Philip Tipton1, Yoav Piura1, Steven Dunham2, Nihal Satyadev1, Neill Graff-Radford1, Gregory Day1
1Mayo Clinic Florida, 2Washington University School of Medicine
Objective:
To improve recognition of patients who will develop rapid progressive dementia (RPD).
Background:
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.
Design/Methods:
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.
Results:
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.
Conclusions:
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.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.