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Abstract Details

FDG-PET Patterns Associate With Survival in Patients With Prion Disease
Aging, Dementia, and Behavioral Neurology
P4 - Poster Session 4 (5:00 PM-6:00 PM)
3-005
To assess the association of patterns of network degeneration, measured via FDG-PET, and survival in patients with prion disease.

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.

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. 

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.

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.
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.
Nick Corriveau-Lecavalier, PhD Dr. Corriveau-Lecavalier has nothing to disclose.
Yoav Piura, MD 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.
Brian Appleby, MD (University Hospitals Case Medical Center) Dr. Appleby has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia. Dr. Appleby has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Ionis. Dr. Appleby has received personal compensation in the range of $0-$499 for serving as a Consultant for Sangamo. The institution of Dr. Appleby has received research support from CJD Foundation. The institution of Dr. Appleby has received research support from Ionis. The institution of Dr. Appleby has received research support from Alector. The institution of Dr. Appleby has received research support from CDC. The institution of Dr. Appleby has received research support from NIH. Dr. Appleby has received publishing royalties from a publication relating to health care. Dr. Appleby has received publishing royalties from a publication relating to health care.
Dror Shir, MD (Mayo Clinic) Dr. Shir has nothing to disclose.
Leland Barnard (Mayo Clinic) Leland Barnard has nothing to disclose.
Venkatsampath Raja Gogineni (Mayo Clinic) Venkatsampath Raja Gogineni has nothing to disclose.
David T. Jones, MD (Mayo Clinic) Dr. Jones has stock in Cephlodyne Neurotechnologies, Inc.. Dr. Jones has received intellectual property interests from a discovery or technology relating to health care.