Neurology Unit, University of Cambridge, UK.
Neurology. 2012 May 22;78(21):1670-7. doi: 10.1212/WNL.0b013e3182574f79. Epub 2012 May 9.
Primary progressive aphasia (PPA) has been proposed to comprise 3 discrete clinical subtypes: semantic, agrammatic/nonfluent, and logopenic. Recent consensus recommendations suggest a diagnostic framework based primarily on clinical and neuropsychological findings to classify these variants. Our objective was to evaluate the extent to which patients with PPA would conform to the proposed tripartite system and whether the clustering pattern of elements of the linguistic profile suggests discrete clinical syndromes.
A total of 46 patients with PPA were prospectively recruited to the Cambridge Longitudinal Study of PPA. Sufficient data were collected to assess all consensus-proposed diagnostic domains. By comparing patients' performances against those of 30 age- and education-matched healthy volunteers, z scores were calculated, and values of 1.5 SDs outside control participants' means were considered abnormal. Raw test scores were used to undertake a principal factor analysis to identify the clustering pattern of individual measures.
Of the patients, 28.3%, 26.1%, and 4.3% fitted semantic, nonfluent/agrammatic, and logopenic categories respectively, and 41.3% did not fulfill the diagnostic recommendations for any of the 3 proposed variants. There was no significant between-group difference in age, education, or disease duration. Furthermore, the outcome of the factor analysis was in keeping with discrete semantic and nonfluent/agrammatic syndromes but did not support a logopenic variant.
Taken together, the results of this prospective data-driven study suggest that although a substantial proportion of patients with PPA have neither the semantic nor the nonfluent variants, they do not necessarily conform to a discrete logopenic variant.
原发性进行性失语症（PPA）已被提出包含 3 种离散的临床亚型：语义性、语法性/非流利性和完全性失语症。最近的共识建议提出了一种主要基于临床和神经心理学发现的诊断框架，用于对这些变体进行分类。我们的目的是评估 PPA 患者在多大程度上符合所提出的三分系统，以及语言特征的聚类模式是否提示离散的临床综合征。
共前瞻性招募了 46 名 PPA 患者参加剑桥进行性失语症纵向研究。收集了足够的数据来评估所有共识建议的诊断领域。通过将患者的表现与 30 名年龄和教育程度匹配的健康志愿者进行比较，计算了 z 分数，并且将超过对照组平均值 1.5 个标准差的值视为异常。使用原始测试分数进行主因子分析，以确定个体测量的聚类模式。
在患者中，分别有 28.3%、26.1%和 4.3%符合语义性、非流利/语法性和完全性失语症类别，而 41.3%的患者不符合 3 种建议变体中的任何一种诊断标准。组间在年龄、教育程度或疾病持续时间方面无显著差异。此外，因子分析的结果与离散的语义性和非流利/语法性综合征一致，但不支持离散的完全性失语症变体。
综上所述，这项前瞻性数据驱动研究的结果表明，尽管相当一部分 PPA 患者既没有语义性变体，也没有非流利性变体，但他们不一定符合离散的完全性失语症变体。
