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J Alzheimers Dis. 2016 Oct 4;54(3):1039-1045.

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

Author information

1
Department of Neurology and Psychiatry, Sapienza, University of Rome, Italy.
2
School of Psychology, University of South Wales, Pontypridd, UK.

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

PMID:
27567870
DOI:
10.3233/JAD-160525
[Indexed for MEDLINE]

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