Friedreich ataxia (FRDA) is an autosomal recessive inherited multisystem disease, the classical neurological features of which reflect a specific neuropathology, characterized by marked differences in the vulnerability of neuronal systems.1,2 First described in 1863 by Nikolaus Friedreich, a German physician, the genetic cause of Friedreich's ataxia was discovered in 19963: a homozygous expansion of a guanine-adenine-adenine (GAA) trinucleotide repeat in intron 1 of the frataxin gene (FXN), or rarely, a point mutation or large deletion in one FXN allele and GAA expansion in the other. The gene discussed is FXN; the disease is Friedreich ataxia.