An 83-year-old Japanese man presented with gait disturbance followed by rapidly-progressive cognitive impairment. Magnetic resonance diffusion-weighted images showed extensive hyperintense regions in the cerebral cortex. Four weeks after symptom onset, myoclonus appeared, and the patient developed difficulty swallowing; intravenous peripheral continuous infusions without vitamin supplementation were administered during the last two months of the patient’s life. The patient reached the akinetic mutism state and died 12 weeks after symptom onset due to sepsis. The brain weighed 940 g and showed general cerebral atrophy. Extensive spongiform change were observed in the cerebral cortex, striatum, thalamus, and cerebellar cortex, but gliosis was generally mild. Numerous newly-developed hemorrhage foci were observed in the mammillary body, the areas adjacent to the third and fourth ventricles, and the periaqueduct of the midbrain; however, proliferation of capillaries and endothelium and collections of macrophages were relatively inconspicuous. These findings suggested comorbidity with the acute stage of Wernicke encephalopathy (WE). Immunostaining showed extensive diffuse synaptic-type prion protein deposition in the gray matter. According to the neuropathological, genetic, and molecular findings, the present case was finally diagnosed as MM1-type sporadic Creutzfeldt-Jakob disease (CJD) with WE. We should remain alert to the diagnosis of WE when CJD is suspected, and it is necessary to consider the complications of both diseases. This report emphasizes the importance of pathological investigations for the diagnosis of CJD, WE, and the coexistence of both.

一名83岁的日本男子出现步态障碍，随后迅速发展为认知障碍。磁共振扩散加权图像显示大脑皮层中广泛的高强度区域。症状发作四周后，出现肌阵挛，患者吞咽困难。在患者生命的最后两个月内，进行了不添加维生素的静脉周围连续输注。该患者达到了运动性mut默状态，并在症状发作后因败血症死亡12周。大脑重940克，显示出一般性脑萎缩。在大脑皮层，纹状体，丘脑和小脑皮层中观察到广泛的海绵状变化，但神经胶质变性一般较轻。在乳头体内观察到许多新近形成的出血灶，与第三和第四脑室相邻的区域，以及中脑的水周导管；然而，毛细血管和内皮的增殖以及巨噬细胞的收集相对不明显。这些发现提示患有韦尼克脑病（WE）的急性期合并症。免疫染色显示灰质中广泛的弥漫性突触型病毒蛋白沉积。根据神经病理学，遗传学和分子学发现，本病例最终被诊断为伴WE的MM1型散发性Creutzfeldt-Jakob病（CJD）。当怀疑患有克雅氏病时，我们应该对WE的诊断保持警惕，有必要考虑两种疾病的并发症。该报告强调了病理学调查对于诊断克雅氏病，WE以及两者共存的重要性。