Indeed, it has been hypothesized that cystic leukoencephalopathy without megalencephaly, caused by loss of RNASET2 function, is a lysosomal storage disease, with accumulation of excess RNA eliciting an autoimmune and neuroinflammatory response akin to what is observed in CMV and AGS. Here, RNASET2 is linked to Aicardi-Goutieres syndrome.