We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Patterns of glucose hypometabolism can help differentiate FTLD-FET from other types of FTLD.
- Authors
Garcia-Guaqueta, Danna P.; Ghayal, Nikhil B.; Lowe, Val J.; Dickson, Dennis W.; Whitwell, Jennifer L.; Josephs, Keith A.
- Abstract
Introduction: FTLD-FET is a newly described subtype of frontotemporal lobar degeneration (FTLD characterized by pathologic inclusions of FET proteins: fused in sarcoma (FUS), Ewing sarcoma, and TATA-binding protein-associated factor 2N (TAF15)). Severe caudate volume loss on MRI has been linked to FTLD-FUS, yet glucose hypometabolism in FTLD-FET has not been studied. We assessed [18F] fluorodeoxyglucose PET (FDG-PET) hypometabolism in FTLD-FET subtypes and compared metabolism to FTLD-tau and FTLD-TDP. Methods: We retrospectively reviewed medical records of 26 autopsied FTLD patients (six FTLD-FET, ten FTLD-Tau, and ten FTLD-TDP) who had completed antemortem FDG-PET. We evaluated five regions, caudate nucleus, medial frontal cortex, lateral frontal cortex, and medial temporal using a 0–3 visual rating scale and validated our findings quantitatively using CORTEX-ID suite Z scores. Results: Of the six FTLD-FET cases (three females) with median age at onset = 36, three were atypical FTLD-U (aFTLD-U) and three were neuronal intermediate filament inclusion disease (NIFID). bvFTD was the most common presentation. Four of the six FTLD cases (3 aFTLD-U + 1 NIFID) showed prominent caudate hypometabolism relatively early in the disease course. FTLD-tau and FTLD-TDP did not show early prominent caudate hypometabolism. Hypometabolism in medial and lateral temporal cortex was associated with FTLD-TDP, while FTLD-tau had normal-minimal regional metabolism. Discussion: Prominent caudate hypometabolism, especially early in the disease course, appears to be a hallmark feature of the aFTLD-U subtype of FTLD-FET. Assessing caudate and temporal hypometabolism on FDG-PET will help to differentiate FTLD-FET from FTLD-tau and FTLD-TDP.
- Subjects
FRONTAL lobe; CAUDATE nucleus; EWING'S sarcoma; CYTOPLASMIC filaments; TEMPORAL lobe; FRONTOTEMPORAL lobar degeneration
- Publication
Journal of Neurology, 2024, Vol 271, Issue 9, p6264
- ISSN
0340-5354
- Publication type
Article
- DOI
10.1007/s00415-024-12583-y