From the Department of Neurology (K.R., M.D.S., A.W., E.V., A.P., H.K., A.E.H.), Johns Hopkins School of Medicine, Baltimore, MD; Department of Cognitive Science (J.S., A.E.H.), Johns Hopkins University, Baltimore, MD; Department of Communication Disorders (S.S.), Chapman University, Orange, CA; Department of Radiology (A.V.F.), Johns Hopkins School of Medicine; and Department of Physical Medicine and Rehabilitation (A.E.H.), Johns Hopkins University School of Medicine.
Neurology. 2022 Nov 1;99(18):e2044-e2051. doi: 10.1212/WNL.0000000000201040. Epub 2022 Aug 17.
It is widely agreed that primary progressive aphasia (PPA) is a clinical syndrome with at least 3 distinct variants that differ in phenotype, areas of atrophy, and most common underlying neurodegenerative disease. The distinction between logopenic variant PPA (lvPPA) and other variants is important for prognosis and medical management. However, differentiating logopenic from nonfluent agrammatic variant can be difficult. We aimed to identify a novel behavioral assessment that (1) distinguishes logopenic from the other variants with a high degree of accuracy and (2) correlates with left superior temporal-inferior parietal atrophy (previously shown to be associated with this variant). The location of atrophy was measured using a novel, clinically useful imaging analysis.
In this observational cohort study of 227 individuals with PPA, participants were administered sentence reading and repetition subtests from a standard battery. A subset of 41 participants were administered enhanced reading and repetition subtests with 5 longer sentences, of which 13 had brain imaging. Ratios of word-level and sentence-level accuracy in reading:repetition were calculated. We used one-way analysis of variance (ANOVA) to determine whether either or both ratios of reading:repetition independently discriminated between variants and test to determine whether the ratios distinguished between nonfluent and logopenic variants. We identified receiver operating characteristics and Pearson correlations between the reading:repetition ratios and ratio of left:right superior temporal-inferior parietal volume.
The sentence reading:repetition ratio using the new stimuli significantly differed across the 3 variants ( < 0.00001) and differed between nonfluent and logopenic variants (t(27) = 4.1; = 0.0003). The area under the curve for distinguishing logopenic from other variants was 0.85 (0.71-0.99), and the diagnostic accuracy was 87.5%. The sentence reading:repetition ratio correlated with left:right superior temporal-inferior parietal volume ( = 0.69; = 0.0087), but not with left:right volume of regions of interest associated with other variants.
Results provide an efficient and reliable clinical assessment, and a novel imaging analysis, to distinguish the clinical syndrome of logopenic variant from other variants of PPA. Results also support the proposal that lvPPA reflects a defect in phonological short-term memory caused by atrophy in the superior temporal-inferior parietal cortex.
This study provides Class III evidence that the sentence reading:repetition ratio distinguished logopenic PPA from other PPA variants.
人们普遍认为原发性进行性失语症（PPA）是一种临床综合征，至少有 3 种不同的变体，其表型、萎缩区域和最常见的潜在神经退行性疾病不同。鉴别流利型非语法性变体 PPA（lvPPA）和其他变体对于预后和医疗管理很重要。然而，区分失语法性和非流利性失语法性变体可能很困难。我们旨在确定一种新的行为评估方法，（1）以高度准确性区分失语法性变体和其他变体，（2）与左颞顶-下顶叶萎缩相关（先前表明与该变体相关）。使用一种新的、临床有用的成像分析来测量萎缩的位置。
在这项对 227 名 PPA 患者的观察性队列研究中，参与者接受了标准电池的句子阅读和重复测试。41 名参与者中的一部分接受了增强的阅读和重复测试，使用了 5 个更长的句子，其中 13 个有大脑成像。计算阅读：重复的单词和句子准确性比值。我们使用单因素方差分析（ANOVA）来确定阅读：重复的比值是否可以独立区分变体， 检验来确定这些比值是否可以区分非流利性和失语法性变体。我们确定了阅读：重复比值与左/右颞顶-下顶叶体积比值之间的受试者工作特征曲线和 Pearson 相关性。
使用新刺激的句子阅读：重复比在 3 种变体之间有显著差异（ < 0.00001），并且在非流利性和失语法性变体之间也有差异（t（27）= 4.1； = 0.0003）。区分失语法性变体的曲线下面积为 0.85（0.71-0.99），诊断准确性为 87.5%。句子阅读：重复比与左/右颞顶-下顶叶体积相关（ = 0.69； = 0.0087），但与其他变体相关的左/右区域的体积无相关性。
结果提供了一种高效可靠的临床评估方法和一种新的成像分析方法，可区分失语法性变体 PPA 与 PPA 的其他变体。结果还支持 lvPPA 反映了由颞顶-下顶叶皮层萎缩引起的语音短期记忆缺陷的提议。
本研究提供了 III 级证据，表明句子阅读：重复比可区分失语法性 PPA 与其他 PPA 变体。
