Introduction <p>Data on the population-based incidence of primary progressive aphasia (PPA), including all variants and Alzheimer’s disease (AD) biomarker profiles, are scarce.</p> Methods <p>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.</p> Results <p>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.</p> Discussion <p>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.</p>

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

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

摘要

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.