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Amyloid in dementia associated with familial FTLD: not an innocent bystander.

  • Author(s): Naasan, Georges
  • Rabinovici, Gil D
  • Ghosh, Pia
  • Elofson, Jonathan D
  • Miller, Bruce L
  • Coppola, Giovanni
  • Karydas, Anna
  • Fong, Jamie
  • Perry, David
  • Lee, Suzee E
  • Yokoyama, Jennifer S
  • Seeley, William W
  • Kramer, Joel H
  • Weiner, Michael W
  • Schuff, Norbert
  • Jagust, William J
  • Grinberg, Lea T
  • Pribadi, Mochtar
  • Yang, Zhongan
  • Sears, Renee
  • Klein, Eric
  • Wojta, Kevin
  • Rosen, Howard J
  • et al.
Abstract

Patients with frontotemporal lobar degeneration (FTLD) can show superimposed amyloid pathology, though the impact of amyloid on the clinical presentation of FTLD is not well characterized. This cross-sectional case-control study compared clinical features, fluorodeoxyglucose-positron emission tomography metabolism and gray matter volume loss in 30 patients with familial FTLD in whom amyloid status was confirmed with autopsy or Pittsburgh compound B-PET. Compared to the amyloid-negative patients, the amyloid-positive patients performed significantly worse on several cognitive tests and showed hypometabolism and volume loss in more temporoparietal regions. Our results suggest that in FTLD amyloid positivity is associated with a more Alzheimer's disease-like pattern of neurodegeneration.

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