title: Cerebral involvement in spinal and bulbar muscular atrophy (Kennedy's disease): a pilot study of PET.
abstract: OBJECTIVE
  To investigate possible cerebral involvement in patients with spinal and bulbar muscular atrophy (SBMA) by (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET).
  
  
  METHODS
  Ten patients with molecularly-confirmed SBMA and 5 age- and gender-matched healthy controls were recruited for brain FDG-PET studies. The data were analyzed and compared using the statistical parametric mapping (SPM) method.
  
  
  RESULTS
  Glucose hypometabolism in frontal areas of the cerebrum was found in patients with SBMA. However, no significant correlation with clinical variables, such as CAG repeat length, age at onset, or serum testosterone levels, was noted.
  
  
  CONCLUSIONS
  The perturbation of cerebral glucose metabolism in patients with SBMA argues against SBMA being a pure lower motor and sensory neuron syndrome. Mutations in the androgen receptor gene might have a more widespread effect in the cerebrum than previously recognized.
authors.affiliation: Department of Neurology, National Yang-Ming University, Taipei, Taiwan; Institute of Neuroscience, National Yang-Ming University, Taipei, Taiwan; Division of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, New Taipei City, Taiwan; Department of Neurology, Taipei Veterans General Hospital, Taipei, Taiwan.
authors.forename: Tzu-Hsien
authors.initials: TH
authors.lastname: Lai
authors.forename: Ren-Shyan
authors.initials: RS
authors.lastname: Liu
authors.forename: Bang-Hung
authors.initials: BH
authors.lastname: Yang
authors.forename: Po-Shan
authors.initials: PS
authors.lastname: Wang
authors.forename: Kon-Ping
authors.initials: KP
authors.lastname: Lin
authors.forename: Yi-Chung
authors.initials: YC
authors.lastname: Lee
authors.forename: Bing-Wen
authors.initials: BW
authors.lastname: Soong
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D001931:Brain Mapping; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000473:pathology; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D049268:Positron-Emission Tomography
chemical_list: D019788:Fluorodeoxyglucose F18
keywords: Androgen receptor; Kennedy's disease; Motor neuron disease; Polyglutamine disease; Positron emission tomography; Spinal and bulbar muscular atrophy