Striatal hypometabolism in premanifest and manifest Huntington’s disease patients

  • 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
Original Article

DOI: 10.1007/s00259-016-3445-y

Cite this article as:
López-Mora, D.A., Camacho, V., Pérez-Pérez, J. et al. Eur J Nucl Med Mol Imaging (2016) 43: 2183. doi:10.1007/s00259-016-3445-y

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.

Keywords

Huntington’s disease 18F-FDG PET/CT Neurodegeneration imaging Premanifest HD Striatal hypometabolism Movement disorder 

Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Diego Alfonso López-Mora
    • 1
  • Valle Camacho
    • 1
  • Jesús Pérez-Pérez
    • 2
  • Saül Martínez-Horta
    • 2
  • Alejandro Fernández
    • 1
  • Frederic Sampedro
    • 3
  • Alberto Montes
    • 1
  • Gloria Andrea Lozano-Martínez
    • 4
  • Beatriz Gómez-Anson
    • 4
  • Jaime Kulisevsky
    • 2
  • Ignasi Carrió
    • 1
  1. 1.Nuclear Medicine Department, Hospital Sant PauAutonomous University of BarcelonaBarcelonaSpain
  2. 2.Movement Disorders Unit, Neurology Department, Hospital Sant PauAutonomous University of BarcelonaBarcelonaSpain
  3. 3.University of BarcelonaBarcelonaSpain
  4. 4.Neuroradiology, Radiology Department, Hospital Sant PauAutonomous University of BarcelonaBarcelonaSpain

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