Corticodentatonigral Degeneration With Neuronal Achromasia

Abstract

IN RECENT years staff members of the Neurology Service at the Massachusetts General Hospital have had the opportunity of examining three adult patients suffering from a slowly progressive neurological illness that defied all attempts at exact diagnosis and effective treatment. This strange disorder was characterized by severe impairment in the control of muscular movements, by abnormalities in posture and by involuntary motor activity. Despite serious disability, there was little muscular weakness, and mental faculties were relatively spared until the end. After years of increasing incapacity, all three patients died of the effects of the illness. Postmortem neuropathological studies showed a disease process in the central nervous system that was unlike any that we had previously seen or had been described by others. Since it appears to us that we may be dealing with a hitherto unrecognized disorder of the central nervous system, we feel justified in reporting our findings