Zarranz J J, Rivera Pomar J M, Salisachs P
J Neurol Sci. 1979 Oct;43(2):291-300. doi: 10.1016/0022-510x(79)90122-9.
We present two patients aged 66 and 69, with a rapidly progressive disease (10 and 15 months' duration) in which the presenting symptom was instability of gait. Later dementia was also a prominent feature. One case had myoclonus. Repeated EEGs showed symmetrical slowing in one case and periodic generalised bursts of triphasic waves at 1 cps superimposed upon a slow (3-4 cps) background activity in the other. The pathological findings consisted of classical Creutzfeld-Jakob disease (CJD), Kuru plaques (KP) were disseminated in the brain, but were more numerous in the cerebellum, putamen and thalamus. Neurons with large vacuoles in the cytoplasm were numerous in the putamen, thalamus and anterior horns. Stress is laid upon the common findings in both CJD and Kuru (K) (clinical features, pathological data, lack of antibody response, transmissibility, change in pattern on transmission). The possibility of a common origin of the two diseases is discussed.
我们报告了两名分别为66岁和69岁的患者,患有快速进展性疾病(病程分别为10个月和15个月),首发症状为步态不稳。后来痴呆也成为突出特征。其中1例有肌阵挛。多次脑电图检查显示,1例表现为对称性慢波,另1例在慢(3 - 4次/秒)背景活动上叠加有每秒1次的周期性广泛性三相波爆发。病理结果为经典型克雅氏病(CJD),库鲁斑(KP)在脑内散在分布,但在小脑、壳核和丘脑更为多见。壳核、丘脑和前角可见大量胞质内有大空泡的神经元。着重阐述了CJD和库鲁病(K)的共同发现(临床特征、病理数据、缺乏抗体反应、传染性、传播时模式的改变)。并讨论了这两种疾病共同起源的可能性。