脑脊液α-突触核蛋白种子扩增检测在进行性核上性麻痹和皮质基底节综合征中的评估
Evaluation of Cerebrospinal Fluid α-Synuclein Seed Amplification Assay in Progressive Supranuclear Palsy and Corticobasal Syndrome.
作者信息
Vaughan David P, Fumi Riona, Theilmann Jensen Marte, Hodgson Megan, Georgiades Tatiana, Wu Lesley, Lux Danielle, Obrocki Ruth, Lamoureux Jennifer, Ansorge Olaf, Allinson Kieren S J, Warner Thomas T, Jaunmuktane Zane, Misbahuddin Anjum, Leigh P Nigel, Ghosh Boyd C P, Bhatia Kailash P, Church Alistair, Kobylecki Christopher, Hu Michele T M, Rowe James B, Blauwendraat Cornelis, Morris Huw R, Jabbari Edwin
机构信息
Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Movement Disorders Centre, UCL Queen Square Institute of Neurology, London, United Kingdom.
出版信息
Mov Disord. 2024 Dec;39(12):2285-2291. doi: 10.1002/mds.30019. Epub 2024 Sep 20.
BACKGROUND
Seed amplification assay (SAA) testing has been developed as a biomarker for the diagnosis of α-synuclein-related neurodegenerative disorders.
OBJECTIVE
The objective of this study was to assess the rate of α-synuclein SAA positivity in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) and to analyze clinical and pathological features of SAA-positive and -negative cases.
METHODS
A total of 96 cerebrospinal fluid samples from clinically diagnosed PSP (n = 59) and CBS (n = 37) cases were analyzed using α-synuclein SAA.
RESULTS
Six of 59 (10.2%) PSP cases were α-synuclein SAA positive, including one case who was MSA-type positive. An exploratory analysis showed that PSP cases who were Parkinson's disease-type positive were older and had a shorter disease duration compared with SAA-negative cases. In contrast, 11 of 37 (29.7%) CBS cases were α-synuclein SAA positive, including two cases who were MSA-type positive.
CONCLUSIONS
Our results suggest that α-synuclein seeds can be detected in PSP and CBS using a cerebrospinal fluid α-synuclein SAA, and in PSP this may impact on clinical course.
背景
种子扩增分析（SAA）检测已被开发作为诊断α-突触核蛋白相关神经退行性疾病的生物标志物。
目的
本研究的目的是评估进行性核上性麻痹（PSP）和皮质基底节综合征（CBS）中α-突触核蛋白SAA阳性率，并分析SAA阳性和阴性病例的临床及病理特征。
方法
使用α-突触核蛋白SAA对96份来自临床诊断为PSP（n = 59）和CBS（n = 37）病例的脑脊液样本进行分析。
结果
59例PSP病例中有6例（10.2%）α-突触核蛋白SAA呈阳性，其中1例为多系统萎缩（MSA）型阳性。一项探索性分析显示，与SAA阴性病例相比，帕金森病型阳性的PSP病例年龄更大，病程更短。相比之下，37例CBS病例中有11例（29.7%）α-突触核蛋白SAA呈阳性，其中2例为MSA型阳性。
结论
我们的结果表明，使用脑脊液α-突触核蛋白SAA可在PSP和CBS中检测到α-突触核蛋白种子，且在PSP中这可能会影响临床病程。
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