title: Primary Progressive Orofacial Apraxia: A Ten-Year Long Follow-Up Case Report.
abstract: 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.
authors: Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
authors: Alessandro
authors: A
authors: Trebbastoni
authors: Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
authors: Fabrizia
authors: F
authors: D'Antonio
authors: Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
authors: Carlo
authors: C
authors: de Lena
authors: Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
authors: Emanuela
authors: E
authors: Onesti
authors: School of Psychology, University of South Wales, Pontypridd, UK.
authors: Bev
authors: B
authors: John
authors: Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
authors: Maurizio
authors: M
authors: Inghilleri
mesh_terms: D000368:Aged; D018888:Aphasia, Primary Progressive / Q000150:complications / Q000000981:diagnostic imaging* / Q000523:psychology; D001072:Apraxias / Q000150:complications / Q000000981:diagnostic imaging* / Q000523:psychology; D005152:Facial Muscles / Q000000981:diagnostic imaging*; D005260:Female; D005500:Follow-Up Studies; D005625:Frontal Lobe / Q000000981:diagnostic imaging; D006801:Humans; D019636:Neurodegenerative Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000523:psychology; D009483:Neuropsychological Tests
keywords: Apraxia of speech; cerebrospinal fluid biomarkers; frontotemporal lobar degeneration; orofacial apraxia; primary progressive aphasia; primary progressive apraxia of speech; single-photon emission computed tomography