MRI of Hallervorden-Spatz disease

  • A. C. A. Magalhães
  • R. P. Maciel
  • L. A. Bacheschi
  • S. C. Mathias
  • J. R. Menezes
  • A. Magalhães
Conference paper


Two male and two female patients, aged between 10 and 28 years (mean =16 years) with clinical features of Hallervorden-Spatz disease (HSD) were submitted to MRI examination with a 1.5 T system. T1- and T2-weighted spin-echo sequences were obtained. Two patients received paramagnetic contrast medium. All four cases had low signal in the globus pallidus. Three also had central high signal (target configuration). In one there was also low signal in the substantia nigra. There was no enhancement with contrast medium. The diagnosis of HSD was supported by the MRI study in all four cases. This method proved to be useful for an “in vivo” diagnosis of HSD, making MRI the procedure of choice for confirmation in patients with clinical findings of Hallervorden-Spatz disease.

key words

Hallervorden-Spatz disease Basal ganglia disease Brain magnetic resonance imaging 


  1. 1.
    Atlas WS (1991) Magnetic resonance imaging of the brain and spine. Raven Press, New York, pp 594–597.Google Scholar
  2. 2.
    Dooling EC, Schoene WC et al (1974) Hallervorden-Spatz syndrome. Arch Neurol 30:70–83.PubMedCrossRefGoogle Scholar
  3. 3.
    Galluci M, Cardona F, Arachi M et al (1990) Follow-up MR studies in Hallervorden-Spatz disease. J Comput Assist Tomogr 14:118–120.CrossRefGoogle Scholar
  4. 4.
    Savoiardo M, Halliday WC, Nardocci N, Strada L, DÍncerti L, Angelini L, Rumi V, Tesoro-Tess JD (1993) Hallervorden-Spatz disease: MR and pathologic findings. AJNR 14:155–162.PubMedGoogle Scholar
  5. 5.
    Sethi KD, Adams RJ, Loring DW, el Gammal T (1988) Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations. Ann Neurol 24:692–694.PubMedCrossRefGoogle Scholar
  6. 6.
    Tanfani G, Mascalchi M, DalPozzo GC et al (1987) MRI in a case of Hallervorden-Spatz disease. J Comput Assist Tomogr 11:1057–1058.PubMedCrossRefGoogle Scholar
  7. 7.
    Trussart V, Leboucq N, Carlander B, Billiard M, Castan P (1993) Hallervorden-Spatz syndrome and MRI: “tiger’s eye”. One case. J Neuroradiol 20:70–75.PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • A. C. A. Magalhães
    • 1
  • R. P. Maciel
  • L. A. Bacheschi
  • S. C. Mathias
  • J. R. Menezes
  • A. Magalhães
    • 2
  1. 1.University of São Paulo School of MedicineSão PauloBrazil
  2. 2.Department of Radiology, Section of Magnetic Resonance, Hospital das ClínicasUniversity of São PauloSáo PauloBrazil

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