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Hallenvorden-Spatz disease |
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eMedicine: Background: Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Onset is most commonly in late childhood or early adolescence, but cases with adult onset have been described (Jankovic et al, 1985). The disease can be familial or sporadic. When familial, it is inherited recessively and has been linked to chromosome 20 (Taylor et al, 1996). Recently, a mutation in the pantothenate kinase (PANK2) gene on band 20p13 has been described in patients with typical HSD (Zhou et al, 2001).
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