TREX1 and Aicardi-Goutieres syndrome 1: Herein we report the clinic-neuroradiological features of two patients presenting with AGS-like and CADASIL-like phenotypes, who harbored two different monoallelic, likely pathogenic TREX1 missense variants affecting the catalytic domain (c.407C>T − p.A136V and c.520A>G − p.R174G).