Toyoshima Yasuko, Takahashi Hitoshi, Katada Shinnichi, Kojima Naoyuki, Tada Mari, Tani Takashi, Koike Ryoko, Nozawa Takanori, Aida Izumi, Nakajima Takashi, Onodera Osamu, Kakita Akiyoshi
Department of Neurology, Brain Disease Center, Agano Hospital, Agano, Japan.
Departments of Pathology, Brain Research Institute, Niigata University, Niigata, Japan.
Neuropathology. 2021 Dec;41(6):450-456. doi: 10.1111/neup.12777. Epub 2021 Nov 14.
Parkinson's disease (PD) is one of the most common neurodegenerative disorders. The cardinal neuropathological features of PD include selective and progressive loss of pigmented neurons in the substantia nigra, deficiencies in dopaminergic signaling in the striatum, and occurrence of phosphorylated α-synuclein-identified Lewy bodies in the nervous system. Parkinsonism, the clinical presentation of movement disorders seen in PD, is a feature shared commonly by other pathologically distinct neurodegenerative diseases, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA). Consequently, it is sometimes difficult to distinguish PD from such parkinsonism-related neurological disorders. In addition, parkinsonism is not always a feature of certain neurodegenerative diseases, and it can sometimes develop as a result of various forms of drug intoxication or cerebrovascular disease. Here, we describe the clinicopathological features of three patients (cases 1, 2, and 3) diagnosed as having PSP, MSA, and PD, respectively, in each of whom the postmortem histopathological diagnosis differed from the final clinical diagnosis. Neuropathologically, they had suffered from coexistent disorders: PD, MSA, and argyrophilic grain disease (case 1); PD (case 2); and vascular parkinsonism (case 3). The variety of patients showing features of parkinsonism underlines the importance of careful long-term follow up followed by postmortem neuropathological evaluation.
帕金森病（PD）是最常见的神经退行性疾病之一。PD的主要神经病理学特征包括黑质中色素神经元的选择性和进行性丧失、纹状体中多巴胺能信号传导缺陷以及神经系统中出现磷酸化α-突触核蛋白鉴定的路易小体。帕金森综合征是PD中出现的运动障碍临床表现，是其他病理上不同的神经退行性疾病（如进行性核上性麻痹（PSP）、皮质基底节变性（CBD）和多系统萎缩（MSA））共同具有的特征。因此，有时很难将PD与这类帕金森综合征相关的神经系统疾病区分开来。此外，帕金森综合征并不总是某些神经退行性疾病的特征，有时它可能是由各种形式的药物中毒或脑血管疾病引起的。在此，我们描述了三名分别被诊断为患有PSP、MSA和PD的患者（病例1、2和3）的临床病理特征，其中每例患者的死后组织病理学诊断均与最终临床诊断不同。神经病理学上，他们患有并存疾病：PD、MSA和嗜银颗粒病（病例1）；PD（病例2）；以及血管性帕金森综合征（病例3）。表现出帕金森综合征特征的患者的多样性凸显了进行仔细的长期随访并随后进行死后神经病理学评估的重要性。
