Preferred Label : Aicardi-Goutières syndrome;
ICD-11 definition : Aicardi-Goutières syndrome is an inherited, subacute encephalopathy characterised
by the association of basal ganglia calcification, leukodystrophy and cerebrospinal
fluid (CSF) lymphocytosis.;
ICD-11 synonym : Encephalopathy with basal ganglia calcification; Encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal
fluid;
Origin ID : 2025818872;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
Aicardi-Goutières syndrome is an inherited, subacute encephalopathy characterised
by the association of basal ganglia calcification, leukodystrophy and cerebrospinal
fluid (CSF) lymphocytosis.