Epelbaum Stéphane, Saade Yasmina Michel, Flamand Roze Constance, Roze Emmanuel, Ferrieux Sophie, Arbizu Céline, Nogues Marie, Azuar Carole, Dubois Bruno, Tezenas du Montcel Sophie, Teichmann Marc
Department of Neurology, National Reference Center for "PPA and rare dementias", Institute for Memory and Alzheimer's Disease, Pitié Salpêtrière Hospital, AP-HP, Paris, France.
Institut du Cerveau, ICM, INSERM U 1127, CNRS UMR 7225, Sorbonne Université, Paris, France.
Front Neurol. 2021 Jan 5;11:571657. doi: 10.3389/fneur.2020.571657. eCollection 2020.
Primary progressive aphasias (PPA) have been investigated by clinical, therapeutic, and fundamental research but examiner-consistent language tests for reliable reproducible diagnosis and follow-up are lacking. We developed and evaluated a rapid language test for PPA ("PARIS") assessing its inter-examiner consistency, its power to detect and classify PPA, and its capacity to identify language decline after a follow-up of 9 months. To explore the reliability and specificity/sensitivity of the test it was applied to PPA patients ( = 36), typical amnesic Alzheimer's disease (AD) patients ( = 24) and healthy controls ( = 35), while comparing it to two rapid examiner-consistent language tests used in stroke-induced aphasia ("LAST", "ART"). The application duration of the "PARIS" was ~10 min and its inter-rater consistency was of 88%. The three tests distinguished healthy controls from AD and PPA patients but only the "PARIS" reliably separated PPA from AD and allowed for classifying the two most frequent PPA variants: semantic and logopenic PPA. Compared to the "LAST" and "ART," the "PARIS" also had the highest sensitivity for detecting language decline. The "PARIS" is an efficient, rapid, and highly examiner-consistent language test for the diagnosis, classification, and follow-up of frequent PPA variants. It might also be a valuable tool for providing end-points in future therapeutic trials on PPA and other neurodegenerative diseases affecting language processing.
原发性进行性失语症（PPA）已通过临床、治疗和基础研究进行了调查，但缺乏用于可靠重复诊断和随访的检查者一致的语言测试。我们开发并评估了一种针对PPA的快速语言测试（“PARIS”），评估其检查者间的一致性、检测和分类PPA的能力，以及在9个月随访后识别语言衰退的能力。为了探索该测试的可靠性和特异性/敏感性，将其应用于PPA患者（n = 36）、典型遗忘型阿尔茨海默病（AD）患者（n = 24）和健康对照者（n = 35），同时将其与用于中风性失语症的两种快速检查者一致的语言测试（“LAST”、“ART”）进行比较。“PARIS”的应用时长约为10分钟，其评分者间一致性为88%。这三种测试将健康对照者与AD和PPA患者区分开来，但只有“PARIS”能可靠地将PPA与AD区分开，并能够对两种最常见的PPA变体进行分类：语义性和语音性PPA。与“LAST”和“ART”相比，“PARIS”在检测语言衰退方面也具有最高的敏感性。“PARIS”是一种高效、快速且检查者高度一致的语言测试，可用于常见PPA变体的诊断、分类和随访。它也可能是在未来针对PPA和其他影响语言处理的神经退行性疾病的治疗试验中提供终点指标的有价值工具。
