Structural and functional brain abnormalities place phenocopy frontotemporal dementia (FTD) in the FTD spectrum

Rebecca M E Steketee, Rozanna Meijboom, Esther E Bron, Robert Jan Osse, Inge de Koning, Lize C Jiskoot, Stefan Klein, Frank Jan De Jong, Aad van der Lugt, John C van Swieten, Marion Smits

Research output: Contribution to journalArticlepeer-review

Abstract

PURPOSE: 'Phenocopy' frontotemporal dementia (phFTD) patients may clinically mimic the behavioral variant of FTD (bvFTD), but do not show functional decline or abnormalities upon visual inspection of routine neuroimaging. We aimed to identify abnormalities in gray matter (GM) volume and perfusion in phFTD and to assess whether phFTD belongs to the FTD spectrum. We compared phFTD patients with both healthy controls and bvFTD patients.

MATERIALS & METHODS: Seven phFTD and 11 bvFTD patients, and 20 age-matched controls underwent structural T1-weighted magnetic resonance imaging (MRI) and 3D pseudo-continuous arterial spin labeling (pCASL) at 3T. Normalized GM (nGM) volumes and perfusion, corrected for partial volume effects, were quantified regionally as well as in the entire supratentorial cortex, and compared between groups taking into account potential confounding effects of gender and scanner.

RESULTS: PhFTD patients showed cortical atrophy, most prominently in the right temporal lobe. Apart from this regional atrophy, GM volume was generally not different from either controls or from bvFTD. BvFTD however showed extensive frontotemporal atrophy. Perfusion was increased in the left prefrontal cortex compared to bvFTD and to a lesser extent to controls.

CONCLUSION: PhFTD and bvFTD show overlapping cortical structural abnormalities indicating a continuum of changes especially in the frontotemporal regions. Together with functional changes suggestive of a compensatory response to incipient pathology in the left prefrontal regions, these findings are the first to support a possible neuropathological etiology of phFTD and suggest that phFTD may be a neurodegenerative disease on the FTD spectrum.

Original languageEnglish
Pages (from-to)595-605
Number of pages11
JournalNeuroImage: Clinical
Volume11
DOIs
Publication statusPublished - Apr 2016

Keywords

  • Aged
  • Brain
  • Brain Mapping
  • Case-Control Studies
  • Cerebrovascular Circulation
  • Frontotemporal Dementia
  • Gray Matter
  • Humans
  • Magnetic Resonance Imaging
  • Middle Aged
  • Neuropsychological Tests
  • Psychiatric Status Rating Scales
  • Spin Labels

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