Five cases of Creutzfeldt-Jakob disease: A clinical-pathological contribution

Clinico-pathological features are reported in five cases of Creutzfeldt-Jakob disease. In three cases only the brain was examined, while in the other two a complete autopsy was performed. In one case a brain biopsy was performed and in another two a piece of cerebral cortex was taken within three hours after death; the brain material was frozen and sent for inoculation in monkeys to Dr. Gajdusek of NINDS-Bethesda, with positive results in two. From the clinical point of view, our cases may be classified as follows: two as the 'classic' type; one as the 'amaurotic' type; and the last two as 'ataxic-amyotrophic' forms according to Alema and Brion. All five patients were females from 56 to 73 yr of age. The duration of the disease ranged from 3 to 7 months. From the histological point of view, in the two 'classic' cases the outstanding feature was the neuronal loss in all cortical mantle, mainly in the fronto-parietal cortex; furthermore, in one there was a severe and diffuse spongiform state. In the 'amaurotic' case, the three types of lesion were mainly localized in the occipital cortex. In the two 'ataxic-amyotrophic' forms, the neuronal loss was particularly evident in the cerebellar cortex and especially in the granular layer. The Kuru plaques were seen especially in the cerebellum in four of the five cases examined. The aim of our study is to contribute to the epidemiology of this disease.