Identification of intracerebral hemorrhage in the early-phase of MM1+2C-type sporadic Creutzfeldt-Jakob disease: A case report
Identification of intracerebral hemorrhage in the early-phase of MM1+2C-type sporadic Creutzfeldt-Jakob disease: A case report
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MM1 2C型散发性克雅氏病早期脑出血的鉴别：一例报告
DOI：
10.1111/neup.12658
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发表时间：
2020
期刊：
影响因子：
2.3
通讯作者：
Yoshida M.
中科院分区：
文献类型：
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作者：
Akagi A;Iwasaki Y;Yamamoto A;Matsuura H;Ikeda T;Mimuro M;Riku Y;Miyahara H;Kitamoto T;Yoshida M.
我们报告一例早期散发性克雅氏病（sCJD）并发脑出血（ICH），根据尸检分类为MM1 + 2C型。一名61岁的日本男子因说话困难来我院就诊，包括重复使用相同的单词。患者在症状出现后第7天入院，磁共振弥散加权成像显示额叶皮质和尾状核高信号区。在症状出现后第11天，头部计算机断层扫描显示脑出血在右侧枕叶和顶叶。常规实验室检查和血管造影未发现脑出血的病因。出现症状后第15天观察四肢肌阵挛和嗜睡。他在症状出现大约两个月后达到不动性缄默症的状态。脑脊液试验显示实时震动诱导的转换和14‐3‐3蛋白阳性。脑电图显示周期性尖波复合体。根据诊断标准诊断为可能的克雅氏病。肺炎复发后，于出现症状后第103天去世。尸检显示右侧扣带回后脑出血。未见可能引起脑出血的病理改变。虽然sCJD对脑出血发病的影响是不可否认的，但脑出血的病因尚不清楚。朊蛋白免疫组化结果显示：(1)脑出血组织中突触型沉积物较弱；(2)大脑皮层突触型沉积，表现为细空泡；(3)颞下回和舌回的空泡周围型沉积物，经常出现大的汇合空泡。虽然临床上可以认为是MM1型sCJD，但该病例的神经病理学诊断为MM1 + 2C型sCJD。研究表明，脑出血可能发生在早期sCJD。为了改善sCJD的预后，治疗并发症和仔细随访是很重要的。此外，病理诊断是sCJD类型诊断必不可少的。
We report a case of early‐phase sporadic Creutzfeldt–Jakob disease (sCJD) complicated by intracerebral hemorrhage (ICH), classified as MM1 + 2C‐type based on autopsy. A 61‐year‐old Japanese man presented to our hospital with speaking difficulties including repeated usage of the same words. He was hospitalized on the seventh day after symptom onset, and diffusion‐weighted images on magnetic resonance imaging showed hyperintense regions in the frontal cortex and caudate nucleus. On the 11th day after symptom onset, head computed tomography revealed ICH in the right occipital and parietal lobes. Routine laboratory evaluations and angiography revealed no cause of ICH. Myoclonus of the extremities and drowsiness were observed on the 15th day after symptom onset. He reached the state of akinetic mutism approximately two months after symptom onset. The cerebrospinal fluid test revealed positive real‐time quaking‐induced conversion and 14‐3‐3 protein. Electroencephalography revealed periodic sharp wave complexes. A clinical diagnosis of probable Creutzfeldt–Jakob disease was made according to the diagnostic criteria. After a relapse of pneumonia, he passed away on the 103rd day after symptom onset. Postmortem examination revealed ICH in the right posterior cingulate gyrus. No pathological change that might have caused ICH was obtained. Although the effect of sCJD on the onset of ICH is undeniable, the cause of ICH was unknown. Prion protein immunohistochemistry revealed the following results: (1) weak synaptic‐type deposits in the tissue rarefacted by ICH; (2) synaptic‐type deposits in the cerebral cortex, which showed fine vacuoles; and (3) perivacuolar‐type deposits in the inferior temporal gyrus and lingual gyrus, which showed frequent large confluent vacuoles. Although it could be considered MM1‐type sCJD clinically, this case was neuropathologically diagnosed as having MM1 + 2C‐type sCJD. It was shown that ICH may occur in early‐phase sCJD. To improve sCJD prognosis, treatment of complications and careful follow up are important. Furthermore, pathological diagnosis is indispensable for sCJD type diagnosis.