Wernicke encephalopathy and Creutzfeldt-Jakob disease
Wernicke encephalopathy and Creutzfeldt-Jakob disease
复制标题
DOI：
10.1007/s00415-009-5038-1
复制
发表时间：
2009-06-01
影响因子：
6
通讯作者：
Hauw, J. J.
中科院分区：
文献类型：
--
作者：
Bertrand, A.;Brandel, J. P.;Hauw, J. J.
我们评估了2001年至2006年法国克雅氏病神经病理学网络所有657例疑似克雅氏病（CJD）病例中韦尼克脑病（WE）的患病率。在尸检诊断WE时，回顾临床、生物学和影像学资料。5例疑似散发性克雅氏病患者均未发现克雅氏病。在这5例病例中，4例观察到肌阵挛，2例观察到CSF 14-3-3蛋白。在另外14例病例中，WE合并CJD，其中13例为散发。这些主要属于散发性CJD的分子变异，与疾病持续时间长有关。这强调了在怀疑克雅二氏症时对WE诊断保持警惕的必要性。
We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.