Among the autosomal recessive cerebellar ataxias (ARCA), Friedreich’s ataxia (FRDA) is the most common subtype, followed by ataxia-telangiectasia (AT), and ataxia oculomotor apraxia type 1 (AOA1) and type 2 (AOA2) [7]. The gene discussed is APTX; the disease is ataxia telangiectasia.