Association of PSP phenotypes with survival: A brain-bank study
Association of PSP phenotypes with survival: A brain-bank study
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DOI：
10.1016/j.parkreldis.2021.01.015
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发表时间：
2021-02-10
影响因子：
4.1
通讯作者：
Compta, Yaroslau
中科院分区：
文献类型：
--
作者：
Guasp, Mar;Molina-Porcel, Laura;Compta, Yaroslau
简介：MDS-PSP标准通过在Richardson综合征（PSP-RS）中添加其他表现（如PSP-帕金森综合征（PSP-P）、PSP-单纯步态冻结（PSP-PGF）、PSP-言语（PSP-SL）、PSP-额叶（PSP-F）、PSP-姿势不稳定（PSP-PI）和PSP-皮质基底综合征（PSPCBS））来扩展PSP的表型谱。PSP表型之间预后差异的证据很少，主要集中在PSP-RS与非PSP-RS。使用脑库队列，我们不仅在PSP-RS与非PSP-RS中评估PSP存活率，而且在PSP-RS +皮质与皮质下表型中评估PSP存活率。方法：回顾性分析32例PSP患者和30例其他退行性帕金森综合征（帕金森病[PD; n = 11]，多系统萎缩[MSA; n = 11]，皮质基底节变性[CBD; n = 8]）的MDS-PSP诊断标准。我们在神经病理学证实的PSP病例中进行了生存统计，考虑了PSP-RS与非PSP-RS和PSP-RS + PSP-皮质（PSP-F + PSP-SL + PSP-CBS）与PSP-皮质下（PSP-P + PSP-PGF）表型。我们还调整生存分析PSP tau scores.Results：诊断的敏感性为100%，特异性范围为47%至87%，排除的情况下，符合“提示PSP”的定义，在他们的病程早期，但与其他临床特征更好地匹配与非PSP病理诊断。生存期显着较短的PSP-RS与非PSP-RS的情况下，但它是更显着较短的PSP-RS + PSP-皮质与PSP-皮质下，独立的PSP tau评分，这是不相关的survival.Conclusions：PSP-皮质下表型似乎有较长的生存期比PSP-RS和皮质表型。当告知患者临床诊断时，这可能具有预后相关性。
Introduction: The MDS-PSP criteria expand the phenotypic spectrum of PSP by adding to Richardson's syndrome (PSP-RS) other presentations such as PSP-parkinsonism (PSP-P), PSP-pure-gait-freezing (PSP-PGF), PSP-speechlanguage (PSP-SL), PSP-frontal (PSP-F), PSP-postural-instability (PSP-PI) and PSP-corticobasal-syndrome (PSPCBS). Evidence about the prognostic differences between PSP phenotypes is scarce and focused on PSP-RS vs. non-PSP-RS. Using a brain-bank cohort we assessed PSP survival not only in PSP-RS vs. non-PSP-RS, but also in PSP-RS + cortical vs. subcortical phenotypes. Besides, we assessed sensitivity and specificity of the MDS-PSP criteria in of PSP and other degenerative parkinsonisms.Methods: We retrospectively applied the MDS-PSP diagnostic criteria to 32 definite PSP cases and 30 cases with other degenerative parkinsonian syndromes (Parkinson's disease [PD; n = 11], multiple system atrophy [MSA; n = 11], corticobasal degeneration [CBD; n = 8]). We conducted survival statistics in neuropathologically confirmed PSP cases considering PSP-RS vs. non-PSP-RS and PSP-RS + PSP-cortical (PSP-F + PSP-SL + PSP-CBS) vs. PSP-subcortical (PSP-P + PSP-PGF) phenotypes. We also adjusted survival analyses for PSP tau scores.Results: Diagnostic sensitivity was 100% and specificity ranged from 47% to 87% when excluding cases that met the "suggestive of PSP" definition early in their disease course but with other clinical features better matching with a non-PSP pathological diagnosis. Survival was significantly shorter in PSP-RS vs. non-PSP-RS cases, but it was more markedly shorter in PSP-RS + PSP-cortical vs. PSP-subcortical, independently of PSP tau scores, which were not associated with survival.Conclusions: PSP-subcortical phenotypes appear to have longer survival than PSP-RS and cortical phenotypes. This might be of prognostic relevance when informing patients upon clinical diagnosis.