Brandel Jean-Philippe, Heath Craig A, Head Mark W, Levavasseur Etienne, Knight Richard, Laplanche Jean-Louis, Langeveld Jan Pm, Ironside James W, Hauw Jean-Jacques, Mackenzie Jan, Alpérovitch Annick, Will Robert G, Haïk Stéphane
Institut National de la Santé et de la Recherche Médicale, Equipe Avenir Human Prion Diseases, Paris, F-75013, France.
Ann Neurol. 2009 Mar;65(3):249-56. doi: 10.1002/ana.21583.
Variant Creutzfeldt-Jakob disease (vCJD) was first reported in the United Kingdom in 1996. Since then, the majority of cases have been observed in the United Kingdom where there was a major epidemic of bovine spongiform encephalopathy. France was the second country affected. To address the hypothesis of the involvement of a common strain of agent, we have compared clinical, neuropathological, and biochemical data on vCJD patients from both countries.
In France and the United Kingdom, epidemiological and clinical data were obtained from analysis of medical records and direct interview of the family of the patients using the same standardized questionnaire in both countries. When brain material was available, we performed with similar methods a comparative study of brain lesions and PrP(res) glycoform ratios in both vCJD populations.
Clinical data, genetic background, neuropathological finding, and biochemical findings in the 185 patients observed in France (n = 23) and the United Kingdom (n = 162) were similar except for age at death. Currently, blood transfusion is a risk factor identified only in the United Kingdom.
The close similarity between the cases of vCJD in France and the United Kingdom supports the hypothesis that a common strain of infectious agent is involved in both countries. The 5-year delay in the peak that we observed in France compared with the United Kingdom fits well with the increase in the importation of beef products to France from the United Kingdom between 1985 and 1995.
变异型克雅氏病（vCJD）于1996年首次在英国被报道。自那时起，大多数病例在英国被观察到，该国曾发生过牛海绵状脑病大流行。法国是第二个受影响的国家。为了验证涉及一种共同病原体菌株的假说，我们比较了来自两国的vCJD患者的临床、神经病理学和生物化学数据。
在法国和英国，通过分析病历以及使用两国相同的标准化问卷直接访谈患者家属来获取流行病学和临床数据。当有脑组织材料时，我们用类似的方法对两国vCJD患者群体的脑病变和PrP（res）糖型比例进行了比较研究。
在法国观察到的23例和英国观察到的162例共185例患者中，除死亡年龄外，临床数据、遗传背景、神经病理学发现和生物化学发现相似。目前，输血是仅在英国被确定的一个风险因素。
法国和英国vCJD病例之间的密切相似性支持了两国涉及一种共同感染病原体菌株的假说。我们观察到法国与英国相比，发病高峰延迟了5年，这与1985年至1995年间从英国进口到法国的牛肉产品增加情况相符。
