Murray K, Ritchie D L, Bruce M, Young C A, Doran M, Ironside J W, Will R G
National Creutzfeldt-Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2 XU, UK.
J Neurol Neurosurg Psychiatry. 2008 Jan;79(1):14-8. doi: 10.1136/jnnp.2006.104570. Epub 2007 May 23.
Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.
Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken, and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.
In the UK, two cases of sporadic CJD in adolescents have been identified, dying at ages 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.
The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential for accurate diagnosis of human prion disease.
散发性克雅氏病（CJD）主要影响老年人群，45岁以下的病例罕见，发病或死亡年龄小于20岁的情况更是罕见。
自1970年起可获取英国散发性CJD系统研究的数据。对临床和病理数据进行回顾，以识别非典型病例，包括散发性CJD年龄范围两端的病例。对非典型病例进行详细分析，并在部分病例中开展实验室传播研究，以提供有关感染因子特征的信息。
在英国，已确诊两例青少年散发性CJD病例，分别于16岁和20岁死亡。首例病例早于牛海绵状脑病流行，第二例病例的特征，包括实验室传播研究结果，均符合散发性而非变异型CJD的诊断。
本报告中的病例表明，散发性CJD可在非常年轻的年龄发病，变异型CJD并非该年龄组中发生的唯一形式的CJD，且神经病理学检查对于准确诊断人类朊病毒病至关重要。
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