Aicardi-Goutières-syndrome (AGS) comprises a group of monogenetic disorders characterized by aberrant immune activation (1). The spectrum of neurological features includes acquired microcephaly with cerebral atrophy, basal ganglia calcification, leukodystrophy, chronic cerebrospinal lymphocytosis, and/or elevated concentration of type I interferons (IFNs) resulting in high morbidity and mortality (2). AGS can be caused by mutations in several genes involved in sensing or modifying nucleic acids.
