[Peroneal atrophy in Tunisia. Study of 70 cases, pure or associated with other heredodegenerative diseases]

Seventy cases of hereditary peripheral neuropathy of Charcot-Marie-Tooth type have been studied. One group of 40 cases from 30 families had a pure peripheral neuropathy, the other 30 from 20 families having other associated inherited nervous defects. The classification of Dyck and Lambert (1968) modified by Dyck (1975) was used, but it proved difficult to distinguish pure types and transitional forms were common. Histological criteria appeared more reliable than clinical features and were the most constant finding within a given family. In forms associated with other abnormalities a hypertrophic and a neuronal form could be distinguished but similar difficulties in classification were encountered as the mode of genetic transmission, age of onset, clinical features and nerve conduction velocity were comparable in the two groups. Discrepancies between electrophysiological and histological findings may result from examining motor nerves with the former technique and sensory with the latter. Despite subdivision there is still a sharp distinction between the various forms of Charcot-Marie-Tooth disease and the hypertrophic neuropathy of Déjerine-Sottas. The genetic pattern is complicated by the frequent association with other inherited abnormalities.