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Hallervorden-Spatz disease

progressive neurodegenerative disease
  • extrapyramidal dysfunction (parkonsonism)
  • autosomal recessive inhertance

CT findings

  • high-signal lesions in globus pallidus bilaterally

MRI findings (T2-weighted spin-echo)

  • hypointensity in globus pallidus (iron accumulation)
  • small area of hyperintensity in internal segment (so-called "eye of the tiger" sign) corresponding to area of gliosis and vacuolation

James Lowe, DM BM BS MRCPath  -  13 November 1997
Last updated:  1 October 2013


 

Related CHORUS documents:
parkinsonism
brain iron
Wilson disease
Huntington disease (chorea-athetosis)
progressive supranuclear palsy (Steele-Richardson-Olszewski syndome)
acanthosis nigricans

 

Copyright © 2013, Charles E. Kahn, Jr.