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Abstract Details

Causes of Rapidly Progressive Dementia in a Prospectively Accrued Cohort
Aging, Dementia, and Behavioral Neurology
P2 - Poster Session 2 (11:45 AM-12:45 PM)
7-009
Describe a diagnostic approach to the evaluation of patients with suspected rapidly progressive dementia (RPD) and review common causes of RPD.
Overlap in clinical features and the results of diagnostic tests confounds the etiologic diagnoses of RPD and contributes to diagnostic delays, missed treatment opportunities, and poor patient outcomes. There is a need to improve diagnostic accuracy in patients with RPD.
Patients with RPD were prospectively enrolled and evaluated in inpatient settings and outpatient subspecialty (memory) clinics at two tertiary care centers from 2016-2022. Etiologic diagnoses were independently assigned by two dementia specialists, integrating clinical features and the results of accessible diagnostic tests, and referencing established diagnostic criteria. Diagnostic disagreements were resolved via blinded review by a third specialist when required. Clinicopathologic correlation was evaluated using neuropathological and genetic data when available.
225 patients with suspected RPD were assessed, of which 154 patients (68.4%) met established criteria for RPD. The median age-at-symptom onset of RPD was 66.0 years (range: 18.3-84.7); 46.7% were female. Diagnostic inter-rater reliability (91% agreement; Cohen’s κ=0.88, p<0.001) and clinicopathologic correlation were excellent (100% agreement in 24 patients with neuropathologic or genetic data). Autoimmune encephalitis was the leading cause of RPD (82/154, 53.2%), followed by Alzheimer disease and related dementias (45/154, 29.2%), including frontotemporal lobar degeneration, Lewy body disease, and vascular cognitive impairment. Creutzfeldt-Jakob disease accounted for 30/154 cases (19.4%). Other causes included neurosarcoidosis, metabolic disruptions, neoplasms, and other autoimmune/inflammatory diseases.
Etiologic diagnoses can be reliably established in patients with RPD using available clinical data. Potentially treatable causes of RPD are common emphasizing the need for diagnostic approaches that improve recognition of treatment-responsive patients early in the symptomatic course.
Authors/Disclosures
Gregory S. Day, MD, MSc, FAAN (Mayo Clinic)
PRESENTER 		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.
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.
Evelyn Lazar, MD (JFK Medical Center) Dr. Lazar has nothing to disclose.
Nihal Satyadev, MD, MPH (Mayo Clinic) Dr. Satyadev has nothing to disclose.
Yuka Martens (Mayo Clinic) No disclosure on file
Steven R. Dunham, Jr., MD 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 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.