Aicardi-Goutieres syndrome (Monarch Disease Ontology term MONDO_0018866) (AGS) is an inherited, subacute encephalopathy characterized by the association of basal ganglia calcification, leukodystrophy and cerebrospinal fluid (CSF) lymphocytosis. Also known as: Aicardi Goutieres syndrome, Aicardi-Goutières Syndrome, Cree encephalitis, encephalopathy with basal ganglia calcification, encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal fluid.