CHRONIC MULTIPLE PERIPHERAL NEUROPATHY IN CHILDHOOD

Abstract

Attention is directed to the occurrence of chronic polyneuropathy in childhood. The difficulty of adequate sensory examination in children and the slow evolution of the disease tend to obscure the diagnosis and cause confusion with dystrophy or other diseases.

A review of the literature indicates that among chronic polyneuropathies of unknown etiology a number of different causes may lead to a common pathology, based on an increase in fibrous tissue and eventuating in hypertrophy. Histologic examination of nerves proves that the pathology of chronic interstitial polyneuritis may occur without palpable hypertrophy. Reference to the literature also suggests that the life span of affected individuals may be reasonably normal.

Early signs in childhood include disability in locomotion out of proportion to measurable weakness, later with frank sensory ataxia, deformities of the spine and feet and increase in the total protein in cerebrospinal fluid. The course is either progressive, or less frequently, remittent. Muscle and, less frequently, nerve biopsies are helpful diagnostically; the latter sometimes suggest the type of disease present.

In this series of patients no clear evidence of thiamine deficiency was detected by measurements of pyruvate in whole blood after administering a glucose load. No achlorhydria was found.

The possibility that continuing allergic factors are of importance was explored by treatment with cortisone. One child exhibited encouraging evidence of improvement on this regimen.