Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration

Katerina A. Tetzloff, Joseph R. Duffy, Edythe A. Strand, Mary Margaret Machulda, Sarah M. Boland, Rene Utianski, Hugo Botha, Matthew L. Senjem, Christopher Schwarz, Keith Anthony Josephs, Jennifer Lynn Whitwell

Research output: Contribution to journalArticle

6 Scopus citations

Abstract

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 49 year-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.

Original languageEnglish (US)
Pages (from-to)1-10
Number of pages10
JournalNeurocase
DOIs
StateAccepted/In press - May 26 2018

Keywords

  • Apraxia of speech
  • corticobasal degeneration
  • FDG-PET
  • longitudinal
  • MRI

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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