Atypical Hallervorden-Spatz disease with preserved cognition and obtrusive obsessions and compulsions

Academic Article


  • We describe the case of an adult female with Hallervorden-Spatz disease (HSD), "eye-of-the-tiger" sign on cranial magnetic resonance imaging scan, and two mutations in the pantothenate kinase 2 (PANK2) gene. Symptomatic presentation included stuttering dysarthria, dystonic posturing, increased limb and axial muscle tone, choreoathetosis, stereotyped motor behaviors, and obsessive-compulsive symptomatology since adolescence. Extensive neuropsychological testing at 40 and 44 years of age revealed a relatively normal IQ and stable cognitive pattern overall. This case demonstrates that HSD patients who survive into middle age should not be assumed to have a progressive dementia. In such cases, atypical behavioral problems such as persistent obsessions and compulsions may be present instead. © 2005 Movement Disorder Society.
  • Published In

  • Movement Disorders  Journal
  • Digital Object Identifier (doi)

    Author List

  • Nicholas AP; Earnst KS; Marson DC
  • Start Page

  • 880
  • End Page

  • 886
  • Volume

  • 20
  • Issue

  • 7