Creutzfeldt-Jakob disease in unusually young patients who consumed venison
Creutzfeldt-Jakob disease in unusually young patients who consumed venison
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DOI：
10.1001/archneur.58.10.1673
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发表时间：
2001-10-01
影响因子：
--
通讯作者：
Nichols, CR
中科院分区：
文献类型：
--
作者：
Belay, ED;Gambetti, P;Nichols, CR
背景：人类的克雅氏病（CJD）和鹿和麋鹿的慢性消耗性疾病（CWD）发生在美国。最近报道了3例经常食用鹿肉或麋鹿肉的异常年轻的CJD患者，引起了人们对CWD可能的人畜共患传播的担忧。目的：研究CWD向人类传播的可能性。1997- 2000年在美国的28岁、28岁和30岁的CJD患者。我们查阅了医疗记录，采访了家庭成员和州野生动物和农业官员。脑组织样本用组织病理学、免疫组化、免疫印迹或朊蛋白基因分析进行检测。主要结果测量：是否存在已确定的CJD危险因素，在CWD流行区猎鹿和麋鹿，并比较3例患者与新变异CJD和牛海绵状脑病之间的人畜共患联系的证据。没有一名患者有确定的CJD风险因素或欧洲旅行史。两名患者狩猎狩猎动物，1人是猎人的女儿。与新变异型CJD患者不同，这3例患者没有独特的神经病理学表现、临床病理学同质性、朊病毒蛋白基因密码子129的一致性或朊病毒特征与经典变异型不同。尽管3名食用鹿肉的异常年轻的克雅氏病患者的出现表明可能与慢性消耗病有关，我们的跟进调查并没有发现有力证据证明两者有因果关系。持续的CJD监测对于继续评估CWD传播给人类的风险（如果有的话）仍然很重要。
Background: Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD.Objective: To examine the possible transmission of CWD to humans.Patients: Three unusually young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000.Methods: We reviewed medical records and interviewed family members and state wildlife and agriculture officials. Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein gene analyses.Main Outcome Measures: Presence or absence of established CJD risk factors, deer and elk hunting in CWD- endemic areas, and comparison of the evidence for the 3 patients with that of a zoonotic link between new variant CJD and bovine spongiform encephalopathy.Results: None of the patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion protein gene, or prion characteristics different from those of classic variants.Conclusions: Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.