Abstract Details

Title Interrater Reliability of the Multidomain Impairment Rating (MIR) Scale in Frontotemporal Lobar Degeneration: Data from the ALLFTD Consortium

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-005

Background The ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) Consortium aims to characterize individuals with FTLD to prepare for clinical trials. The MIR was developed to be similar to the CDR® Dementia Staging Instrument (CDR®) scale and the CDR® plus NACC FTLD. The FTLD syndromes have a complex array of cognitive, behavioral/psychiatric and motor symptoms that can contribute to functional impairment, and one of the goals of the MIR is to capture the multidomain contributions to functional impairment.

Objective To assess the interrater reliability of the Multidomain Impairment Rating (MIR) Scale for use in Frontotemporal Lobar Degeneration (FTLD)-related research and clinical trials.

Design/Methods Four additional domains were developed and added to the CDR® plus NACC FTLD; Concentration and Multitasking, Visuospatial Functioning, Psychiatric Features, and Motor. Each added domain is rated on a five-point scale: 0 (normal), 0.5 (questionably or minimally impaired), 1 (mildly but definitely impaired), 2 (moderately impaired), and 3 (severely impaired) from information based on the interviews with the participant and the informant, and the neurological examination findings. An interrater reliability study was performed between four raters on 60 ALLFTD participants. Weighted cohen kappa and intraclass correlation coefficients (ICC) statistics measured interrater reliability.

Results Clinical diagnosis for the 60 participants were 11 behavioral variant frontotemporal dementia (bvFTD), 2 amyotrophic lateral sclerosis (ALS), 2 FTD/ALS, 5 corticobasal syndrome, 3 primary progressive aphasia, 3 progressive supranuclear palsy, 14 mild behavioral and/or cognitive impairment, and 20 normal. The global MIR score and the MIR sum of all subdomain scores (MIR-SS) both showed excellent interrater reliability for pairwise rater comparisons with weighted cohen kappa ranging from 0.88 to 0.94 for the global MIR score and with ICC ranging from 0.90 to 0.99 for the MIR-SS.

Conclusions The MIR scale may serve as a useful comprehensive clinical assessment tool in natural history studies and clinical trials in FTLD.

Authors/Disclosures
Toji Miyagawa, MD, PhD (Mayo Clinic) PRESENTER	Dr. Miyagawa has nothing to disclose.
Jennifer Merrilees (UCSF)	No disclosure on file
Jeremy Syrjanen	No disclosure on file
Danielle Brushaber	No disclosure on file
Walter Kremers	No disclosure on file
Julie A. Fields, PhD (Mayo Clinic)	The institution of Dr. Fields has received research support from National Institutes of Health. The institution of Dr. Fields has received research support from Patient-Centered Outcomes Research Institute.
Leah K. Forsberg, PhD (Mayo Clinic)	Ms. Forsberg has nothing to disclose.
Hilary Heuer (UCSF)	No disclosure on file
Edward Huey (Brown University)	No disclosure on file
David S. Knopman, MD, FAAN (Mayo Clinic)	Dr. Knopman has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for DIAN TU study. The institution of Dr. Knopman has received research support from NIH.
John Kornak	No disclosure on file
Adam L. Boxer, MD, PhD (University of California, San Francisco)	An immediate family member of Dr. Boxer has received personal compensation for serving as an employee of Kaiser Permanente. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ono. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Third Rock. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Transposon. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Oligomerix. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GSK. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Humana. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Wave. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Boehringer Ingelheim. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Oscotec. Dr. Boxer has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arvinas. Dr. Boxer has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arkuda. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AZTherapeutics. Dr. Boxer has stock in Alector. Dr. Boxer has stock in Arkuda. Dr. Boxer has stock in Arvinas. An immediate family member of Dr. Boxer has stock in AZtherapies. Dr. Boxer has stock in TrueBinding. The institution of Dr. Boxer has received research support from Biogen. The institution of Dr. Boxer has received research support from Eisai. The institution of Dr. Boxer has received research support from Regeneron. The institution of Dr. Boxer has received research support from NIH. The institution of Dr. Boxer has received research support from Bluefield Project. Dr. Boxer has received research support from Rainwater Charitable Foundation. The institution of Dr. Boxer has received research support from Association for Frontotemporal Degeneration.
Howard J. Rosen, MD (UCSF)	Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai Pharmaceuticals. Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. The institution of Dr. Rosen has received research support from NIH. The institution of Dr. Rosen has received research support from State of CA. Dr. Rosen has a non-compensated relationship as a Consultant with Alector that is relevant to AAN interests or activities. Dr. Rosen has a non-compensated relationship as a Consultant with Prevail Therapeutics that is relevant to AAN interests or activities. Dr. Rosen has a non-compensated relationship as a consultant with Alchemab that is relevant to AAN interests or activities.
Bradley F. Boeve, MD, FAAN (Mayo Clinic)	Dr. Boeve has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Rainwater Charitable Foundation. The institution of Dr. Boeve has received research support from Alector. The institution of Dr. Boeve has received research support from EIP Pharma. The institution of Dr. Boeve has received research support from Transposon. The institution of Dr. Boeve has received research support from Cognition Therapeutics. Dr. Boeve has received publishing royalties from a publication relating to health care.