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.

中文翻译：

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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

我们报告了一例早期散发性克雅氏病 (sCJD) 并发脑出血 (ICH) 的病例，根据尸检分类为 MM1 + 2C 型。一名 61 岁的日本男性因重复使用相同词等口语困难就诊于我院。他在症状出现后第 7 天住院，磁共振成像的弥散加权图像显示额叶皮层和尾状核的高信号区域。症状出现后第 11 天，头部计算机断层扫描显示右侧枕叶和顶叶 ICH。常规实验室评估和血管造影未发现 ICH 的原因。症状出现后第15天观察到四肢肌阵挛和嗜睡。在症状出现大约两个月后，他达到了无动力缄默状态。脑脊液检测显示实时震颤诱发转化和 14-3-3 蛋白阳性。脑电图显示周期性尖波复合波。根据诊断标准做出可能的克雅氏病的临床诊断。肺炎复发后，于出现症状后第 103 天去世。尸检显示右侧后扣带回有脑出血。未获得可能导致 ICH 的病理变化。尽管不可否认 sCJD 对 ICH 发病的影响，但 ICH 的原因尚不清楚。朊病毒蛋白免疫组化显示以下结果：（1）ICH稀少的组织中微弱的突触型沉积物；(2)大脑皮层突触型沉积物，表现为细小空泡；(3) 颞下回和舌回的液泡周围型沉积，表现为大的融合液泡。虽然临床上可考虑为 MM1 型 sCJD，但该病例经神经病理学诊断为 MM1+2C 型 sCJD。表明ICH可能发生在早期sCJD。为了改善 sCJD 的预后，并发症的治疗和仔细的随访很重要。此外，病理诊断对于sCJD型诊断是必不可少的。