From the Department of Neurology, University of Texas Southwestern, Dallas, Texas, USA.
Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, New York, USA.
J Neuropathol Exp Neurol. 2022 Sep 19;81(10):796-806. doi: 10.1093/jnen/nlac068.
The Essential Tremor Centralized Brain Repository is the largest repository of prospectively collected essential tremor (ET) brains (n = 231). Hence, we are uniquely poised to address several questions: What proportion of ET cases has Lewy pathology (LP)? What is the nature of that pathology and how does it relate to other comorbidities? Each brain had a complete neuropathological assessment, including α-synuclein immunostaining. We created a 10-category classification scheme to fully encapsulate the patterns of LP observed. Four metrics of cerebellar pathology were also quantified. Mean age at death = 89.0 ± 6.4 years. Fifty-eight (25.1%) had LP and 46 (19.9%) had early to late stages of Parkinson disease (PD). LP was very heterogeneous. Of 58 cases with LP, 14 (24.1%) clinically developed possible PD or PD after a latency of 5 or more years. There was a similar degree of cerebellar pathology in ET cases both with and without LP. In summary, 1 in 4 ET cases had LP-a proportion that seems higher than expected based on studies among control populations. Heterogeneous LP likely reflects clinical associations between ET and PD, and ET with Alzheimer disease-type neuropathology. These data further our understanding of ET and its relatedness to other degenerative diseases.
特发性震颤中枢知识库是最大的前瞻性特发性震颤 (ET) 脑库 (n = 231)。因此，我们有独特的优势来解决几个问题：有多少 ET 病例有路易体病理 (LP)？这种病理学的性质是什么，它与其他合并症有何关系？每个大脑都进行了完整的神经病理学评估，包括α-突触核蛋白免疫染色。我们创建了一个 10 类分类方案，以充分包含观察到的 LP 模式。还量化了四项小脑病理学指标。平均死亡年龄为 89.0 ± 6.4 岁。58 例（25.1%）有 LP，46 例（19.9%）有早期到晚期帕金森病 (PD)。LP 非常多样化。在 58 例 LP 病例中，14 例（24.1%）在 5 年或更长时间的潜伏期后出现了可能的 PD 或 PD。无论是否存在 LP，ET 病例的小脑病理学程度相似。总之，每 4 例 ET 病例中就有 1 例存在 LP，这一比例似乎高于基于对照人群研究的预期。LP 的异质性可能反映了 ET 与 PD 之间的临床关联，以及 ET 与阿尔茨海默病型神经病理学之间的关联。这些数据进一步加深了我们对 ET 及其与其他退行性疾病的关系的理解。
