期刊论文详细信息
Arquivos de Neuro-Psiquiatria | |
Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases | |
Carlos M. De Castro Costa1  Jean Marie Brucher2  Christian Laterre1  | |
[1],Catholic University of Louvain Laboratory of NeuropathologyBrussels ,Belgium | |
关键词: sporadic Creutzfeldt-Jakob disease; dementia; periodic EEG; neuropathology; prion protein (PrP) amyloid plaque; prion disease; doença de Creutzfeldt-Jakob do tipo esporádico; demência; EEG periódico; neuropatologia; placa amilóide de proteína prion (PrP); doença por prion; | |
DOI : 10.1590/S0004-282X1998000300003 | |
来源: SciELO | |
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【 摘 要 】
The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56%) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people.【 授权许可】
CC BY
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