INTRODUCTIONLewy body disease (LBD) is a common primary or co‐pathology in neurodegenerative syndromes. An alpha‐synuclein seed amplification assay (αSyn‐SAA) is clinically available, but clinical performance, especially lower sensitivity in amygdala‐predominant cases, is not well understood.METHODSAntemortem CSF from neuropathology‐confirmed LBD cases was tested with αSyn‐SAA (N = 56). Diagnostic performance and clinicopathological correlations were examined.RESULTSSimilar to prior reports, sensitivity was 100% for diffuse and transitional LBD (9/9), and overall specificity was 96.3% (26/27). Sensitivity was lower in amygdala‐predominant (6/14, 42.8%) and brainstem‐predominant LBD (1/6, 16.7%), but early spread outside these regions (without meeting criteria for higher stage) was more common in αSyn‐SAA‐positive cases (6/7, 85.7%) than negative (2/13, 15.4%).DISCUSSIONIn this behavioral neurology cohort, αSyn‐SAA had excellent diagnostic performance for cortical LBD. In amygdala‐ and brainstem‐predominant cases, sensitivity was lower, but positivity was associated with anatomical spread, suggesting αSyn‐SAA detects early LBD progression in these cohorts.Highlights A cerebrospinal fluid alpha‐synuclein assay detects cortical LBD with high sensitivity/specificity. Positivity in prodromal stages of LBD was associated with early cortical spread. The assay provides precision diagnosis of LBD that could support clinical trials. The assay can also identify LBD co‐pathology, which may impact treatment responses.

中文翻译：

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行为神经病学尸检队列中脑脊液α-突触核蛋白种子扩增测定的临床病理学相关性

简介路易体病（LBD）是神经退行性综合征中常见的原发性或并发病理。 α-突触核蛋白种子扩增测定 (αSyn-SAA) 在临床上可用，但临床表现，尤其是杏仁核为主病例的较低敏感性，尚不清楚。氮= 56）。检查诊断性能和临床病理学相关性。 结果与之前的报告类似，弥漫性和移行性 LBD 的敏感性为 100% (9/9)，总体特异性为 96.3% (26/27)。杏仁核为主的 LBD（6/14，42.8%）和脑干为主的 LBD（1/6，16.7%）的敏感性较低，但早期扩散到这些区域之外（不符合更高阶段的标准）在 αSyn-SAA 中更为常见‐阳性病例 (6/7, 85.7%) 多于阴性病例 (2/13, 15.4%)。讨论 在这个行为神经学队列中，αSyn-SAA 对皮质 LBD 具有出色的诊断性能。在杏仁核和脑干为主的病例中，敏感性较低，但阳性与解剖学扩散相关，表明 αSyn-SAA 可以检测到这些队列中的早期 LBD 进展。 脑脊液 α-突触核蛋白检测以高灵敏度/特异性检测皮质 LBD。 LBD 前驱阶段的阳性与早期皮质扩散有关。 该检测提供了 LBD 的精确诊断，可以支持临床试验。 该检测还可以识别 LBD 共同病理，这可能会影响治疗反应。