Gregory Day1, Nick Corriveau-Lecavalier2, Yoav Piura1, Brian Appleby3, Dror Shir2, Leland Barnard2, Venkatsampath Raja Gogineni2, David Jones2
1Neurology, Mayo Clinic in Florida, 2Neurology, Mayo Clinic, 3University Hospitals Case Medical Center
Objective:
To assess the association of patterns of network degeneration, measured via FDG-PET, and survival in patients with prion disease.
Background:
Patients with prion disease classically present with rapid progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising questions surrounding the patient- and disease-specific factors that contribute to variability in presentation and survival.
Design/Methods:
Clinical and FDG-PET data were obtained from 40 patients with prion disease (21 females) evaluated at our tertiary care center from 2006-2023. Spectral decomposition of covariance was performed on FDG-PET images by projecting them onto a set of latent factors generated in an external dataset to yield patient-specific eigenvalues. These eigenvalues were input into a hierarchical clustering algorithm to generate data-driven clusters, which were then compared to disease duration and other relevant demographic and clinical variables.
Results:
Median age at FDG-PET was 65.3 years (range 22.8-84.9) with survival from FDG-PET ranging from 0.3-19.2 (median 3.7) months. The optimal clustering solution generated four data-driven clusters, termed “Neocortical” (n = 7), “Transitional” (n = 12), “Temporo-parietal” (n = 13), and “Deep nuclei” (n = 6). Deep nuclei and transitional clusters had a shorter time from FDG-PET to death than the neocortical cluster. This difference associated with greater hypometabolism of deep nuclei relative to neocortical areas. FDG-PET-patterns were not associated with relevant demographic (age, sex) or clinical (CSF T-tau, 14-3-3) variables.
Conclusions:
Greater hypometabolism within deep nuclei versus neocortical areas associated with more rapid decline in patients with prion disease; the opposite pattern associated with longer disease duration. These findings suggest that FDG-PET informs large-scale network physiology and may inform the relationship between spreading pathology and survival in patients with prion disease.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.