Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology

Francesca Caso, Benno Gesierich, Maya Henry, Manu Sidhu, Amanda Lamarre, Miranda Babiak, Bruce L. Miller, Gil D. Rabinovici, Eric J. Huang, Giuseppe Magnani, Massimo Filippi, Giancarlo Comi, William W. Seeley, Maria Luisa Gorno-Tempini

Research output: Contribution to journalArticlepeer-review

Abstract

The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer's disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.

Original languageEnglish
Pages (from-to)95-106
Number of pages12
JournalBehavioural Neurology
Volume26
Issue number1-2
DOIs
Publication statusPublished - 2013

Keywords

  • Alzheimer disease
  • apraxia of speech
  • Frontotemporal dementia
  • Nonfluent primary progressive aphasia
  • PiB-PET
  • Pick's disease
  • PPA
  • Voxel-based morphometry

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Clinical Neurology
  • Neurology

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