Overall mortality rate is high in AGS patients, and it is usually related to genotype: 34% of cases carrying RNASEH2A, RNASEH2C, and TREX1 pathogenic variants died, the majority of whom (81%) by the age of 10 years old; otherwise, only the 8% of patients carrying RNASEH2B pathogenic variants may die (146). This evidence concerns the gene TREX1 and Aicardi-Goutieres syndrome.