Dystonia--L-dopa responsive or juvenile parkinsonism?

Abstract

Four cases of dystonia occurring in two families are reported. The first symptoms consisting of dystonia and rigidity appeared early in childhood, in the first months in one family and of ages two and five years respectively in the other. In two cases, transient tremor was noted. These four children have been treated with L-dopa with prompt spectacular results, in cases 1 and 2, with more gradual less complete results in the others. L-dopa treatment was continued twelve, eleven, six, and five years, respectively, without any developmental problems. Motor function remains satisfactory and school work is normal. The only secondary effect observed was the occurrence of dyskinesia. The relation between L-dopa responsive dystonia and Parkinson's disease is discussed.