[Virus-induced subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): a report of 10 cases with clinico-pathologic study].
This article is a report of clinico-pathological study of 10 cases, including 6 cases from Western Australia and 4 cases from Beijing and Qingdao of China. In all the 10 patients there were typical clinical features. Prodromal duration varied from 1.5 to 3 months and the dementia duration from 1 to 9.5 months. The clinical manifestations consisted of rapidly progressive dementia, myoclonic jerks. Pyramidal, extrapyramidal tract signs and cerebellar signs and periodic EEG findings. Neuropathological changes of this disease were spongiform changes, neuronal degeneration and loss of the ganglia cells and hypertrophy and hyperplasia of astrocytes. 2 of the 10 patients had brain biopsy and the other 8 had autopsy. Typical membrane bound vacuoles were shown under electron microscope. The frontal temporal and occipital lobes were more seriously affected than the parietal lobe and particularly the pre and postcentral gyri. The small and medial size ganglion cells were more seriously involved than large neuronal cells. Pyramidal cells were only mildly. In cases with a short duration, (less than 5 mouths), the spongiform changes were marked, but in those with a longer duration more than 6 months, they were mild. Neuronal loss and hypertrophy and hyperplasia of the astrocytes were mild in cases with short duration but were marked in those with longer duration. The clinical diagnosis, EEG findings, neuropathological features etiology and prognosis of this disease are discussed.
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D000328:Adult; D000368:Aged; D001253:Astrocytes / Q000473:pathology; D001921:Brain / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged