Primary Progressive Orofacial Apraxia: A Ten-Year Long Follow-Up Case Report

Alessandro Trebbastoni, Fabrizia D'Antonio, Carlo de Lena, Emanuela Onesti, Bev John, Maurizio Inghilleri

Research output: Contribution to journalArticlepeer-review


Orofacial apraxia (OA) as the main symptom in neurodegenerative disorders has not been yet reported. We present the case of a woman with a 22-month long history of isolated OA, studied with cerebrospinal fluid biomarkers and repeated clinical, neuropsychological, and morpho-functional evaluations. Baseline morpho-functional neuroimages revealed a left frontal operculum hypoperfusion with a widespread fronto-temporal involvement at follow-up. Cerebrospinal fluid concentrations of tau and amyloid-β were normal. The ten-year long clinical observation disclosed progressive OA worsening and the late onset of frontal functions impairment and extrapyramidal signs. The early and late stages of a neurodegenerative syndrome with OA as the main clinical feature were characterized.

Original languageEnglish
Pages (from-to)1039-1045
Number of pages7
JournalJournal of Alzheimer's Disease
Issue number3
Publication statusPublished - 4 Oct 2016


  • Apraxia of speech
  • cerebrospinal fluid biomarkers
  • frontotemporal lobar degeneration
  • orofacial apraxia
  • primary progressive aphasia
  • primary progressive apraxia of speech
  • single-photon emission computed tomography


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