Utility of the global CDR^® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium

Abstract

Introduction-We created global rating scoring rules for the CDR® plus NACC FTLD to detect and track early frontotemporal lobar degeneration (FTLD) and to conduct clinical trials in FTLD.Methods-The CDR plus NACC FTLD rating was applied to 970 sporadic and familial participants from the baseline visit of Advancing Research and Treatment in Frontotemporal Lobar Degeneration (ARTFL)/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (LEFFTDS). Each of the eight domains of the CDR plus NACC FTLD was equally weighed in determining the global score. An interrater reliability study was completed for 40 participants.Results-The CDR plus NACC FTLD showed very good interrater reliability. It was especially useful in detecting clinical features of mild non-fluent/agrammatic variant primary progressive aphasia participants.Discussion-The global CDR plus NACC FTLD score could be an attractive outcome measure for clinical trials in symptomatic FTLD, and may be useful in natural history studies and clinical trials in FTLD spectrum disorders.

Rates of Brain Atrophy Across Disease Stages in Familial Frontotemporal Dementia Associated With MAPT, GRN, and C9orf72 Pathogenic Variants

“…Neuropsychological tests included the Uniform Data Set (version 3.0) neuropsychological battery. 28 Functional status was quantified using CDR + NACC FTLD 27 , 29 , 30 (details are provided in the Supplement ). Brain imaging was not used for diagnosis or severity rating.…”

Emotion recognition of morphed facial expressions in presymptomatic and symptomatic frontotemporal dementia, and Alzheimer’s dementia

“…Mutation carriers were from either microtubule-associated protein tau ( MAPT ; n = 7), progranulin ( GRN ; n = 22) or C9orf72 ( n = 18) families. Mutation carriers were deemed to be presymptomatic when they did not fulfill clinical diagnostic criteria for bvFTD [ 1 ], PPA [ 2 ] or FTD-ALS [ 22 ], and had CDR ® plus Behaviour and Language domains from the NACC FTLD Module (CDR ® plus NACC FTLD) [ 25 ] of 0. The investigators and participants were blinded for the genetic status of at-risk participants, except for those that underwent predictive testing at their own request.…”

“…The Mini-Mental State Examination (MMSE) [ 26 ] was administered as measure of global cognitive functioning. The clinical dementia rating scale (CDR) [ 27 ] was used as a measure of disease severity in patients with AD, while patients with bvFTD from the Erasmus Medical Center as well as FTD-RisC participants were assessed about functional changes in behaviour, neuropsychiatric symptoms, cognition and language by means of the CDR ® plus NACC FTLD [ 25 ] during the study visit or afterwards in a telephone interview.…”

An MRI-based strategy for differentiation of frontotemporal dementia and Alzheimer’s disease

“…We first included 47 subjects with FTD (19 bvFTD, 12 svPPA, 2 nfvPPA, and 14 not otherwise specified) from NACC. Then, 47 subjects with probable AD and 47 NC perfectly matching those with FTD (Table 1) in age, educational level, gender, race, and global CDR score (except for NC) using the CDR® Dementia Staging Instrument plus NACC FTLD Behavior & Language Domains [27][28][29] were selected from NACC as well. Subjects were included if their diagnoses were stable (> 1 year) and 3D T1weighted MRI scans were available ± 6 months of a visit.…”