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Abstract Details

Application of a Standardized and Reproducible Definition of Rapidly Progressive Dementia
Aging, Dementia, and Behavioral Neurology
P10 - Poster Session 10 (11:45 AM-12:45 PM)
9-014
Develop and apply a standardized and reproducible definition of rapidly progressive dementia (RPD) in clinical and research settings. 
A lack of clear diagnostic criteria for RPD has resulted in wide-ranging definitions and wider-ranging estimates of RPD frequency (1-30%). A standardized and reproducible definition of RPD that clearly differentiates patients with RPD from individuals with “typically progressive dementia” is needed to promote collaborative multi-institutional research.
The Clinical Dementia Rating® (CDR) is a validated and reproducible measure of cognitive function derived via semi-structured interview with a patient and knowledgeable collateral source. We labelled patients as rapidly progressive if they progressed from CDR 0 (cognitively normal) to ≥1 (dementia) within 1 year or CDR 0 to ≥2 (complete dependence on others for activities of daily living) within 2 years. Proposed criteria were applied to patients referred for the evaluation of suspected RPD to Mayo Clinic Florida (Jacksonville, FL) and Washington University School of Medicine (St. Louis, MO), and to participants with dementia included within the National Alzheimer’s Coordination Center (NACC) dataset.
71/87 (81.6%) of Mayo Clinic patients and 84/169 (49.7%) of Washington University patients met the proposed definition of RPD (χ2=24.5, p < 0.0001). Neurodegenerative (including prion disease) and autoimmune diseases were the most common causes of RPD at both centers, accounting for 59/155 (38.1%) and 52/155 (33.5%) of total cases, respectively. 901/32211 (2.8%) patients in the NACC dataset met RPD criteria. Almost all cases were attributed to neurodegenerative diseases (894/901, 99.2%).The criteria selected a distinct population when considering time-to-CDR in the NACC dataset (Mann-Whitney U statistic: 131,000, p < 0.0001). 
Our CDR-based RPD definition can identify patients with exceptional rates of decline across multiple clinics and research sites. Future studies will compare the performance of a CDR-based definitions versus other time- or test-based criteria for RPD.
Authors/Disclosures
Nihal Satyadev, MD, MPH (Mayo Clinic)
PRESENTER 		Dr. Satyadev has nothing to disclose.
Philip W. Tipton, MD Dr. Tipton has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AbbVie. Dr. Tipton has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Alzheimer's Tennessee. Dr. Tipton has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Charlotte County Medical Society, Inc.
Neill R. Graff-Radford, MD, FAAN (Mayo Clinic Jacksonville) The institution of Dr. Graff-Radford has received research support from Biogen. The institution of Dr. Graff-Radford has received research support from Lilly. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Biogen. Dr. Graff-Radford has received publishing royalties from a publication relating to health care.
Gregory S. Day, MD, MSc, FAAN (Mayo Clinic) Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has or had stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Amgen Pharmaceuticals. The institution of Dr. Day has received research support from AVID Radiopharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with 好色先生. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing 好色先生, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Ionis Pharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a 好色先生al Case Development + Presentation (video) with PeerDirect (P\S\L Group). Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development / Presentation (non-CME) with MJH Life Sciences (NeurologyLive). Dr. Day has a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities.