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Abstract Details

Utility of the FTLD-CDR in the mild FTLD: Data from the ARTFL/LEFFTDS Consortium
Aging, Dementia, and Behavioral Neurology
P3 - Poster Session 3 (5:30 PM-6:30 PM)
9-001
Our study was performed to propose a framework for detecting early clinical changes of the frontotemporal lobar degeneration (FTLD) spectrum diseases.
The widely used standard 6-domain Clinical Dementia Rating (CDRstd) primarily focuses on AD, thus gives more weight on memory and orientation impairment, and less on behavioral and language issues. To broaden the utility of the CDRstd and to capture early symptomatology in FTLD patients, CDRstd was expanded to add and include behavior/comportment/personality (BEHAV) and language (LANG) domains to create the 8-domain FTLD-modified CDR (FTLD-CDR). A few studies have demonstrated the utility of FTLD-CDR in FTLD participants.
We analyzed the CDRstd and FTLD-CDR among 861 sporadic and familial participants from the baseline visits of the ARTFL/LEFFTDS Consortium.
For the CDRstd-SB <4 mildly impaired participants, the frequency of BEHAV ratings ≥0.5 and ≥1, respectively, were: behavioral variant frontotemporal dementia (bvFTD) 100.0%/79.6%, mild behavioral changes (MCI-beh) 100.0%/61.5%, nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) 43.9%/7.3%, and semantic variant PPA (svPPA) 80.6%/32.3%. The ratings for LANG (ratings ≥0.5 and ≥1) were: bvFTD 53.7%/13.0%, MCI-beh 23.1%/0%, nfvPPA 100.0%/92.7%, and svPPA 100.0%/90.3%. There were no significant differences in the FTLD-CDR profile between familial and sporadic bvFTD participants. All 11 MCI-beh/bvFTD/PPA participants who were rated as normal (CDRstd 0) had abnormal FTLD-CDR ratings.
Added BEHAV and LANG domains in the FTLD-CDR were able to capture early symptomatology of FTLD.
Authors/Disclosures
Toji Miyagawa, MD, PhD (Mayo Clinic)
PRESENTER 		Dr. Miyagawa has nothing to disclose.
No disclosure on file
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.
Ralitza H. Gavrilova, MD (Mayo Clinic) Dr. Gavrilova has nothing to disclose.
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
Joel Kramer, PhD (UCSF Medical Center) The institution of Dr. Kramer has received research support from tau consortium. Dr. Kramer has received publishing royalties from a publication relating to health care.
Walter Kremers The institution of Walter Kremers has received research support from NIH. The institution of an immediate family member of Walter Kremers has received research support from NIH.
Kelsey Rankin (UCSF) No disclosure on file
Katya Rascovsky (University of Pennsylvania Perelman School of Medicine) No disclosure on file
Jeremy Syrjanen Jeremy Syrjanen has nothing to disclose.
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.
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 $10,000-$49,999 for serving as a Consultant for Oscotec. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arrowhead. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Neurocrine Biosciences. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Switch. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arvinas. Dr. Boxer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alector. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. 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 $500-$4,999 for serving as a Consultant for Janssen . Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Boxer has stock in Alector. Dr. Boxer has stock in Arvinas. Dr. Boxer has stock in Neurovanda. 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-5. Dr. Boxer has received research support from Rainwater Charitable Foundation. Dr. Boxer has received research support from GHR Foundation. Dr. Boxer has received intellectual property interests from a discovery or technology relating to health care.
Howard J. Rosen, MD (UCSF) Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly . Dr. Rosen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alector. 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 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.