Studies on Lesions of the Basal Ganglia in Defectives: (1) a Case oféTat Dysmyélinisé(Hallervorden-Spatz Disease)

Abstract

In the following paper a case ofétat dysmyéliniséof the globus pallidus, or as it is often synonymously called,“Hallervorden-Spatz disease”, will be presented. Records of thoroughly studied cases of this condition are still rather scanty and there is much need for a systematic extension of experience, the more so since, as Helfand (16, 17) has recently pointed out, progress in the elucidation of the condition has not always been enhanced by generalizations from findings based on too small a case-material. We are happy in having at our disposal the vast resources of the London County Mental Hospitals. It is hoped to follow up this paper by publishing further cases of a rather different nature, though with some characteristics in common with the case described here.