The clinical diagnosis in patients with parkinsonian disorders can be challenging, and a definite diagnosis requires neuropathological confirmation. The aim of this study was to examine whether a clinical diagnosis of Parkinson's disease (PD) and atypical parkinsonian disorders predict the presence of Lewy pathology (LP) and concomitant neuropathological lesions.We included 293 donors with a history of parkinsonism without dementia at disease onset, collected by the Netherlands Brain Bank (NBB) from 1989 to 2015. We retrospectively categorized donors according the International Parkinson and Movement Disorder Society clinical diagnostic criteria for PD (MDS-PD criteria) as 'not PD', 'probable PD' or 'established PD'. We compared the final clinical diagnosis to presence of neuropathological lesions as defined by BrainNet Europe and National Institute on Aging - Alzheimer's Association guidelines.LP was present in 150 out of 176 donors (85%) with a clinical diagnosis of PD, in 8 out of 101 donors (8%) with atypical parkinsonian disorders and in 4 out of 16 donors (25%) without a definite clinical diagnosis. Independent from age at death, stages of amyloid-β, but not neurofibrillary tau or neuritic plaques, were higher in donors with LP compared to other types of pathology (p = 0.009). The MDS-PD criteria at a certainty level of 'probable PD' predicted presence of LP with a diagnostic accuracy of 89.3%. Among donors with LP, 'established PD' donors showed similar Braak α-synuclein stages and stages of amyloid-β, neurofibrillary tau and neuritic plaques compared to 'not PD' or 'probable PD' donors.In conclusion, both a clinical diagnosis of PD as well as MDS-PD criteria accurately predicted presence of LP in NBB donors. LP was associated with more widespread amyloid-β pathology, suggesting a link between amyloid-β accumulation and LP formation.

中文翻译：

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荷兰大型尸检系列中帕金森氏病的神经病理学相关性。

帕金森病患者的临床诊断可能具有挑战性，明确的诊断需要神经病理学确认。这项研究的目的是检查帕金森氏病（PD）和非典型帕金森病的临床诊断是否预示了路易氏病（LP）和伴随的神经病理性病变的存在。我们纳入了293名有帕金森病病史且无痴呆的供体，由1989年至2015年由荷兰脑库（NBB）收集。我们根据国际帕金森氏症和运动障碍学会针对PD的临床诊断标准（MDS-PD标准）对捐赠者进行了回顾性分类，分为“非PD”，“可能的PD”或“建立PD'。我们将最终临床诊断与神经病病变的存在进行了比较，这是由BrainNet Europe和美国衰老协会（Alzheimer's Association）定义的。在176名有PD临床诊断的捐献者中，有150名（85％）存在LP有非典型帕金森病的101位捐献者（8％）和16位捐献者中有4位（25％）没有明确的临床诊断。与死亡年龄无关，与其他病理类型相比，LP献血者的淀粉样蛋白-β分期（而非神经原纤维tau或神经斑）的阶段性更高（p = 0.009）。在“可能的PD”的确定性水平上的MDS-PD标准可预测LP的存在，诊断准确性为89.3％。在患有LP的供体中，“既定的PD”供体表现出相似的Braakα-突触核蛋白阶段和淀粉样β阶段，与“非PD”或“可能的PD”供体相比，神经原纤维tau和神经斑块。总之，PD的临床诊断以及MDS-PD标准都能准确预测NBB供体中LP的存在。LP与更广泛的淀粉样β病理学相关联，表明淀粉样β积累与LP形成之间的联系。