[Hereditary neuropathies in childhood. Study of 31 patients].
Authors present the results of prospective clinical and electrophysiological study on 31 children diagnosed of inherited sensory-motor neuropathy. Patients were grouped according to their electrophysiological findings and their mode of inheritance, which was ascertained in all the cases by clinical and electrophysiological study of their parents. The large number of patients, felt into a group with identical characteristics of type I, HSMN of Dick and Lambert. A second group of patients, with a wide range of clinical manifestations, in some cases suggestive of type III HSMN, showed a sporadic mode of inheritance and evidence of demyelination on electrical studies. The third group of patients, which were classified as type II HSMN, showed again, a wide range of clinical manifestations, with some patients severely involved, and thus, casting some doubt on the benign prognosis of this type of neuropathy claimed by some authors.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004576:Electromyography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009422:Nervous System Diseases / Q000145:classification / Q000235:genetics* / Q000503:physiopathology; D009431:Neural Conduction; D011446:Prospective Studies
Neuropatías hereditarias en la infancia. Estudio de 31 pacientes.