Heinemann U, Krasnianski A, Meissner B, Varges D, Kallenberg K, Schulz-Schaeffer W J, Steinhoff B J, Grasbon-Frodl E M, Kretzschmar H A, Zerr I
National Reference Center for TSE, Department of Neurology, Georg-August University Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen, Germany.
Brain. 2007 May;130(Pt 5):1350-9. doi: 10.1093/brain/awm063.
Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a worldwide incidence of 1-1.5 per million. As in other countries, a CJD surveillance unit with a clinical and neuropathological approach was established in Goettingen (Germany) in 1993. Here we report the epidemiological data from a prospective 12-year surveillance. Since 1993, there has been an increasing incidence of CJD, from 0.7 in 1993 to 1.6 in 2005 with a quite stable level since 1998. During this period, the proportion of patients with MV and VV codon 129 genotype rose, possibly because of better identification of atypical subtypes. Six percent of all patients had a PRNP mutation, mainly D178N-129M (FFI), E200K and V210I. Iatrogenic CJD was a rare phenomenon. No patient infected by cadaveric growth hormone extracts was reported. Furthermore, no variant CJD patient has yet been identified in Germany. Differential diagnoses revealed a variety of neurodegenerative diseases, with Alzheimer's disease in the lead. One-third of the non-CJD patients included in this study suffered from a potentially treatable disorder such as metabolic or inflammatory diseases. The incidence and mortality rates in Germany are similar to those in other European countries. In contrast, however, acquired forms, such as iatrogenic and variant CJD are still rare in Germany or have not yet been identified.
克雅氏病（CJD）是一种罕见的致命性神经退行性疾病，全球发病率为百万分之一至百万分之一点五。与其他国家一样，1993年在德国哥廷根成立了一个采用临床和神经病理学方法的克雅氏病监测单位。在此，我们报告一项为期12年的前瞻性监测的流行病学数据。自1993年以来，克雅氏病的发病率一直在上升，从1993年的0.7上升至2005年的1.6，自1998年以来保持相当稳定的水平。在此期间，甲硫氨酸/缬氨酸密码子129基因型患者的比例有所上升，这可能是因为对非典型亚型的识别能力有所提高。所有患者中有6%携带PRNP突变，主要是D178N-129M（致死性家族性失眠症）、E200K和V210I。医源性克雅氏病是一种罕见现象。未报告有患者因尸体生长激素提取物感染。此外，德国尚未发现变异型克雅氏病患者。鉴别诊断发现了多种神经退行性疾病，其中阿尔茨海默病最为常见。本研究纳入的非克雅氏病患者中有三分之一患有潜在可治疗的疾病，如代谢性或炎症性疾病。德国的发病率和死亡率与其他欧洲国家相似。然而，相比之下，医源性和变异型克雅氏病等后天性形式在德国仍然罕见或尚未被发现。
