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Abstract Details

Improving Early Recognition of Potentially Treatable Causes of Rapidly Progressive Dementia
Aging, Dementia, Cognitive, and Behavioral Neurology
P5 - Poster Session 5 (11:45 AM-12:45 PM)
2-001
RPD includes patients with less than two years from the onset of cognitive impairment to incapacitation due to dementia. Although RPD is often associated with invariably fatal neurodegenerative diseases such as Creutzfeldt-Jakob disease, treatable forms of RPD are increasingly recognized in clinical practice. Early recognition of treatment responsive causes of RPD is associated with earlier treatment and better outcomes.
To improve recognition of patients with potentially treatable causes of rapidly progressive dementia (RPD).
154 patients with RPD were prospectively enrolled from February 2016 to August 2022 in studies of RPD at two tertiary care centers. Two neurologists independently determined etiologic diagnoses. Causes of RPD were further classified as treatment-responsive or non-responsive. Demographic, clinical, and paraclinical features associated with treatable causes of RPD were identified using stepwise multivariate logistic regression.
82/154 patients (53.2%) had potentially treatable causes of RPD, including autoimmune conditions (n=51, 62.2%), vasculopathies (n=17, 20.7%), psychiatric conditions (n=4, 4.8%), and nutritional deficiencies (n=4, 4.8%). Younger age at symptom onset (OR=1.22 per decade, 95%CI: 1.11-1.36), seizures at presentation (OR=6.86, 95%CI: 2.27-20.68), CSF pleocytosis (OR=6.47, 95%CI: 2.36-17.84), and MRI features suggestive of autoimmune encephalitis (OR=6.19, 95%CI: 1.21-31.79) were associated with greater odds of a treatable cause of RPD. Model performance was good (area-under-the curve = 0.81; 95%CI: 0.75-0.87; p<0.001), corresponding to >80% accuracy in detection of patients with potentially treatable causes of RPD.
Treatment-responsive causes of RPD were common in our series. Younger age, early seizures, and CSF- or imaging-based evidence of inflammation should prompt consideration of treatable causes, with early initiation of treatment when indicated.
Authors/Disclosures
Nihal Satyadev, MD, MPH (Mayo Clinic)
PRESENTER
Mr. Satyadev has nothing to disclose.
Philip W. Tipton, MD (Mayo Clinic) 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.
Yuka Atagi Martens, PhD (Mayo Clinic) Dr. Martens has nothing to disclose.
Steven Richard Dunham, Jr., MD (Washington University School of Medicine) Dr. Dunham has nothing to disclose.
Michael D. Geschwind, MD, PhD, FAAN (UCSF) Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..
John C. Morris, MD, FAAN (Washington University) Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CBR International Advisory Board. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cure Alzheimers Fund. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for LEADS Steering Commitee. The institution of Dr. Morris has received research support from NIH grants. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care.
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 Novartis. The institution of Dr. Graff-Radford has received research support from AbbVie. 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 $5,000-$9,999 for serving as a Consultant for Parabon Nanolabs. The institution of Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Eli Lilly. 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 a Consultant for Ionis. 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 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 Chan Zuckerberg Initiative. The institution of Dr. Day has received research support from Alzheimer's Association. 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 Horizon Therapeutics. 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 American Academy of Neurology. 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 Education, Inc. Dr. Day has a non-compensated relationship as a Clinical Director with AntiNMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities.