Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
Department of Neurosurgery, Local incorporated Administrative Agency, Rinku General Medical Center, Izumisano, Japan.
Neuropathology. 2020 Aug;40(4):399-406. doi: 10.1111/neup.12658. Epub 2020 May 4.
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.
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，根据尸检结果，该病例被归类为 MM1+2C 型。一名 61 岁的日本男性因语言困难，包括重复使用相同的词语而就诊。他在发病后第 7 天入院，磁共振成像的弥散加权图像显示额叶和尾状核的高信号区域。在发病后第 11 天，头部计算机断层扫描显示右侧枕叶和顶叶的 ICH。常规实验室检查和血管造影均未发现 ICH 的原因。在发病后第 15 天出现四肢肌阵挛和嗜睡。在发病后大约两个月，他达到无动性缄默症状态。脑脊液检测显示实时震颤诱导转换和 14-3-3 蛋白阳性。脑电图显示周期性尖波复合物。根据诊断标准，临床诊断为可能的克雅氏病。在肺炎复发后，他在发病后第 103 天去世。尸检显示右侧后扣带回的 ICH。未获得可能导致 ICH 的病理变化。虽然 sCJD 对 ICH 发病的影响不可否认，但 ICH 的原因尚不清楚。朊病毒蛋白免疫组织化学显示以下结果：（1）ICH 组织稀疏处的弱突触型沉积物；（2）皮质的突触型沉积物，显示出细小的空泡；（3）颞下回和舌回的围空泡型沉积物，显示出频繁的大融合空泡。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。结果表明，ICH 可能发生在早期 sCJD 中。为了改善 sCJD 的预后，治疗并发症和密切随访非常重要。此外，病理诊断对于 sCJD 类型诊断是必不可少的。
