Alafuzoff Irina, Hartikainen Päivi
Department of Immunology, Genetics and Pathology, Uppsala University, Department of Pathology, Uppsala University Hospital and Rudbeck Laboratory, Uppsala, Sweden.
Department of Neurology, Institute of Clinical Medicine, University of Eastern Finland and Department of Neurology, Kuopio University Hospital, Kuopio, Finland.
Handb Clin Neurol. 2017;145:339-353. doi: 10.1016/B978-0-12-802395-2.00024-9.
A neurodegenerative disorder displaying an altered α-synuclein (αS) in the brain tissue is called α-synucleinopathy (αS-pathy) and incorporates clinical entities such as Parkinson disease (PD), PD with dementia, dementia with Lewy bodies, and multiple-system atrophy. Neuroradiologic techniques visualizing αS pathology in the brain or assays of αS in the cerebrospinal fluid or blood are probably available and will be implemented in the near future but currently the definite diagnosis of αS-pathy relies on a postmortem examination of the brain. Since the 1980s immunohistochemical technique based on the use of antibodies directed to proteins of interest has become a method of choice for neuropathologic diagnosis. Furthermore, since the 1990s it has been acknowledged that progressions of most neurodegenerative pathologies follow a certain predictable time-related neuroanatomic distribution. Currently, for Lewy body disease, two staging techniques are commonly used: McKeith and Braak staging. Thus, the neuropathologic diagnosis of a αS-pathy is based on detection of altered αS in the tissue and registration of the neuroanatomic distribution of this alteration in the brain. The clinicopathologic correlation is not absolute due to the quite frequent observation of incidental and concomitant αS pathology.
一种在脑组织中显示α-突触核蛋白（αS）改变的神经退行性疾病被称为α-突触核蛋白病（αS病），它包括帕金森病（PD）、伴有痴呆的PD、路易体痴呆和多系统萎缩等临床实体。可视化大脑中αS病理的神经放射学技术或脑脊液或血液中αS的检测方法可能已经存在，并将在不久的将来得到应用，但目前αS病的明确诊断依赖于对大脑的尸检。自20世纪80年代以来，基于使用针对感兴趣蛋白质的抗体的免疫组织化学技术已成为神经病理学诊断的首选方法。此外，自20世纪90年代以来，人们已经认识到大多数神经退行性疾病的进展遵循一定的可预测的与时间相关的神经解剖分布。目前，对于路易体病，常用两种分期技术：麦基思分期和布拉克分期。因此，αS病的神经病理学诊断基于组织中改变的αS的检测以及大脑中这种改变的神经解剖分布的记录。由于偶然和伴随的αS病理现象相当常见，临床病理相关性并非绝对。
