In addition to the typical leukoencephalopathy and intracranial calcifications, the radiological spectrum of AGS has been widened to include RNASEH2B-associated delayed myelination, TREX1 deep WM cysts, widespread calcifications involving the basal ganglia, thalami, and deep WM [17], and striatal necrosis associated with ADAR1 [18,19]. This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.