title: Brain fluorodeoxyglucose PET in adrenoleukodystrophy.
abstract: OBJECTIVE
  To investigate the cerebral glucose metabolism in subjects with X-linked adrenoleukodystrophy (X-ALD) by using brain [(18)F]-fluorodeoxyglucose PET (FDG-PET).
  
  
  METHODS
  Cross-sectional study in which 12 adults with various forms of X-ALD underwent clinical evaluation and brain MRI, followed by brain FDG-PET, neuropsychological assessment, and personality and psychopathology evaluation using the Symptom Checkist-90-Revised (SCL-90-R) and the Millon Clinical Multiaxial Inventory-III (MCMI-III).
  
  
  RESULTS
  When compared to healthy control subjects (n = 27) by using Statistical Parametric Mapping 8 software, the patients with X-ALD-with or without brain MRI changes-showed a pattern of increased glucose metabolism in frontal lobes and reduced glucose metabolism in cerebellum and temporal lobe areas. On single case analysis by Scenium software, we found a similar pattern, with significant (p < 0.02) correlation between the degree of hypermetabolism in the frontal lobes of each patient and the corresponding X-ALD clinical scores. With respect to personality, we found that patients with X-ALD usually present with an obsessive-compulsive personality disorder on the MCMI-III, with significant (p < 0.05) correlation between glucose uptake in ventral striatum and severity of score on the obsessive-compulsive subscale.
  
  
  CONCLUSIONS
  We examined cerebral glucose metabolism using FDG-PET in a cohort of patients with X-ALD and provided definite evidence that in X-ALD the analysis of brain glucose metabolism reveals abnormalities independent from morphologic and signal changes detected by MRI and related to clinical severity. Brain FDG-PET may be a useful neuroimaging technique for the characterization of X-ALD and possibly other leukodystrophies.
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. ettore.salsano@istituto-besta.it.
authors.forename: Ettore
authors.initials: E
authors.lastname: Salsano
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Marotta
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Manfredi
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Giovagnoli
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Farina
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Savoiardo
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Pareyson
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Benti
authors.affiliation: From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Graziella
authors.initials: G
authors.lastname: Uziel
mesh_terms: D000326:Adrenoleukodystrophy / Q000000981:diagnostic imaging* / Q000378:metabolism*; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism*; D001931:Brain Mapping / Q000379:methods; D003430:Cross-Sectional Studies; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010551:Personality / Q000502:physiology; D010552:Personality Assessment; D049268:Positron-Emission Tomography; D011569:Psychiatric Status Rating Scales; D019275:Radiopharmaceuticals; D012984:Software; D055815:Young Adult
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D005947:Glucose