A new variant of Creutzfeldt-Jakob disease in the UK
A new variant of Creutzfeldt-Jakob disease in the UK
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DOI：
10.1016/s0140-6736(96)91412-9
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发表时间：
1996-04-06
期刊：
影响因子：
168.9
通讯作者：
Smith, PG
中科院分区：
文献类型：
--
作者：
Will, RG;Ironside, JW;Smith, PG
背景1990年在英国重新建立了克雅氏病（CJD）的流行病学监测，以确定牛海绵状脑病（BSE）在牛中流行后这种疾病发生的任何变化。还获得了提到克雅二氏病的死亡证明。获得所有转诊病例的临床详情，并通过向患者亲属发放标准问卷获得有关CJD潜在风险因素的信息。对约70%的可疑病例进行了神经病理学检查。流行病学研究CJD使用类似的方法，英国的研究已经在法国，德国，意大利和荷兰之间进行了1993年和1995年。结果10例CJD已确定在英国在最近几个月的一个新的神经病理学的配置文件。其他不寻常的一致特征包括病例的年轻，临床表现和CJD典型脑电图特征的缺乏。在欧洲监测系统中的其他国家尚未发现类似病例。解释这些病例似乎代表了克雅氏病的一种新变种，可能是英国独有的。这增加了它们与BSE有因果关系的可能性。尽管这可能是对这一病例群最合理的解释，但仅根据这一证据无法证实与BSE的联系。这是至关重要的，以获得进一步的信息，目前和过去的临床和神经病理学的档案克雅氏病在英国和其他地方。
Background Epidemiological surveillance of Creutzfeldt-Jakob disease (CJD) was reinstituted in the UK in 1990 to identify any changes in the occurrence of this disease after the epidemic of bovine spongiform encephalopathy (BSE) in cattle.Methods Case ascertainment of CJD was mostly by direct referral from neurologists and neuropathologists. Death certificates on which CJD was mentioned were also obtained. Clinical details were obtained for all referred cases, and information on potential risk factors for CJD was obtained by a standard questionnaire administered to patients' relatives. Neuropathological examination was carried out on approximately 70% of suspect cases. Epidemiological studies of CJD using similar methodology to the UK study have been carried out in France, Germany, Italy, and the Netherlands between 1993 and 1995.Findings Ten cases of CJD have been identified in the UK in recent months with a new neuropathological profile. Other consistent features that are unusual include the young age of the cases, clinical findings, and the absence of the electroencephalogram features typical for CJD. Similar cases have not been identified in other countries in the European surveillance system.Interpretation These cases appear to represent a new variant of CJD, which may be unique to the UK. This raises the possibility that they are causally linked to BSE. Although this may be the most plausible explanation for this cluster of cases, a link with BSE cannot be confirmed on the basis of this evidence alone. It is essential to obtain further information on the current and past clinical and neuropathological profiles of CJD in the UK and elsewhere.