title: Longitudinal magnetic resonance imaging study shows progressive pyramidal and callosal damage in Friedreich's ataxia.
abstract: BACKGROUND
  Spinal cord and peripheral nerves are classically known to be damaged in Friedreich's ataxia, but the extent of cerebral involvement in the disease and its progression over time are not yet characterized. The aim of this study was to evaluate longitudinally cerebral damage in Friedreich's ataxia.
  
  
  METHODS
  We enrolled 31 patients and 40 controls, which were evaluated at baseline and after 1 and 2 years. To assess gray matter, we employed voxel-based morphometry and cortical thickness measurements. White matter was evaluated using diffusion tensor imaging. Statistical analyses were both cross-sectional and longitudinal (corrected for multiple comparisons).
  
  
  RESULTS
  Group comparison between patients and controls revealed widespread macrostructural differences at baseline: gray matter atrophy in the dentate nuclei, brainstem, and precentral gyri; and white matter atrophy in the cerebellum and superior cerebellar peduncles, brainstem, and periventricular areas. We did not identify any longitudinal volumetric change over time. There were extensive microstructural alterations, including superior cerebellar peduncles, corpus callosum, and pyramidal tracts. Longitudinal analyses identified progressive microstructural abnormalities at the corpus callosum, pyramidal tracts, and superior cerebellar peduncles after 1 year of follow-up.
  
  
  CONCLUSIONS
  Patients with Friedreich's ataxia present more widespread gray and white matter damage than previously reported, including not only infratentorial areas, but also supratentorial structures. Furthermore, patients with Friedreich's ataxia have progressive microstructural abnormalities amenable to detection in a short-term follow-up.
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Thiago J R
authors.initials: TJ
authors.lastname: Rezende
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Cynthia B
authors.initials: CB
authors.lastname: Silva
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Clarissa L
authors.initials: CL
authors.lastname: Yassuda
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Brunno M
authors.initials: BM
authors.lastname: Campos
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Anelyssa
authors.initials: A
authors.lastname: D'Abreu
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Fernando
authors.initials: F
authors.lastname: Cendes
authors.affiliation: Medical Genetics, School of Medical Sciences, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Iscia
authors.initials: I
authors.lastname: Lopes-Cendes
authors.affiliation: Department of Neurology and Neuroimaging Laboratory, University of Campinas-UNICAMP, Campinas, Sao Paulo, Brazil.
authors.forename: Marcondes C
authors.initials: MC
authors.lastname: França
mesh_terms: D000328:Adult; D017668:Age of Onset; D003337:Corpus Callosum / Q000473:pathology*; D003430:Cross-Sectional Studies; D056324:Diffusion Tensor Imaging; D018450:Disease Progression; D005260:Female; D005621:Friedreich Ataxia / Q000473:pathology*; D066128:Gray Matter / Q000473:pathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008137:Longitudinal Studies; D008297:Male; D011712:Pyramidal Tracts / Q000473:pathology*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013223:Statistics as Topic; D055815:Young Adult
keywords: DTI; Freesurfer; Friedreich's ataxia; MRI; VBM