A retrospective study of Creutzfeldt-Jakob disease in Belgium

被引:15
作者
Pals, P [1 ]
Everbroeck, BV [1 ]
Sciot, R [1 ]
Godfraind, C [1 ]
Robberecht, W [1 ]
Dom, R [1 ]
Laterre, C [1 ]
Martin, JJ [1 ]
Cras, P [1 ]
机构
[1] Univ Antwerp, Neurobiol Lab, Born Bunge Fdn, B-2610 Wilrijk, Belgium
关键词
Creutzfeldt-Jakob disease; dementia; EEG; epidemiology; myoclonus; prion;
D O I
10.1023/A:1007581313067
中图分类号
R1 [预防医学、卫生学];
学科分类号
1004 ; 120402 ;
摘要
Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob disease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.
引用
收藏
页码:517 / 519
页数:3
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