An interdisciplinary approach aiding the diagnosis of primary progressive aphasia: A case report

ABSTRACT Frontotemporal dementia (FTD) is one of the most common causes of early-onset dementia with primary progressive aphasia (PPA) being the second-most-frequent form of this degenerative disease. Despite the similarity with progressive dementia (especially in early stages of Alzheimer´s disease), three types of PPA can be differentiated: semantic, agrammatic and logopenic (subtype discussed in this study). To date, no medications have been shown to improve or stabilize cognitive deficits in patients with PPA. We report the case of a 62-year-old woman with difficulty naming objects and planning. An interdisciplinary evaluation, including imaging and lab exams, together with neuropsychological and personality assessments, confirmed that the patient had logopenic PPA on the basis of repetition difficulty, phonemic and semantic paraphasias and absence of agrammatism. The timing of the assessment in this case, along with the resources available and commitment of an integrated interdisciplinary team, allowed a differential diagnosis (from other classical dementias) to be reached.

摘要 额颞叶痴呆（Frontotemporal dementia, FTD）是早发性痴呆最常见的病因之一，原发性进行性失语（primary progressive aphasia, PPA）是该退行性疾病的第二常见亚型。尽管其与进展性痴呆（尤其阿尔茨海默病（Alzheimer´s disease）早期阶段）存在相似临床表现，但PPA可被划分为三种类型：语义型、语法缺失型与迟语型（为本研究探讨的亚型）。截至目前，尚无药物被证实可改善或稳定PPA患者的认知缺损症状。本文报告1例62岁女性患者，其表现为物体命名困难与计划能力受损。多学科评估涵盖影像学检查、实验室检验，结合神经心理学与人格评估，基于患者的复述障碍、语音错语与语义错语表现，以及无语法缺失的特征，确诊为迟语型PPA。本案例的评估时机、可利用的研究资源以及整合多学科团队的协作投入，使得该病例得以与其他经典痴呆疾病完成鉴别诊断。