[Hypertrophic neuropathy with myotonia (author's transl)]

A case of distal amyotrophia is reported, in which there was associated isolated clinical and electrical myotonia. Nerve conduction rates were very reduced. The nerves showed rarefaction of nerve fibers with segmentary demyelinization and the formation of bulbar swellings. The signification of this observation is discussed, and it would not appear to be related to peripheral nerve lesions of the type sometimes seen in Steinert's disease or from an association of this affection with Charcot-Marie's disease. It would seem more reasonable to suggest the hypothesis of a particular type of association of a peroneal hypertrophic neuropathy and a myotonia similar to certain cases reported in the published literature.