Incidence of primary progressive aphasia in Salento, Italy: a population-based study.

IF 7.6 1区医学 Q1 CLINICAL NEUROLOGY

Alzheimer's Research & Therapy Pub Date : 2026-05-01 DOI:10.1186/s13195-026-02046-z

Daniele Urso, Alessandra Vitulli, Stefano Giannoni-Luza, Valentina Gnoni, Alessia Giugno, Eleonora Rollo, Davide Vilella, Chiara Zecca, Nicolas Ray, Giancarlo Logroscino

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Abstract

Introduction: Data on the population-based incidence of primary progressive aphasia (PPA), including all variants and Alzheimer's disease (AD) biomarker profiles, are scarce.

Methods: We conducted a 4-year, prospective, population-based study in the Salento region of Southern Italy, identifying incident PPA cases through a territory-wide surveillance network. Clinical diagnoses followed consensus criteria; AD pathology was defined by positive CSF or amyloid PET biomarkers.

Results: We identified 35 incident PPA cases, yielding an overall incidence of 1.14 (95% CI, 0.79-1.59) per 100,000 person-years. Incidence peaked in the early 60s for men and in the late 70s for women. The logopenic variant was most frequent and invariably associated with AD pathology. Approximately 20% of nonfluent and semantic cases, and the majority unclassified cases, also showed AD biomarkers.

Discussion: These findings provide real-world epidemiological evidence and underscore the need for systematic biomarker assessment in PPA to guide diagnosis, prognosis, and access to emerging therapies.

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意大利萨伦托原发性进行性失语症的发病率：一项基于人群的研究。

基于人群的原发性进行性失语（PPA）发病率的数据，包括所有变体和阿尔茨海默病（AD）生物标志物谱，很少。方法：我们在意大利南部萨伦托地区进行了一项为期4年的前瞻性、基于人群的研究，通过全区监测网络确定PPA事件病例。临床诊断遵循共识标准；AD病理由CSF或淀粉样PET生物标志物阳性来定义。结果：我们确定了35例PPA病例，总发病率为每10万人年1.14例（95% CI, 0.79-1.59）。男性发病率在60年代初达到顶峰，女性在70年代末达到顶峰。这种基因缺失变异是最常见的，并且总是与AD病理相关。大约20%的不流利和语义的病例，以及大多数未分类的病例，也显示AD生物标志物。讨论：这些发现提供了现实世界的流行病学证据，并强调了在PPA中进行系统的生物标志物评估以指导诊断、预后和获得新兴疗法的必要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Alzheimer's Research & Therapy 医学-神经病学

CiteScore

13.10

自引率

3.30%

发文量

172

审稿时长

>12 weeks

期刊介绍： Alzheimer's Research & Therapy is an international peer-reviewed journal that focuses on translational research into Alzheimer's disease and other neurodegenerative diseases. It publishes open-access basic research, clinical trials, drug discovery and development studies, and epidemiologic studies. The journal also includes reviews, viewpoints, commentaries, debates, and reports. All articles published in Alzheimer's Research & Therapy are included in several reputable databases such as CAS, Current contents, DOAJ, Embase, Journal Citation Reports/Science Edition, MEDLINE, PubMed, PubMed Central, Science Citation Index Expanded (Web of Science) and Scopus.