SETX and ataxia telangiectasia: Indeed, a juvenile onset (usually before the 2nd decade) is more typical in AOA1, AOA2, and Ataxia-Telangiectasia (AT) [27, 38], while adult-onset ataxias with this MRI pattern mostly include SCA6, SCA8, SCA17 and SCAR10 [9, 28, 39–41].