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

Behav Neurol. 2013;26(1-2):95-106. doi: 10.3233/BEN-2012-120255.

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.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Alzheimer Disease / complications
  • Alzheimer Disease / pathology
  • Amyloidosis / complications
  • Amyloidosis / diagnostic imaging
  • Amyloidosis / pathology*
  • Aniline Compounds
  • Carbon Radioisotopes
  • Disease Progression
  • Female
  • Fluorine Radioisotopes
  • Fluorodeoxyglucose F18
  • Frontal Lobe / diagnostic imaging
  • Frontal Lobe / pathology*
  • Functional Neuroimaging / methods
  • Functional Neuroimaging / psychology*
  • Humans
  • Magnetic Resonance Imaging / methods
  • Magnetic Resonance Imaging / psychology
  • Neuropsychological Tests / statistics & numerical data
  • Pick Disease of the Brain / complications
  • Pick Disease of the Brain / pathology*
  • Positron-Emission Tomography / methods
  • Positron-Emission Tomography / psychology
  • Primary Progressive Nonfluent Aphasia / complications
  • Primary Progressive Nonfluent Aphasia / diagnostic imaging
  • Primary Progressive Nonfluent Aphasia / pathology
  • Thiazoles

Substances

  • 2-(4'-(methylamino)phenyl)-6-hydroxybenzothiazole
  • Aniline Compounds
  • Carbon Radioisotopes
  • Fluorine Radioisotopes
  • Thiazoles
  • Fluorodeoxyglucose F18