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Abstract Details

Improving Early Recognition of Patients with Rapidly Progressive Dementia
Aging, Dementia, and Behavioral Neurology
S1 - Innovations in Non-AD Dementia (1:36 PM-1:48 PM)
004
Patients presenting with faster-than-expected cognitive decline may have RPD or a condition that may stabilize or revert to typically progressive dementia. Early distinction between ‘true’ RPD and non-RPD cases is critical for effective patient management and research participation.
To improve recognition of patients who will develop rapid progressive dementia (RPD).
We prospectively enrolled patients with suspected RPD at two tertiary centers (Mayo Clinic, Florida and Washington University, St. Louis) between 2016-2023. Two dementia specialists independently reviewed clinical data and established diagnoses based on published criteria. RPD was defined as progression to dementia (global CDR ≥1) within 1 year or incapacitation (global CDR ≥2) within 2 years of symptom onset. We compared clinical, imaging and biofluid features between RPD and non-RPD groups using univariate statistics, then performed multivariate logistic regression on potentially associated variables (p<0.10) to identify factors independently associated with RPD.

Of 248 patients with suspected RPD, 187 (75.4%) ultimately met RPD criteria. RPD patients were older (62.6±14.4 vs 55.0±18.3 years; p<0.001) and more likely to have Alzheimer (AD: 19.1% vs 4.6%; p=0.008) or Creutzfeldt-Jakob disease (CJD: 28.3% vs 9.8%; p=0.003). Non-RPD cases were often attributed to autoimmune/inflammatory (42.6%) or other causes (e.g., psychiatric, neoplasms, toxic/metabolic conditions; 34.4%). After controlling for age, hallucinations (OR, 2.86; 95%CI: 1.26-6.52), and normal cerebrospinal fluid white blood cell count (<5 cells/mm3; OR, 2.07; 95%CI: 1.05-4.09) were independently associated with RPD. Cortical visual loss (15/183, 8.2%), advanced brain atrophy (27/184, 14.7%) and periodic epileptiform discharges on electroencephalogram (11/153, 71.9%) were exclusive to RPD cases.

Hallucinations, cortical visual loss, substantial atrophy on brain MRI, periodic epileptiform discharges on electroencephalogram, and normal cerebrospinal fluid white blood cell count were independently associated with “true RPD”. Early recognition of these features in patients with suspected RPD may inform clinical care and support enrollment in studies of RPD.
Authors/Disclosures
Yoav Piura, MD
PRESENTER 		Dr. Piura has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Piura has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck.
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.
Steven R. Dunham, Jr., MD (Washington University School of Medicine) Dr. Dunham has nothing to disclose.
Nihal Satyadev, MD, MPH (Mayo Clinic) Dr. Satyadev has nothing to disclose.
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 Parabon Nanolabs. 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 Vigil Neuro. 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 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 a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities.