Rehabilitation of patients with Hallervorden-Spatz syndrome.

Abstract

Hallervorden-Spatz Syndrome is an infrequently occurring disorder involving pigmentary degeneration of the globus pallidus, red nucleus, and substantia nigra. Resultant corticospinal and extrapyramidal signs and symptoms predominate early in the course of the disease, with concomitant intellectual deterioration paralleling the developing muscular disorder. To date, there have been no reports in the literature of rehabilitative intervention in patients with this disabling pathology. We report the case of a 43-year-old man whose illness spanned 20 years. He was referred for rehabilitation after a precipitous decline in function over the course of six months. An interdisciplinary team approach was implemented and various medical strategies were used, including the initiation of carbidopa-levodopa. An improvement in functional skills, communication, and the ability to use cognitive strategies was observed. We conclude that a multidisciplinary team approach and in selected cases, the use of carbidopa-levodopa are valid methods for the rehabilitation of patients with Hallervorden-Spatz Syndrome.