FXN and Friedreich ataxia: The only exception isFriedreich’s ataxia (FRDA), in which reduction of frataxin leads to changesin cellular iron homeostasis.8 However, even in NBIA, iron accumulation is rarely associatedwith genes directly involved in iron metabolism.2 More commonly, iron accumulation occursindirectly, especially in connection with microgliosis and inflammation.9 Pronounced microgliosis hasbeen described in spinocerebellar ataxia type 1 (SCA1).10 Thus, iron accumulation may also play arole in the pathogenesis of hereditary ataxias other than FRDA.