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European Journal of Nuclear Medicine and Molecular Imaging

Published in:

01-11-2016 | Original Article

Striatal hypometabolism in premanifest and manifest Huntington’s disease patients

Authors: Diego Alfonso López-Mora, Valle Camacho, Jesús Pérez-Pérez, Saül Martínez-Horta, Alejandro Fernández, Frederic Sampedro, Alberto Montes, Gloria Andrea Lozano-Martínez, Beatriz Gómez-Anson, Jaime Kulisevsky, Ignasi Carrió

Published in: European Journal of Nuclear Medicine and Molecular Imaging | Issue 12/2016

Abstract

Purpose

To assess metabolic changes in cerebral ¹⁸F-FDG PET/CT in premanifest and manifest Huntington’s disease (HD) subjects compared to a control group and to correlate ¹⁸F-FDG uptake patterns with different disease stages.

Materials and methods

Thirty-three gene-expanded carriers (Eight males; mean age: 43 y/o; CAG > 39) were prospectively included. Based on the Unified Huntington’s Disease Rating Scale Total Motor Score and the Total Functional Capacity, subjects were classified as premanifest (preHD = 15) and manifest (mHD = 18). Estimated time disease-onset was calculated using the Langbehn formula, which allowed classifying preHD as far-to (preHD-A) and close-to (PreHD-B) disease-onset. Eighteen properly matched participants were included as a control group (CG). All subjects underwent brain ¹⁸F-FDG PET/CT and MRI. ¹⁸F-FDG PET/CT were initially assessed by two nuclear medicine physicians identifying qualitative metabolic changes in the striatum. Quantitative analysis was performed using SPM8 with gray matter atrophy correction using the BPM toolbox.

Results

Visual analysis showed a marked striatal hypometabolism in mHD. A normal striatal distribution of ¹⁸F-FDG uptake was observed for most of the preHD subjects. Quantitative analysis showed a significant striatal hypometabolism in mHD subjects compared to CG (p < 0.001 uncorrected, k = 50 voxels). In both preHD groups we observed a significant striatal hypometabolism with respect to CG (p < 0.001 uncorrected, k = 50 voxels). In mHD subjects we observed a significant striatal hypometabolism with respect to both preHD groups (p < 0.001 uncorrected, k = 50 voxels).

Conclusion

¹⁸F-FDG PET/CT might be a helpful tool to identify patterns of glucose metabolism in the striatum across the stages of HD and might be relevant in assessing the clinical status of gene-expanded HD carriers due to the fact that dysfunctional glucose metabolism begins at early preHD stages of the disease. ¹⁸F-FDG PET/CT appears as a promising method to monitor the response to disease-modifying therapies even if applied in premanifest subjects.

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Title: Striatal hypometabolism in premanifest and manifest Huntington’s disease patients
Authors: Diego Alfonso López-Mora
Valle Camacho
Jesús Pérez-Pérez
Saül Martínez-Horta
Alejandro Fernández
Frederic Sampedro
Alberto Montes
Gloria Andrea Lozano-Martínez
Beatriz Gómez-Anson
Jaime Kulisevsky
Ignasi Carrió
Publication date: 01-11-2016
Publisher: Springer Berlin Heidelberg
Published in: European Journal of Nuclear Medicine and Molecular Imaging / Issue 12/2016
Print ISSN: 1619-7070
Electronic ISSN: 1619-7089
DOI: https://doi.org/10.1007/s00259-016-3445-y

At a glance: The STEP trials

Springer Medicine

Striatal hypometabolism in premanifest and manifest Huntington’s disease patients

Abstract

Purpose

Materials and methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Materials and methods

Results

Conclusion

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