Hallervorden-Spatz syndrome.
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.
D001369:Axons / Q000648:ultrastructure; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D005917:Globus Pallidus / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007501:Iron / Q000378:metabolism; D008297:Male; D008928:Mitochondria / Q000648:ultrastructure; D009186:Myelin Sheath / Q000648:ultrastructure; D009457:Neuroglia / Q000648:ultrastructure; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000378:metabolism / Q000473:pathology*
D007501:Iron