Hainfellner J A, Jellinger K, Diringer H, Guentchev M, Kleinert R, Pilz P, Maier H, Budka H
Institute of Neurology, University of Vienna, Austria.
J Neurol Neurosurg Psychiatry. 1996 Aug;61(2):139-42. doi: 10.1136/jnnp.61.2.139.
Between 1969 and 30 September 1995, 79 Austrian patients had Creutzfeldt-Jakob disease (CJD) diagnosed neuropathologically by necropsy or biopsy. The annual incidence has significantly increased in recent years (average 0.18 per million in 1969-85, and 0.67 per million in 1986-94; estimate for 1995: 1.5 per million). Also, the percentage of patients with CJD over 70 years at death increased significantly until 1989 but is since in decline. There is no regional clustering, familial occurrence, or recognised iatrogenic risk. One patient had a 10 year history of intramuscular injection of purified bovine RNA preparation (Regeneresen) from various organs including the brian. The ages at death are symmetrically distributed around the median of 64 years. The median duration of disease is four months. Most patients (76%) died within six months of onset. Retrospectively, 86% of patients fulfilled clinical criteria of probable or possible CJD. Neuropathology showed the classic triad of spongiform change, astrogliosis, and neuronal loss in most cases. Two cases did not show unequivocal tissue alterations, but anti-PrP immunocytochemistry detected PrP deposits also in these cases. It is concluded that the recent rise in incidence of CJD in Austria most likely reflects increased awareness and diagnosis of CJD rather than a real increase. As bovine spongiform encephalopathy (BSE) has not been reported in Austria, the data do not support a link between a rise in incidence of sporadic CJD and BSE.
1969年至1995年9月30日期间，79例奥地利患者经尸检或活检进行神经病理学诊断，确诊为克雅氏病（CJD）。近年来，年发病率显著上升（1969 - 1985年平均为百万分之0.18，1986 - 1994年为百万分之0.67；1995年估计为百万分之1.5）。此外，死亡时年龄超过70岁的CJD患者比例在1989年前显著增加，但此后呈下降趋势。不存在区域聚集、家族发病或公认的医源性风险。一名患者有10年肌肉注射从包括脑在内的各种器官提取的纯化牛RNA制剂（Regeneresen）的病史。死亡年龄以64岁中位数为中心呈对称分布。疾病的中位持续时间为4个月。大多数患者（76%）在发病后6个月内死亡。回顾性分析，86%的患者符合可能或疑似CJD的临床标准。神经病理学在大多数病例中显示出海绵状改变、星形胶质细胞增生和神经元丢失的经典三联征。有2例未显示明确的组织改变，但抗PrP免疫细胞化学检测在这些病例中也发现了PrP沉积物。得出的结论是，奥地利近期CJD发病率的上升很可能反映了对CJD的认识和诊断增加，而非实际增加。由于奥地利未报告牛海绵状脑病（BSE），这些数据不支持散发性CJD发病率上升与BSE之间存在关联。
