Polyneuropathy in Childhood

Summary A clinical material is presented consisting of 43 children below 17 years of age with evidence of impaired function of peripheral nerves. One case occurred in a newborn with muscular hypotonia, weakness and areflexia: Thirteen children had signs of an affection also of the central nervous system. This group included 2 cases of late‐infantile metachromatic leucodystrophy and 2 cases of Friedreich's ataxia. Involvement of both central and peripheral parts of the nervous system causes a complicated clinical picture. Correct interpretation of the findings requires complete neurological and neurophysiological examination. Such an extensive affection of the nervous system may be more common than usually supposed. In 29 patients no signs were found of simultaneous involvement of the central nervous system. The 2 main groups were children with diabetes and children with a chronic hereditary polyneuropathy. All the diabetic children were asymptomatic. The most common clinical picture of chronic hereditary polyneuropathy in childhood was characterized by slow motor development, slow, clumsy movements and weak or absent muscle reflexes. Excavated feet and kyfoscoliosis were seen in some patients. The variation of the clinical picture with age and from family to family is described. The progress of symptoms in polyneuropathy without evidence of involvement of the central nervous system is slow and seems eventually to cease. The prognosis is better than that of progressive spinal muscular atrophy and most of the primary muscle diseases. An optimistic attitude, intense physical therapy and orthopedic measures are therefore justified.