The recognition that mutations of the pantothenate kinase 2 (PANK2) gene are the commonest cause of NBIA [10], also known as pantothenate kinase-associated neurodegeneration (PKAN), NBIA type 1 or HSS, has significantly advanced our understanding of this disorder [11]. This evidence concerns the gene PANK2 and pantothenate kinase-associated neurodegeneration.