Cahan Joshua G, Bonakdarpour Borna
Mesulam Institute for Cognitive Neurology and Alzheimer's Disease, Feinberg School of Medicine, Northwestern University, Chicago, IL USA.
Ken and Ruth Davee Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL USA.
Curr Treat Options Neurol. 2025;27(1):39. doi: 10.1007/s11940-025-00848-4. Epub 2025 Sep 20.
Primary progressive aphasia(PPA) is a rare neurodegenerative condition and variant presentation of Alzheimer's disease or Frontotemporal Dementia. It is characterized by progressive decline isolated to language functions. PPA provides a model for understanding the anatomy of language, where each cortical language center corresponds to distinct PPA subtypes. Understanding this anatomy and its corresponding PPA subtypes helps clinicians choose testing, interpret imaging, and tailor treatment. These subtypes are termed agrammatic/nonfluent, semantic, and logopenic PPA. Each subtype is probabilistically associated with three proteinopathies: the amyloid and tau of Alzheimer's disease and frontotemporal lobar degeneration due to Tau or TDP-43. We will discuss when biomarker testing is indicated and the nuances of choosing among the increasing array of biomarker tests to improve diagnostic certainty. While medical treatment is limited, there are increasing pharmacologic options for treating Alzheimer's disease. Non-pharmacologic strategies can also be tailored to the patient's specific subtype and caregivers' needs.
原发性进行性失语(PPA)是一种罕见的神经退行性疾病,是阿尔茨海默病或额颞叶痴呆的变异表现形式。其特征是仅语言功能进行性衰退。PPA为理解语言解剖结构提供了一个模型,其中每个皮质语言中枢对应不同的PPA亚型。了解这种解剖结构及其相应的PPA亚型有助于临床医生选择检测方法、解读影像学检查结果并制定个性化治疗方案。这些亚型被称为语法缺失/非流利型、语义型和语音性PPA。每种亚型都与三种蛋白病存在概率关联:阿尔茨海默病的淀粉样蛋白和tau蛋白,以及由Tau或TDP-43引起的额颞叶变性。我们将讨论何时需要进行生物标志物检测,以及在越来越多的生物标志物检测方法中进行选择以提高诊断确定性的细微差别。虽然药物治疗有限,但治疗阿尔茨海默病的药物选择越来越多。非药物策略也可以根据患者的特定亚型和护理人员的需求进行调整。