%0 Journal Article %J J Alzheimers Dis %D 2016 %T Primary Progressive Orofacial Apraxia: A Ten-Year Long Follow-Up Case Report. %A Trebbastoni, Alessandro %A D'Antonio, Fabrizia %A de Lena, Carlo %A Onesti, Emanuela %A John, Bev %A Inghilleri, Maurizio %X

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.

%B J Alzheimers Dis %V 54 %P 1039-1045 %8 2016 Oct 04 %G eng %N 3 %1 http://www.ncbi.nlm.nih.gov/pubmed/27567870?dopt=Abstract %R 10.3233/JAD-160525