title: Late-onset neurodegeneration with brain iron accumulation with diffusion tensor magnetic resonance imaging.
abstract: BACKGROUND
  Neuroferritinopathy is an autosomal dominant neurodegenerative disorder that includes a movement disorder, cognitive decline, and characteristic findings on brain magnetic resonance imaging (MRI) due to abnormal iron deposition. Here, we present a late-onset case, along with diffusion tensor imaging (DTI).
  
  
  METHODS
  We report the case of a 74-year-old Caucasian female with no significant past medical history who presented for evaluation of orofacial dyskinesia, suspected to be edentulous dyskinesia given her history of ill-fitting dentures. She had also developed slowly progressive dysarthria, dysphagia, visual hallucinations as well as stereotypic movements of her hands and feet.
  
  
  RESULTS
  The eye-of-the-tiger sign was demonstrated on T2 MRI. Increased fractional anisotropy and T2 hypointensity were observed in the periphery of the globus pallidus, putamen, substantia nigra, and dentate nucleus. T2 hyperintensity was present in the medial dentate nucleus and central globus pallidus.
  
  
  CONCLUSIONS
  The pallidal MRI findings were more typical of pantothenate kinase-associated neurodegeneration (PKAN), but given additional dentate and putamenal involvement, lack of retinopathy, and advanced age of onset, PKAN was less likely. Although the patient's ferritin levels were within low normal range, her clinical and imaging features led to a diagnosis of neuroferritinopathy.
  
  
  CONCLUSIONS
  Neurodegeneration with brain iron accumulation (NBIA) is a rare cause of orofacial dyskinesia. DTI MRI can confirm abnormal iron deposition. The location of abnormal iron deposits helps in differentiating NBIA subtypes. Degeneration of the dentate and globus pallidus may occur via an analogous process given their similar T2 and DTI MRI appearance.
authors.affiliation: Department of Neurology, New York Medical College, Valhalla, N.Y., USA.
authors.forename: Syed Omar
authors.initials: SO
authors.lastname: Shah
authors.forename: Hasit
authors.initials: H
authors.lastname: Mehta
authors.forename: Robert
authors.initials: R
authors.lastname: Fekete
keywords: Diffusion tensor imaging; Hyperkinetic movement disorders; Neurodegeneration with brain iron accumulation; Neuroferritinopathy