Skip to main content
padlock icon - secure page this page is secure

Primary Progressive Aphasia

Buy Article:

$52.00 + tax (Refund Policy)


Few longitudinal studies have explored the progression of cognitive and functional impairment of patients with primary progressive aphasia (PPA). The aims of the study were to describe the clinical, neuroimaging, and genetic features of a cohort of 68 PPA patients, and to outline the natural history of the disease.

Materials and Methods:

A sample of 23 patients with the logopenic variant, 26 with the nonfluent/agrammatic variant, and 19 with the semantic variant was retrospectively collected and followed-up for a maximum of 6 years. Clinical-neuropsychological assessment, fluorodeoxyglucose positron emission tomographic imaging, and genetic analyses were acquired at baseline. Disease progression was evaluated in terms of language impairment, global cognitive decline, and functional dependency.


During follow-up, one third of subjects presented total language loss, and 20% severe functional dependency. Global cognitive decline after the first year (hazard ratio, 5.93; confidence interval, 1.63-21.56) and high schooling (hazard ratio, 0.07; confidence interval, 0.008-0.74) represented risk factors for functional impairment. The apolipoprotein E status was associated with the progression of cognitive decline. Positive family history for dementia was frequent and 3 genetic autosomal dominant mutations were identified.


There were no differences in the progression of PPA subtypes. Genetics plays an important role in disease onset and progression.
No Reference information available - sign in for access.
No Citation information available - sign in for access.
No Supplementary Data.
No Article Media
No Metrics

Keywords: dementia; genetic; neurodegeneration; primary progressive aphasia; progression

Document Type: Research Article

Affiliations: 1: Departments of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA) 2: Biomedical, Experimental and Clinical Sciences “Mario Serio,” Nuclear Medicine Unit, University of Florence, Viale Pieraccini 3: Departments of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), IRCCS Fondazione Don Carlo Gnocchi, Florence, Italy

Publication date: January 1, 2019

  • Access Key
  • Free content
  • Partial Free content
  • New content
  • Open access content
  • Partial Open access content
  • Subscribed content
  • Partial Subscribed content
  • Free trial content
Cookie Policy
Cookie Policy
Ingenta Connect website makes use of cookies so as to keep track of data that you have filled in. I am Happy with this Find out more