Primary Progressive Aphasia

Natural History in an Italian Cohort

Camilla Ferrari, Cristina Polito, Sara Vannucchi, Irene Piaceri, Silvia Bagnoli, Gemma Lombardi, Giulia Lucidi, Valentina Berti, Benedetta Nacmias, Sandro Sorbi

Research output: Contribution to journalArticle

Abstract

Background/Aims: 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. Results: 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. Conclusions: There were no differences in the progression of PPA subtypes. Genetics plays an important role in disease onset and progression.

Original languageEnglish
JournalAlzheimer Disease and Associated Disorders
DOIs
Publication statusAccepted/In press - Jan 1 2018

Fingerprint

Primary Progressive Aphasia
Natural History
Disease Progression
Language
Confidence Intervals
Apolipoproteins E
Semantics
Neuroimaging
Longitudinal Studies
Dementia
Electrons
Mutation
Cognitive Dysfunction

Keywords

  • dementia
  • genetic
  • neurodegeneration
  • primary progressive aphasia
  • progression

ASJC Scopus subject areas

  • Clinical Psychology
  • Gerontology
  • Geriatrics and Gerontology
  • Psychiatry and Mental health

Cite this

Primary Progressive Aphasia : Natural History in an Italian Cohort. / Ferrari, Camilla; Polito, Cristina; Vannucchi, Sara; Piaceri, Irene; Bagnoli, Silvia; Lombardi, Gemma; Lucidi, Giulia; Berti, Valentina; Nacmias, Benedetta; Sorbi, Sandro.

In: Alzheimer Disease and Associated Disorders, 01.01.2018.

Research output: Contribution to journalArticle

Ferrari, C, Polito, C, Vannucchi, S, Piaceri, I, Bagnoli, S, Lombardi, G, Lucidi, G, Berti, V, Nacmias, B & Sorbi, S 2018, 'Primary Progressive Aphasia: Natural History in an Italian Cohort', Alzheimer Disease and Associated Disorders. https://doi.org/10.1097/WAD.0000000000000282
Ferrari, Camilla ; Polito, Cristina ; Vannucchi, Sara ; Piaceri, Irene ; Bagnoli, Silvia ; Lombardi, Gemma ; Lucidi, Giulia ; Berti, Valentina ; Nacmias, Benedetta ; Sorbi, Sandro. / Primary Progressive Aphasia : Natural History in an Italian Cohort. In: Alzheimer Disease and Associated Disorders. 2018.
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