Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Veterans Affairs Pacific Islands Health Care System, Honolulu, HI, USA; Departments of Medicine and John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA; The John A Hartford Foundation Center of Excellence in Geriatrics, Department of Geriatric Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, USA.
Parkinsonism Relat Disord. 2019 Jul;64:124-131. doi: 10.1016/j.parkreldis.2019.03.023. Epub 2019 Mar 28.
Braak et al.'s 2003 paper detailing the caudo-rostral progression of Lewy body pathology (LP) formed the foundation of current understanding of disease spread in Parkinson's disease (PD); however, its methods are difficult to recreate and consequently multiple new staging systems emerged to recapitulate Braak's staging system using standard neuropathological methods and to account for other patterns of LP. Studies using these systems have documented widely variable rates of cases that 'fail to fit' expected patterns of LP spread. This could be due to population differences, features of individual systems, or may constitute under-recognized patterns of disease. We examined 324 neuropathological cases from the Honolulu Asia Aging Study and applied four different LP staging systems to determine the proportion of cases adhering to different staging methodologies and those that 'fail to fit' expected patterns of LP. Of 141 cases with LP (24: PD, 8: Dementia with Lewy bodies (DLB), 109: Incidental Lewy body disease (ILBD)), our application of Braak et al., 2003 classified 83.7%, Müller et al., 2005 classified 87.9%, Beach et al., 2009 classified 100%, and Leverenz et al., 2008 classified 98.6%. There were significant differences in the cases classifiable by the Leverenz and Beach systems versus the Braak and Müller systems (p < 0.001 for each). In this population-based autopsy cohort with a high prevalence of ILBD, the majority of cases were consistent with the progression characterized by the Braak et al. however, the determination of cases as atypical is highly dependent on the staging system applied.
布拉克等人 2003 年的论文详细描述了路易体病理学（LP）的头尾进展，为当前对帕金森病（PD）疾病传播的理解奠定了基础；然而，其方法难以重现，因此出现了多个新的分期系统，以使用标准神经病理学方法重现布拉克的分期系统，并解释 LP 的其他模式。使用这些系统的研究记录了广泛变化的病例未能符合 LP 传播预期模式的比率。这可能是由于人群差异、个别系统的特征，或者可能构成未被认识到的疾病模式。我们检查了来自檀香山亚洲老龄化研究的 324 例神经病理学病例，并应用了四种不同的 LP 分期系统，以确定符合不同分期方法的病例比例和那些未能符合 LP 预期模式的病例比例。在 141 例具有 LP 的病例中（24 例 PD、8 例路易体痴呆（DLB）、109 例意外路易体病（ILBD）），我们应用布拉克等人的方法，2003 年分类 83.7%、穆勒等人的 2005 年分类 87.9%、比奇等人的 2009 年分类 100%、莱文泽等人的 2008 年分类 98.6%。莱文泽和比奇系统与布拉克和穆勒系统可分类的病例存在显著差异（p 值均<0.001）。在这个基于人群的尸检队列中，ILBD 的患病率很高，大多数病例与布拉克等人描述的进展一致，然而，病例被判定为非典型的情况高度依赖于应用的分期系统。
