AGS caused by mutations in TREX1, RNASEH2A, and RNASEH2C usually presents early in the neonatal period as “pseudo-TORCH” manifestations with severe neurological dysfunction in the form of progressive microcephaly, spasticity, psychomotor retardation, and may lead to death [57]. The gene discussed is TREX1; the disease is Aicardi-Goutieres syndrome.