title: Topography of brain glucose hypometabolism and epileptic network in glucose transporter 1 deficiency.
abstract: BACKGROUND
  (18)F fluorodeoxyglucose positron emission tomography ((18)F FDG-PET) facilitates examination of glucose metabolism. Previously, we described regional cerebral glucose hypometabolism using (18)F FDG-PET in patients with Glucose transporter 1 Deficiency Syndrome (Glut1 DS). We now expand this observation in Glut1 DS using quantitative image analysis to identify the epileptic network based on the regional distribution of glucose hypometabolism.
  
  
  METHODS
  (18)F FDG-PET scans of 16 Glut1 DS patients and 7 healthy participants were examined using Statistical parametric Mapping (SPM). Summed images were preprocessed for statistical analysis using MATLAB 7.1 and SPM 2 software. Region of interest (ROI) analysis was performed to validate SPM results.
  
  
  RESULTS
  Visual analysis of the (18)F FDG-PET images demonstrated prominent regional glucose hypometabolism in the thalamus, neocortical regions and cerebellum bilaterally. Group comparison using SPM analysis confirmed that the regional distribution of glucose hypo-metabolism was present in thalamus, cerebellum, temporal cortex and central lobule. Two mildly affected patients without epilepsy had hypometabolism in cerebellum, inferior frontal cortex, and temporal lobe, but not thalamus. Glucose hypometabolism did not correlate with age at the time of PET imaging, head circumference, CSF glucose concentration at the time of diagnosis, RBC glucose uptake, or CNS score.
  
  
  CONCLUSIONS
  Quantitative analysis of (18)F FDG-PET imaging in Glut1 DS patients confirmed that hypometabolism was present symmetrically in thalamus, cerebellum, frontal and temporal cortex. The hypometabolism in thalamus correlated with the clinical history of epilepsy.
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States; Department of Neurology, Comprehensive Epilepsy Center, Columbia University College of Physician & Surgeons, United States. Electronic address: cia11@columbia.edu.
authors.forename: Cigdem Inan
authors.initials: CI
authors.lastname: Akman
authors.affiliation: Department of Radiology, Kreitchman PET Center, Columbia University College of Physician & Surgeons, United States.
authors.forename: Frank
authors.initials: F
authors.lastname: Provenzano
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States.
authors.forename: Dong
authors.initials: D
authors.lastname: Wang
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States.
authors.forename: Kristin
authors.initials: K
authors.lastname: Engelstad
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States.
authors.forename: Veronica
authors.initials: V
authors.lastname: Hinton
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States.
authors.forename: Julia
authors.initials: J
authors.lastname: Yu
authors.affiliation: Department of Radiology, Kreitchman PET Center, Columbia University College of Physician & Surgeons, United States.
authors.forename: Ronald
authors.initials: R
authors.lastname: Tikofsky
authors.affiliation: Department of Radiology, Kreitchman PET Center, Columbia University College of Physician & Surgeons, United States.
authors.forename: Masonari
authors.initials: M
authors.lastname: Ichese
authors.affiliation: Department of Neurology, Division of Pediatric Neurology, Colleen Giblin Research Laboratory, Columbia University College of Physician & Surgeons, United States.
authors.forename: Darryl C
authors.initials: DC
authors.lastname: De Vivo
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism*; D001931:Brain Mapping; D002239:Carbohydrate Metabolism, Inborn Errors / Q000000981:diagnostic imaging / Q000235:genetics / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004827:Epilepsy / Q000000981:diagnostic imaging / Q000235:genetics / Q000378:metabolism*; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism*; D051272:Glucose Transporter Type 1 / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007223:Infant; D008875:Middle Aged; D009004:Monosaccharide Transport Proteins / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D009434:Neural Pathways / Q000000981:diagnostic imaging / Q000378:metabolism; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D012815:Signal Processing, Computer-Assisted; D055815:Young Adult
chemical_list: D051272:Glucose Transporter Type 1; D009004:Monosaccharide Transport Proteins; D019275:Radiopharmaceuticals; C495677:SLC2A1 protein, human; D019788:Fluorodeoxyglucose F18; D005947:Glucose
keywords: Brain glucose metabolism; Glucose transporter deficiency; Positron emission tomography; Statistical parametric mapping