Department of Neurology, Eisei Hospital, 583-15 Kunugidamachi, Hachioji, Tokyo, 193-0942, Japan.
Department of Neuropathology (the Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, 35-2 Sakae-cho, Itabashi-ku, Tokyo, 173-0015, Japan.
BMC Neurol. 2021 Dec 10;21(1):480. doi: 10.1186/s12883-021-02504-1.
Late-stage Parkinson's disease (PD) often presents with neuropsychiatric symptoms such as dementia, psychosis, excessive daytime sleepiness, apathy, depression, and anxiety. However, neuropsychiatric symptoms are the cardinal features of Creutzfeldt-Jakob disease (CJD), raising the possibility that CJD may be an overlooked condition when it accompanies late-stage PD.
We describe a female autopsy case of PD with a typical clinical course of 17 years, in which CJD overlapped with PD during the final year of the patient's life. The patient died aged 85 years. Neuropathological features included widespread Lewy body-related α-synucleinopathy predominantly in the brainstem and limbic system, as well as the typical pathology of methionine/methionine type 1 CJD in the brain.
Our case demonstrates the clinicopathological co-occurrence of PD and CJD in a sporadic patient. The possibility of mixed pathology, including prion pathology, should be taken into account when neuropsychiatric symptoms are noted during the disease course of PD.
晚期帕金森病（PD）常伴有神经精神症状，如痴呆、精神病、日间过度嗜睡、淡漠、抑郁和焦虑。然而，神经精神症状是克雅氏病（CJD）的主要特征，这使得 CJD 在伴随晚期 PD 时可能被忽视。
我们描述了一例 PD 的女性尸检病例，其具有典型的 17 年临床病程，在患者生命的最后一年，CJD 与 PD 重叠。患者 85 岁死亡。神经病理学特征包括广泛的与路易体相关的 α-突触核蛋白病，主要位于脑干和边缘系统，以及大脑中典型的蛋氨酸/蛋氨酸 1 型 CJD 病理。
我们的病例表明 PD 和 CJD 在散发性患者中同时存在临床和病理表现。当 PD 病程中出现神经精神症状时，应考虑包括朊病毒病理学在内的混合病理学的可能性。
