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Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium.

  • Author(s): Miyagawa, Toji
  • Brushaber, Danielle
  • Syrjanen, Jeremy
  • Kremers, Walter
  • Fields, Julie
  • Forsberg, Leah K
  • Heuer, Hilary W
  • Knopman, David
  • Kornak, John
  • Boxer, Adam
  • Rosen, Howie
  • Boeve, Bradley
  • ARTFL/LEFFTDS Consortium
  • et al.
Abstract

INTRODUCTION:Behavior/Comportment/Personality (BEHAV) and Language (LANG) domains were added to the Clinical Dementia Rating (CDR®) for improving evaluation of patients with frontotemporal lobar degeneration (FTLD) (CDR® plus NACC FTLD). METHODS:We analyzed the CDR® plus NACC FTLD among participants from the baseline visit of the Advancing Research and Treatment for Frontotemporal Lobar Degeneration/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects Consortium. RESULTS:The CDR® plus NACC FTLD was able to detect early symptoms in the mildly impaired participants who were rated as CDR® sum of boxes (CDR®-SB) = 0. The CDR®-SB was not sensitive, particularly in participants with mild nonfluent/agrammatic primary progressive aphasia. Participants with familial and sporadic behavioral variant FTD exhibited similar CDR® plus NACC FTLD profiles except that language impairment was more frequent in participants with mild sporadic behavioral variant FTD. Adding the BEHAV and/or LANG domains to the CDR®-SB significantly enhanced discriminatory power in differentiating among the FTLD spectrum disorders. DISCUSSION:The BEHAV and LANG domains enable the CDR® plus NACC FTLD to capture early symptomatology of FTLD.

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