title: Striatal hypometabolism in premanifest and manifest Huntington's disease patients.
abstract: OBJECTIVE
  To assess metabolic changes in cerebral18F-FDG PET/CT in premanifest and manifest Huntington's disease (HD) subjects compared to a control group and to correlate18F-FDG uptake patterns with different disease stages.
  
  
  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 brain18F-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 of18F-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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Nuclear Medicine Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain. dlopezmo@santpau.cat.
authors.forename: Diego Alfonso
authors.initials: DA
authors.lastname: López-Mora
authors.affiliation: Nuclear Medicine Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Valle
authors.initials: V
authors.lastname: Camacho
authors.affiliation: Movement Disorders Unit, Neurology Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Jesús
authors.initials: J
authors.lastname: Pérez-Pérez
authors.affiliation: Movement Disorders Unit, Neurology Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Saül
authors.initials: S
authors.lastname: Martínez-Horta
authors.affiliation: Nuclear Medicine Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Fernández
authors.affiliation: University of Barcelona, Barcelona, Spain.
authors.forename: Frederic
authors.initials: F
authors.lastname: Sampedro
authors.affiliation: Nuclear Medicine Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Montes
authors.affiliation: Neuroradiology, Radiology Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Gloria Andrea
authors.initials: GA
authors.lastname: Lozano-Martínez
authors.affiliation: Neuroradiology, Radiology Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Gómez-Anson
authors.affiliation: Movement Disorders Unit, Neurology Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Jaime
authors.initials: J
authors.lastname: Kulisevsky
authors.affiliation: Nuclear Medicine Department, Hospital Sant Pau, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Ignasi
authors.initials: I
authors.lastname: Carrió
mesh_terms: D000328:Adult; D000368:Aged; D001928:Brain Diseases, Metabolic / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism*; D003342:Corpus Striatum / Q000000981:diagnostic imaging / Q000378:metabolism*; D042241:Early Diagnosis; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000493:pharmacokinetics*; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000000981:diagnostic imaging* / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D000072078:Positron Emission Tomography Computed Tomography / Q000379:methods*; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D055815:Young Adult
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18
keywords: 18F-FDG PET/CT; Huntington’s disease; Movement disorder; Neurodegeneration imaging; Premanifest HD; Striatal hypometabolism