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. 2020 Jan;16(1):79-90.
doi: 10.1016/j.jalz.2019.05.013. Epub 2020 Jan 6.

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

Toji Miyagawa  1 , Danielle Brushaber  1 , Jeremy Syrjanen  1 , Walter Kremers  1 , Julie Fields  1 , Leah K Forsberg  1 , Hilary W Heuer  2 , David Knopman  1 , John Kornak  2 , Adam Boxer  2 , Howie Rosen  2 , Bradley Boeve  1 ; ARTFL/LEFFTDS Consortium
Affiliations

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

Toji Miyagawa et al. Alzheimers Dement. 2020 Jan.

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.

Keywords: Behavior; CDR®; Comportment and personality; Frontotemporal dementia; Frontotemporal lobar degeneration; Language; NACC FTLD Module; Primary progressive aphasia.

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