Autopsied case of sporadic Creutzfeldt-Jakob disease pathologically classified as MM1+2C-type
Autopsied case of sporadic Creutzfeldt-Jakob disease pathologically classified as MM1+2C-type
复制标题
散发性克雅氏病尸检一例，病理学分类为MM1 2C型
DOI：
10.1111/neup.12557
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发表时间：
2019
期刊：
影响因子：
2.3
通讯作者：
Yoshida M.
中科院分区：
文献类型：
--
作者：
Iwasaki Y;Kato H;Ando T;Akagi A;Mimuro M;Miyahara H;Kobayashi A;Kitamoto T;Yoshida M.
我们遇到了一个散发性克雅氏病（CJD）的尸检病例，病理分类为MM 1 +2C-型，朊蛋白（PrP）的蛋白质印迹分析主要显示1型瘙痒症PrP（PrPSc），但也有部分混合型2型PrPSc。一名日本妇女在86岁时主诉视力障碍，随后出现定向障碍和记忆障碍。磁共振成像（MRI）显示弥散加权图像上大脑皮质高信号。患者在症状发作后2个月死亡;她的病情未达到运动不能性缄默状态，脑电图和肌阵挛上的周期性尖波复合体未被识别。大脑重量为1100 g，神经病理学检查显示大脑皮层中存在广泛的细空泡型海绵状变化。在一些皮质区域，还存在大融合空泡型海绵状变化。神经胶质增生和肥大性星形胶质细胞增生通常是轻度的，神经元的组织稀疏和神经元丢失不明显。PrP免疫染色显示大脑灰质中弥漫性突触型PrP沉积，但一些具有大融合空泡的区域显示空泡周围型沉积。我们推测，根据临床病理结果和以前的报告，大多数MM 1型散发性CJD病例可能与2型PrPSc相关，至少部分与大脑某些区域相关。
We encountered an autopsy case of sporadic Creutzfeldt‐Jakob disease (CJD) pathologically classified as MM1+2C‐type, where Western blot analysis of prion protein (PrP) mainly showed type‐1 scrapie PrP (PrPSc) but also, partially, mixed type‐2 PrPSc. A Japanese woman complained of visual disorder at the age of 86 years and then showed disorientation and memory disturbances. Magnetic resonance imaging (MRI) showed cerebral cortical hyperintensity on diffusion‐weighted images. The patient died 2 months after the onset of symptoms; her condition did not reach the akinetic mutism state and periodic sharp‐wave complexes on electroencephalography and myoclonus were not recognized. The brain weighed 1100 g and neuropathological examination showed extensive fine vacuole‐type spongiform changes in the cerebral cortex. In some cortical regions, large confluent vacuole‐type spongiform changes were also present. Gliosis and hypertrophic astrocytosis were generally mild, and tissue rarefaction of the neuropil and neuronal loss were not apparent. PrP immunostaining showed diffuse synaptic‐type PrP deposition in the cerebral gray matter, but some regions with large confluent vacuoles showed perivacuolar‐type deposition. We speculated, based on the clinicopathological findings and previous reports, that most MM1‐type sporadic CJD cases may be associated with type‐2 PrPSc, at least partially, within certain regions of the cerebrum.