Journal
NEUROCASE
Volume 24, Issue 2, Pages 111-120Publisher
ROUTLEDGE JOURNALS, TAYLOR & FRANCIS LTD
DOI: 10.1080/13554794.2018.1477963
Keywords
Apraxia of speech; MRI; FDG-PET; longitudinal; corticobasal degeneration
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Funding
- National Institute of Neurological Disorders and Stroke [R21-NS89757]
- National Institute on Deafness and Other Communication Disorders [R01-DC010367, R01-DC12519]
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Primary progressive apraxia of speech (PPAOS) is a neurodegenerative disorder in which AOS is the sole presenting complaint. We report clinical and neuroimaging data spanning 10 years from disease onset-to-death in a 49year-old male PPAOS patient, DY, who died with corticobasal degeneration. He presented with AOS with normal neuroimaging. Abnormalities in the caudate nucleus, supplementary motor area, cingulate, insula, and Broca's area were observed after five years, with involvement of motor cortex and development of agrammatism, Parkinsonism, and dysarthria three years later. Cognitive impairment and temporoparietal atrophy were late features. This data provides important insight into disease progression of corticobasal degeneration when presenting as PPAOS.
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