title: Changes of cerebral white matter in patients suffering from Pantothenate Kinase-Associated Neurodegeneration (PKAN): A diffusion tensor imaging (DTI) study.
abstract: BACKGROUND
  To look for microstructural white matter alterations in patients with dystonia due to Pantothenate Kinase-Associated Neurodegeneration.
  
  
  METHODS
  We examined 21 genetically confirmed patients and an age-matched group of 21 healthy controls by diffusion tensor imaging. Evaluation of data was performed by tract-based spatial statistics analysis and a voxel-wise comparison of calculated maps of fractional anisotropy. Findings were compared between groups and correlated to the dystonia score of the Burke-Fahn-Marsden Scale (p ≤ 0.05).
  
  
  RESULTS
  Patients showed reductions of fractional anisotropy mainly in the periventricular substance surrounding the third ventricle, in the medial part of both putamina and in the frontal white matter including the anterior limbs of the internal capsules and the corpus callosum. Infratentorially, the cerebellar white matter and dorsal parts of the pons and medulla were affected.
  
  
  CONCLUSIONS
  In addition to cortical grey matter changes, we now have a second structural finding pointing to a more widespread affection of cerebral tissue in PKAN dystonia than just the lesion and iron accumulation in the globus pallidus.
authors.affiliation: Dep. of Radiology, Santo Domingo, Dominican Republic. Electronic address: peter.stoeter@gmx.de.
authors.forename: P
authors.initials: P
authors.lastname: Stoeter
authors.affiliation: Dep. of Neurology, Santo Domingo, Dominican Republic.
authors.forename: P
authors.initials: P
authors.lastname: Roa-Sanchez
authors.affiliation: Dep. of Radiology, Santo Domingo, Dominican Republic.
authors.forename: H
authors.initials: H
authors.lastname: Speckter
authors.affiliation: Dep. of Medical Science, CEDIMAT, Santo Domingo, Dominican Republic.
authors.forename: E
authors.initials: E
authors.lastname: Perez-Then
authors.affiliation: Philips Medical Systems LatAm, Sao Paulo, Brazil.
authors.forename: B
authors.initials: B
authors.lastname: Foerster
authors.affiliation: Dep. of Radiology, Santo Domingo, Dominican Republic.
authors.forename: C
authors.initials: C
authors.lastname: Vilchez
authors.affiliation: Dep. of Radiology, Santo Domingo, Dominican Republic.
authors.forename: J
authors.initials: J
authors.lastname: Oviedo
authors.affiliation: Dep. of Neuroradiology, University Clinic RWTH Aachen, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Rodriguez-Raecke
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002540:Cerebral Cortex / Q000473:pathology; D056324:Diffusion Tensor Imaging; D004421:Dystonia / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000473:pathology*; D066127:White Matter / Q000473:pathology*
keywords: Diffusion tensor imaging; Pantothenate Kinase-Associated Neurodegeneration; White matter tracts