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December 1977

Hallervorden-Spatz Syndrome

Author Affiliations

From the Neuropathology Laboratory (Drs Vakili and Zeman), and the Departments of Pediatric Neurology (Dr Drew) and Nuclear Medicine (Dr Von Schuching), Indiana University Medical Center, Indianapolis, and the Analytical Chemistry Division (Mr Becker), National Bureau of Standards, Washington, DC. Dr Vakili is now with the Department of Pathology, Indiana University Medical Center.

Arch Neurol. 1977;34(12):729-738. doi:10.1001/archneur.1977.00500240017003

• Two siblings with Hallervorden-Spatz syndrome showed striking homotypism and homochronism. Neuropathologic examination and electron microscopic studies were done; neutron activation analysis showed an increase in the uptake of iron in the basal ganglia. Of particular relevance is the application of radioactive iron studies in the clinical course of this syndrome. These studies disclosed an increase in the uptake of iron in the area of the basal ganglia in one sibling and in another isolated patient. This procedure will be helpful toward the clinical diagnosis.

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