Autopsied case of non-plaque-type dura mater graft-associated Creutzfeldt-Jakob disease presenting with extensive amyloid- deposition
Autopsied case of non-plaque-type dura mater graft-associated Creutzfeldt-Jakob disease presenting with extensive amyloid- deposition
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DOI：
10.1111/neup.12503
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发表时间：
2018-10-01
期刊：
影响因子：
2.3
通讯作者：
Yoshida, Mari
中科院分区：
文献类型：
--
作者：
Iwasaki, Yasushi;Imamura, Kazuhiro;Yoshida, Mari
我们报告一例非斑块型硬脑膜移植物相关性克雅氏病（dCJD）的尸检病例，脑内有广泛的β淀粉样蛋白（A β）沉积。一名39岁的日本女性出现记忆障碍和异常行为。患者有开颅术和硬脑膜移植物移植治疗头部损伤的病史，发生在她19岁时。磁共振成像（MRI）显示扩散加权图像上大脑皮层和纹状体高信号，特别是硬脑膜移植右侧。她的临床症状，包括快速进展的认知功能障碍，肌阵挛和脑电图周期性尖波复合体，无法与典型的散发性CJD病例区分。患者于发病后11个月死亡，病理检查显示广泛的海绵状变性伴朊蛋白（PrP）沉积，无库鲁斑;这些观察结果与典型的散发性CJD病例基本相同。此外，A β免疫组化显示大脑新皮质广泛弥漫性染色、斑块型沉积、软脑膜阳性染色和大脑淀粉样血管病。虽然MRI结果提示病理受累起源于硬脑膜材料移植的右侧，但PrP和A β沉积没有明显的区域化和偏侧性。几乎未发现Tau病理学（包括神经元缠结）。蛋白质磷酸化突触核蛋白和磷酸化反式激活反应DNA结合蛋白43 kDa的免疫染色未检测到。虽然这份报告只描述了一个病例，但根据详细的临床和病理观察以及以前的dCJD报告，人们做出了各种推测。特别是，这份报告提供了重要的深入了解的特点和进展的dCJD病理及其与A β病理的关系。
We present an autopsied case of non-plaque-type dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) with extensive amyloid-beta (A beta) deposition in the brain. A 39-year-old Japanese woman presented with memory disturbance and abnormal behavior. The patient had a history of craniotomy with dura matter-graft transplant for a head injury which occurred when she was 19years old. Magnetic resonance imaging (MRI) showed hyperintensities in the cerebral cortex and striatum on diffusion-weighted images, particularly on the dura mater-grafted right side. Her clinical symptoms, including rapidly progressing cognitive impairment, myoclonus, and periodic sharp wave complexes on electroencephalogram, could not be distinguished from typical sporadic CJD cases. The patient died 11months after symptom onset, and pathological investigations showed extensive spongiform degeneration with prion protein (PrP) deposition without Kuru plaques; these observations were essentially the same as those of typical sporadic CJD cases. Furthermore, A beta immunohistochemistry showed extensive diffuse staining in the cerebral neocortex, plaque-type deposition, positive staining in the pia mater, and cerebral amyloid angiopathy. Although the MRI findings suggested that the pathological involvement originated from the dura mater-grafted right side, the PrP and A beta depositions showed no apparent regionalization and laterality. Tau-pathology including neurofibrillary tangles was hardly identified. The proteins phosphorylated -synuclein and phosphorylated transactivation response DNA-binding protein 43 kDa were not detected on immunostaining. Although this report describes only one case, various speculations were made based on detailed clinical and pathological observations in conjunction with previous reports of dCJD. In particular, this report provides significant insight into the characteristics and progression of dCJD pathology and its relationship with A beta pathology.