The autopsy findings in 30 patients aged 26 to 70 were examined, including 18 women aged 26 to 70 and 12 men aged 48 to 70; the average age being 54. The disease lasted 4 wk to 6 yr. In its course the majority (26 cases) showed severe ataxia. All the cases belonged to the sporadic form of Jakob Creutzfeldt disease. The morphologic picture of the changes in the cerebellum is dominated by a more or less moderately severe to subtotal disappearance of the Purkinje cells, various forms of degeneration of Purkinje cells, focal or diffuse increase of Bergmann's astroglia, status spongiosus mainly in the region of the Purkinje cell layer and Bergmann's astroglia, considerable lipopigment deposition in the dentate nuclei exceeding the age norm, and the presence of vascular coils indicating atrophy of the parenchyma. The assumption is justified that in Jakob Creutzfeldt disease the pathologic process in the cerebellum takes place mainly in the Purkinje cell layer and Bergmann's glia layer, presumably at the same time, and that it is based on a disturbance of the glioneural unit in the sense of Seitelberger's glioneural dystrophy. The topography of the atrophic changes in the cerebellum and the extreme involvement of the Purkinje cell layer, suggests that additional, circulation induced injuries may be superimposed on the basic process. It is possible that the main disappearance of Purkinje cells in the peripheral lobules comes about by a pressure variation between the supratentorial and infratentorial space.
|Translated title of the contribution||The morphological changes of the cerebellum in Jakob Creutzfeldt disease|
|Number of pages||6|
|Publication status||Published - Jan 1 1974|
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Clinical Neurology