Chronic peripheral neuropathy in childhood: an overview.

One hundred and twenty-five cases of biopsy-proven subacute or chronic peripheral polyneuropathy occurring in children under 17 years of age are reviewed. Ninety-three (74%) of the cases have been examined by the authors. At least 71% of cases were considered to be of genetic origin. Hereditary motor and sensory neuropathies were confirmed in over 40% of the cases. From among these a previously poorly defined entity (hereditary motor and sensory neuropathy of neuronal type with onset in early childhood) was delineated. It constituted 7.2% of the cases. When clinical and neurophysiological investigations of the family are combined with histopathological investigation of the affected child, a correct aetiological diagnosis can be reached in over 80% of cases of chronic polyneuropathy in childhood.