title: Brain MR diffusion tensor imaging in Kennedy's disease.
abstract: BACKGROUND
  Kennedy's disease (KD) is a progressive degenerative disorder affecting lower motor neurons. We investigated the correlation between disease severity and whole brain white matter microstructure, including upper motor neuron tracts, by using diffusion-tensor imaging (DTI) in eight patients with KD in whom disease severity was evaluated using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS).
  
  
  METHODS
  From DTI acquisitions we obtained maps of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (L1) and radial diffusivities (L2, L3). We then employed tract-based spatial statistics (TBSS) to investigate within-patient correlations of DTI invariants with ALSFRS and disease duration (DD).
  
  
  RESULTS
  We found a significant correlation between low ALSFRS and 1) low FA values in association commissural and projection fibers, and 2) high L3 values in commissural tracts and fronto-parietal white matter. Additionally, we found a significant association between longer DD and 1) low FA in the genu and body of corpus callosum, association fibers and midbrain and 2) high L1 in projection and association tracts.
  
  
  CONCLUSIONS
  The associations between clinical variables and white matter microstructural changes in areas thought to be spared by the disease process support the hypothesis of a multisystem involvement in the complex pathogenic mechanisms responsible for the clinical disability of these patients.
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Garaci
authors.affiliation: Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy Department of Radiology, Athinoula A. Martinos Center for Biomedical Imaging, USA and Harvard Medical School, USA toschi@med.uniroma2.it.
authors.forename: Nicola
authors.initials: N
authors.lastname: Toschi
authors.affiliation: Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Lanzafame
authors.affiliation: Department of Systems Medicine, Section Neurology, University of Rome Tor Vergata, Italy.
authors.forename: Girolama A
authors.initials: GA
authors.lastname: Marfia
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy.
authors.forename: Simone
authors.initials: S
authors.lastname: Marziali
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Meschini
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Di Giuliano
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Simonetti
authors.affiliation: Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Guerrisi
authors.affiliation: Department of Systems Medicine, Section Neurology, University of Rome Tor Vergata, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Massa
authors.affiliation: Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University Hospital Tor Vergata, Italy Department of Biomedicine and Prevention, Faculty of Medicine, University of Rome Tor Vergata, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Floris
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology*; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000473:pathology*; D056324:Diffusion Tensor Imaging / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009413:Nerve Fibers, Myelinated / Q000473:pathology*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
keywords: ALSFRS; DTI; Kennedy’s disease; TBSS; disease duration; fractional anisotropy; mean diffusivity