PICKʼS DISEASE

Abstract

Since the publication of Pick''s first article describing the clinical and pathologic syndrome which now bears his name, more than 200 cases of focal cerebral atrophy with progressive mental deterioration have been reported. From a study of 7 cases, the author concludes that there are 2 types of pathologic change in this syndrome. In one group of cases there is widespread loss of nerve cells with profound disturbance of architecture in the regions of focal atrophy. The few remaining nerve cells are chiefly of the swollen type with an eccentric nucleus at one pole. There is extensive loss of myelin in the subcortex with proliferation of astrocytes. Patients with this type of disturbance show progressive mental and physical deterioration but their motor function generally remains unimpaired or overactive whether the temporal or frontal lobes are affected, until the terminal stages when they become completely vegetative. The other group shows a picture suggestive of progressive presenile dementia but, in addition, exhibits neurologic changes indicative of disturbances in more diffuse fields. In these patients the cortical architecture was only slightly disturbed and the projection fibers displayed a normal pattern. The loss of nerve cells was much less extensive and the nerve cell alterations were nonspecific in character. Ballooned nerve cells with eccentric nuclei were rarely observed and argyrophilic inclusions were not demonstrated. In spite of the divergence in the microscopic picture, the gross appearance of the brain[long dash]a lobar or focal atrophy[long dash]does not present striking differences in the 2 types.