Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting
Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting
复制标题
DOI：
10.1001/archneur.56.3.357
复制
发表时间：
1999-03-01
影响因子：
--
通讯作者：
Kitamoto, T
中科院分区：
文献类型：
--
作者：
Shimizu, S;Hoshi, K;Kitamoto, T
背景：许多报道的医源性克雅氏病（CJD）是在移植被CJD朊病毒污染的尸体硬脑膜（硬脑膜相关性CJD）后发生的。已知其临床病理与散发性克雅氏病相似。我们在此报告2例具有非典型临床病理特征的硬脑膜相关性CJD尸检病例。患者：2例患者在尸体硬脑膜移植神经外科治疗后10年或11年出现进行性共济失调和精神恶化，分别于发病后8个月和17个月死亡。结果：临床表现不典型，无肌阵挛发作或迟发性肌阵挛，脑电图显示周期性同步放电。他们在病理学上有几个方面是独特的。最显著的特征是在大脑的多个区域存在许多朊蛋白（PrP）斑块。其中一些是“繁花似锦”型，周围是海绵状变化区，已知这是新型克雅氏病的标志。海绵状变性的分布也很独特，在丘脑、基底神经节和小脑齿状核中分布强烈，而在大脑中分布较轻。患者的PrP基因未发生突变。从硬脑膜相关的CJD患者脑组织中提取的PrP异常异构体的大小和糖型模式与从散发的CJD患者脑组织中提取的PrP异常异构体没有明显差异。结论：这2例患者在临床病理上不同于典型的硬脑膜相关CJD病例。在硬脑膜相关性CJD中，它们可能是一种带有花状斑块的亚型。
Background: Many reported cases of iatrogenic Creutzfeldt-Jakob disease (CJD) developed after grafting cadaveric dura mater contaminated with CJD prions (dura-associated CJD). They are known to be clinicopathologically similar to sporadic CJD. We report herein 2 autopsy cases of dura-associated CJD with atypical clinicopathological features.Patients: Two patients presented with progressive ataxia and mental deterioration 10 or 11 years after neurosurgical treatment with cadaveric dural grafting, which led to their deaths at 8 and 17 months, respectively, after onset.Results: The cases were clinically atypical in exhibiting no or late occurrence of myoclonus and periodic synchronous discharges on electroencephalographic studies. They were pathologically unique in several aspects. The most striking feature was the presence of many prion protein (PrP) plaques in multiple areas in the brain. Some of them were the "florid" type surrounded by a zone of spongiform changes known to be a hallmark for the new variant CJD. The distribution of spongiform degeneration was also unique in that it was intense in the thalamus, basal ganglia, and the dentate nuclei of the cerebellum but milder in the cerebrum. There were no mutations in the PrP gene of the patients. There was no major difference in the size and glycoform pattern between the abnormal isoform of PrP extracted from the brain tissue from the dura-associated cases of CJD and that from a sporadic case of CJD.Conclusions: These 2 cases are clinicopathologically distinct from typical dura-associated cases of CJD. They may be a subtype with florid-type plaques in dura-associated CJD.