Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease
Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease
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DOI：
10.1111/j.1440-1789.2007.00847.x
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发表时间：
2008-02-01
期刊：
影响因子：
2.3
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Iwasaki, Yasushi;Mimuro, Maya;Sobue, Gen
我们提出五例硬脑膜相关性克雅氏病(硬脑膜相关的克雅氏病)的临床病理分析，并回顾以往的报道。硬脑膜移植平均年龄54.4±7.3岁，CJD平均发病年龄66.0±8.2岁，平均潜伏期11.6±1.1年。平均死亡年龄67.6±8.7岁，平均病程16.8±10.4个月。在4名接受小脑幕下硬脑膜移植的患者中，CJD的发病症状反映了小脑或脑干功能障碍，而1名接受小脑幕上移植的患者的症状反映了大脑皮层的受累。所有患者均表现为快速进行性认知功能障碍，在疾病早期即可观察到脑电周期性尖波复合波和肌阵挛。神经病理检查亚急性海绵状脑病1例，全脑病变型CJD 4例。大脑新皮质、皮质下灰质和小脑皮质均有不同程度的受累，其严重程度与CJD病程有关。没有出现库鲁菌斑或花斑的病例。PrP免疫组织化学染色显示广泛的突触型PrP沉积。除有硬脑膜移植病史外，我们的硬脑膜CJD病例与典型的散发性CJD病例在临床病理上并无差异。尽管在本研究中没有评估移植硬脑膜部位和神经病理学观察之间的特定关联，但最初的症状似乎与移植部位密切相关，这表明CJD从移植部位直接传播到邻近的大脑。
We present five cases of dura mater-associated Creutzfeldt-Jakob disease (dura-CJD) that were analyzed clinicopathologically and review previous reports. The average age at dura mater transplantation was 54.4 +/- 7.3 years, and the average age at CJD onset was 66.0 +/- 8.2 years, with an average latency period of 11.6 +/- 1.1 years. The average age at death was 67.6 +/- 8.7 years, with an average CJD disease duration of 16.8 +/- 10.4 months. Symptoms of CJD onset in four patients who received dura mater transplantation below the cerebellar tent reflected cerebellar or brainstem dysfunction, whereas symptoms of one patient who received transplantation above the cerebellar tent reflected cerebral cortical involvement. All patients showed rapidly progressive cognitive impairment, and both periodic sharp-wave complexes on electroencephalogram and myoclonus were observed in the early disease stage. Neuropathologic evaluation showed one case of subacute spongiform encephalopathy and four cases of panencephalopathic-type CJD. Widespread cerebral neocortical, subcortical gray matter and cerebellar cortical involvement were observed to varying degrees, and severity tended to be associated with CJD disease duration. There were no instances of kuru plaques or florid plaques. Prion protein (PrP) immunostaining showed widespread synaptic-type PrP deposition. No differences between our dura-CJD cases and typical cases of sporadic CJD were found with respect to clinicopathologic findings, except history of dura mater transplantation. Although a specific association between the dura mater graft site and neuropathologic observations was not evaluated in the present study, the initial symptoms appear to be closely related to the graft site, indicating a direct transmission of CJD from the graft site to the adjacent brain.