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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Coexistence of mixed phenotype Creutzfeldt-Jakob disease, Lewy body disease and argyrophilic grain disease plus histological features of possible Alzheimer's disease: A multi-protein disorder in an autopsy case
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Coexistence of mixed phenotype Creutzfeldt-Jakob disease, Lewy body disease and argyrophilic grain disease plus histological features of possible Alzheimer's disease: A multi-protein disorder in an autopsy case

机译：混合表型Creutzfeldt-Jakob病，路易氏体病和嗜银粒病的并存以及可能的阿尔茨海默氏病的组织学特征：尸检病例中的一种多蛋白疾病

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摘要

We report hereby an autopsy case of sporadic mixed phenotype CJD without hereditary burden and a long-term clinical course. An 80-year old man was diagnosed with mild cognitive impairment 27 months before death, caused by bronchopneumonia and severe respiratory impairment. During this time, the patient developed gradual mental deterioration, some sleeping problems and myoclonus. Other clinical manifestations were progressive gait problems, language deterioration, presence of primitive reflexes and irritability. In keeping with those symptoms, a rapidly evolving dementia was clinically suspected. Cerebrospinal fluid test for 14-3-3 protein was negative. However, an abnormal EEG and MRI at end-stage of disease were finally consistent with CJD. Post-mortem examination revealed a massive cortical neuronal loss with associated reactive astrocytosis, also evident in the white matter. Diffuse spongiform changes involving some basal ganglia, especially medial thalamus, some troncoencephalic nuclei, mainly inferior olivary nucleus and the molecular layer of the cerebellum were seen. Immunorreactive deposits for anti-prion protein antibody were present at different areas of the CNS. Additionally, Lewy bodies were observed at the brainstem and amygdala. Furthermore, argirophilic grains together with oligodendroglial coiled bodies and pre-tangle inclusions in the neurons from the limbic system containing hyperphosphorylated 4R tau were noted. To the best of our knowledge, this is the first case of CJD combined with Lewy body disease and argirophilic grain disease. Furthermore, we believe this case is an extremely rare combination of MM2-cortical-type and MM2-thalamic-type sporadic CJD (sCJD), which explains the broad spectrum of MM2-type sCJD findings and symptoms. Moreover, histological features of possible Alzheimer's disease were also reported.
机译：我们在此报告零星混合表型CJD的尸检病例，没有遗传负担和长期临床过程。一名80岁的男子在死亡前27个月被诊断为轻度认知障碍，由支气管肺炎和严重的呼吸障碍引起。在此期间，患者逐渐出现精神恶化，一些睡眠问题和肌阵挛。其他临床表现是进行性步态问题，语言恶化，原始反射的存在和易怒。为了与这些症状保持一致，临床上怀疑有快速发展的痴呆症。脑脊液中14-3-3蛋白测试阴性。然而，疾病末期的脑电图和MRI异常最终与CJD一致。验尸后发现皮质神经元大量丧失，伴有反应性星形细胞增多，在白质中也很明显。弥漫性海绵状变化涉及到一些基底神经节，特别是内侧丘脑，一些脑小脑核，主要是下橄榄核和小脑分子层。抗-蛋白抗体的免疫反应性沉积物存在于中枢神经系统的不同区域。另外，在脑干和杏仁核处观察到路易体。此外，还注意到嗜银粒与少突神经胶质盘绕体以及来自含高磷酸化4R tau的边缘系统神经元中的缠结前夹杂物。据我们所知，这是首例CJD合并路易体病和嗜银粒病的病例。此外，我们认为这种情况是MM2皮质型和MM2丘脑型散发性CJD（sCJD）的极为罕见的组合，这解释了MM2型sCJD的广泛发现和症状。此外，还报道了可能的阿尔茨海默氏病的组织学特征。

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