Background/ObjectiveThe corticobasal syndrome (CBS) is a complex asymmetric movement disorder, with cognitive impairment. Although commonly associated with the primary 4‐repeat‐tauopathy of corticobasal degeneration, clinicopathological correlation is poor, and a significant proportion is due to Alzheimer's disease (AD). Synaptic loss is a pathological feature of many clinical and preclinical tauopathies. We therefore measured the degree of synaptic loss in patients with CBS and tested whether synaptic loss differed according to β‐amyloid status.MethodsTwenty‐five people with CBS, and 32 age‐/sex‐/education‐matched healthy controls participated. Regional synaptic density was estimated by [11C]UCB‐J non‐displaceable binding potential (BPND), AD‐tau pathology by [18F]AV‐1451 BPND, and gray matter volume by T1‐weighted magnetic resonance imaging. Participants with CBS had β‐amyloid imaging with 11C‐labeled Pittsburgh Compound‐B ([11C]PiB) positron emission tomography. Symptom severity was assessed with the progressive supranuclear palsy‐rating‐scale, the cortical basal ganglia functional scale, and the revised Addenbrooke's Cognitive Examination. Regional differences in BPND and gray matter volume between groups were assessed by ANOVA.ResultsCompared to controls, patients with CBS had higher [18F]AV‐1451 uptake, gray matter volume loss, and reduced synaptic density. Synaptic loss was more severe and widespread in the β‐amyloid negative group. Asymmetry of synaptic loss was in line with the clinically most affected side.DiscussionDistinct patterns of [11C]UCB‐J and [18F]AV‐1451 binding and gray matter volume loss, indicate differences in the pathogenic mechanisms of CBS according to whether it is associated with the presence of Alzheimer's disease or not. This highlights the potential for different therapeutic strategies in CBSs. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

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β-淀粉样蛋白阳性与β-淀粉样蛋白阴性皮质基底综合征的突触丧失差异

背景/目的皮质基底综合征（CBS）是一种复杂的不对称运动障碍，伴有认知障碍。虽然通常与皮质基底节变性的原发性 4 重复 tau 蛋白病相关，但临床病理学相关性较差，其中很大一部分是由于阿尔茨海默病 (AD) 引起的。突触丧失是许多临床和临床前tau蛋白病的病理特征。因此，我们测量了 CBS 患者的突触丧失程度，并测试了突触丧失是否因 β-淀粉样蛋白状态而异。 方法 25 名 CBS 患者和 32 名年龄/性别/教育程度匹配的健康对照参与其中。区域突触密度估计为[11C]UCB-J不可置换结合电位（BPND), AD-tau 病理学 [18F]AV-1451 BPND，以及 T1 加权磁共振成像的灰质体积。患有 CBS 的参与者进行了 β-淀粉样蛋白成像11C标记的匹兹堡化合物-B（[11C]PiB）正电子发射断层扫描。症状严重程度通过进行性核上性麻痹评定量表、皮质基底节功能量表和修订版阿登布鲁克认知检查进行评估。血压的地区差异ND通过方差分析评估组间灰质体积。 结果与对照组相比，CBS 患者的 [18F]AV-1451 摄取、灰质体积损失和突触密度降低。 β-淀粉样蛋白阴性组的突触损失更为严重和广泛。突触损失的不对称性与临床上受影响最严重的一侧一致。讨论[的不同模式11C]UCB-J 和 [18F]AV-1451 结合和灰质体积损失表明 CBS 致病机制的差异（根据 CBS 是否与阿尔茨海默病相关）。这凸显了 CBS 中不同治疗策略的潜力。 © 2024 作者。运动障碍由 Wiley periodicals LLC 代表国际帕金森和运动障碍协会出版。