Early-onset recessive ataxia (AOA1 or EAOH [MIM 208920]) is a progressive syndrome associated with hypoalbuminemia and elevated levels of cholesterol and is caused by mutations in APTX (aprataxin).1–3 Autosomal-recessive spinocerebellar ataxia 1 (AOA2 or SCAR1 [MIM 606002]), a progressive ataxia,4 occurring later than AOA1, is characterized by increased alpha-fetoprotein levels and is caused by mutations in SETX (senataxin).5 Here, SETX is linked to ataxia, early-onset, with oculomotor apraxia and hypoalbuminemia.