ABSTRACT
Pantothenate kinase-associated neurodegeneration (PKAN) is a genetic movement disorder that accounts for the majority of cases of what was formerly called Hallervorden-Spatz syndrome. In 1922, Hallervorden and Spatz reported an autosomal recessive neurodegenerative disorder with retinitis pigmentosa and high levels of iron in brain. Since then, the diagnosis has been expanded to encompass a heterogeneous group of disorders that share the feature of high brain iron. To discredit Hallervorden and Spatz for their objectionable actions during World War II, the eponym has been abandoned and replaced with the term ‘‘neurodegeneration with brain iron accumulation’’ (NBIA). NBIA includes neurological disorders in which basal ganglia iron levels are high. PKAN is one form of NBIA.
