Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations

Ann Neurol. 1988 Nov;24(5):692-4. doi: 10.1002/ana.410240519.


Two women with a presumptive diagnosis of Hallervorden-Spatz syndrome had a combination of dystonia and parkinsonism. One had retinitis pigmentosa. Neuropsychological testing revealed decreased verbal fluency and visuoconstructional and motor deficits. Magnetic resonance imaging performed with a high-field-strength unit (1.5 Tesla) showed striking abnormalities in the globus pallidus bilaterally ("eye-of-the-tiger" sign). Magnetic resonance imaging may prove useful in the diagnosis of Hallervorden-Spatz syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Basal Ganglia Diseases / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging*
  • Neuropsychological Tests
  • Pantothenate Kinase-Associated Neurodegeneration / diagnosis
  • Pantothenate Kinase-Associated Neurodegeneration / pathology*
  • Pantothenate Kinase-Associated Neurodegeneration / psychology