Germline mutations in the DAX-1 gene are responsible for two conditions: dosage-sensitive sex reversal (DSS) which occurs in XY individuals, and another condition called adrenal hypoplasia congenita (AHC) is characterized by adrenal insufficiency and hypogonadotropic hypogonadism in males [69,70]. This evidence concerns the gene NR0B1 and alternating hemiplegia of childhood.