuloperoneal forms.

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