J Korean Neurol Assoc.
1992 Sep;10(3):407-412.
A Case of Suspected Hallervorden-Spatz Disease
- Affiliations
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- 1Department of Neurology, College of Medicine, Pusan National University.
- 2Department of Neurology, Saint Joseph's Hospital.
Abstract
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Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests various symptoms and signs of extrapyramidal and pyramidal involvement. Authors report a 28-year-old female patient with suspected Hallervorden-Spatz disease in the aspects of clinical and MRI findings suggesting metal deposition in the globus pallidus, substantia nigra, and red nucleus on both side.