Hallervorden-Spatz disease
Correct Answer: Is an iron storage disorder in brain
Description: It's a rare disorder characterized by progressive extrapyramidal dysfunction and dementiaA form of familial (inherited recessively-linked to chromosome 20) brain degeneration characterized by iron deposition in the brainA mutation in the pantothenate kinase gene has been describedMRI shows "eye-of-the-tiger sign"The disease is relentlessly progressive and the affected individuals typically die in 2nd or 3rd decade(ref:Harrison 18th /e p2251)
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