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Article type: Research Article
Authors: Trebbastoni, Alessandroa; * | D’Antonio, Fabriziaa | de Lena, Carloa | Onesti, Emanuelaa | John, Bevb | Inghilleri, Maurizioa
Affiliations: [a] Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy | [b] School of Psychology, University of South Wales, Pontypridd, UK
Correspondence: [*] Correspondence to: Alessandro Trebbastoni, MD, PhD, Department of Neurology and Psychiatry, “Sapienza” University of Rome, Viale dell’Università 30, 00185 Rome, Italy. Tel.: +39 06 49914485; Fax: +39 06 49694216; E-mail: [email protected].
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.
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
DOI: 10.3233/JAD-160525
Journal: Journal of Alzheimer's Disease, vol. 54, no. 3, pp. 1039-1045, 2016
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