Session Information
Date: Sunday, October 7, 2018
Session Title: Huntington's Disease
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: By a combined brain PET–MRI using FDG, we wished to simultaneously characterize the structural and metabolic brain changes in premanifest HD gene-expansion carriers.
Background: Huntington’s disease (HD) is an autosomal dominantly inherited neurodegenerative disorder, caused by an expansion of a trinucleotide (CAG) repeat in the huntingtin gene. There is no cure and only sparse symptomatic treatment. Structural brain imaging is the most applied and well documented technique to demonstrate longitudinal structural changes in premanifest and manifest HD gene-expansion carriers. Changes in the striatum are registered as far as 15 years before symptom onset with MRI. PET studies have found hypometabolism in the Caudate nucleus, Putamen and the temporal and frontal cortex years before clinical diagnosis along with hypermetabolism in Thalamus prior to symptom onset.
Methods: We recruited 22 premanifest HD gene-expansion carriers and 17 controls from the Neurogenetics Clinic, Danish Dementia Research Centre, Rigshospitalet, Copenhagen, Denmark. We included individuals with a CAG repeat ≥39 and a Unified Huntingtin’s Disease Rating scale-99 total motor score ≤5.
Results: We found significantly reduced volumes of the Putamen bilaterally and the Globus Pallidus in the right hemisphere. Further we found significantly reduced metabolism in the Putamen bilaterally with a significant correlation between CAG age product and the FDG activity which, however, disappeared when correcting for the reduced volume of Putamen.
Conclusions: Our results indicate that the hypometabolism and atrophy of Putamen are evolving simultaneously. A follow-up study on the cohort will shed more light on the sequential evolution and correlation of structural and metabolic changes.
To cite this abstract in AMA style:
M. Hellem, T. Vinther-Jensen, C. Hansen, E. Budtz-Jørgensen, L. Hjermind, I. Law, J. Nielsen. A Study on Combined Brain Positron Emission Tomography (PET) – Magnetic Resonance Imaging (MRI) Using Fluorodeoxyglucose (18FDG) (FDG-PET/MRI) in Premanifest Huntington’s Disease Gene-expansion Carriers [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/a-study-on-combined-brain-positron-emission-tomography-pet-magnetic-resonance-imaging-mri-using-fluorodeoxyglucose-18fdg-fdg-pet-mri-in-premanifest-huntingtons-disease-gene/. Accessed May 18, 2025.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/a-study-on-combined-brain-positron-emission-tomography-pet-magnetic-resonance-imaging-mri-using-fluorodeoxyglucose-18fdg-fdg-pet-mri-in-premanifest-huntingtons-disease-gene/