The most prevalent ARCAs associated with dystonia include Friedreich ataxia (FXN), ataxia-telangiectasia (ATM), ataxia with isolated vitamin E deficiency (TTPA),POLG-related disorders, ataxia with oculomotor apraxia types 1, 2, and 4 (APTX,SETX, andPNKPrespectively),PRRT2-associated disorders, and autosomal recessive spinocerebellar ataxia type 5 or Galloway–Mowat syndrome (WDR73).3 The gene discussed is SETX; the disease is Ataxia.