Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Hallervorden and Spatz first described the disease, in 1922, as a form of familial brain degeneration characterized by iron deposition in the brain.
The term neurodegeneration with brain iron accumulation type 1 (NBIA-1), instead of HSD, eventually came to be used for this condition,although the most recent term for the disorder is pantothenate kinase-associated neurodegeneration (see the image below). (See Etiology).
The onset of symptoms most commonly occurs in late childhood or early adolescence. The classic presentation is in the late part of the first decade or the early part of the second decade, between ages 7 and 15 years. However, the disease has been reported in infancy, and cases with adult onset have also been described. (See Presentation and Workup.)