Aicardi-Goutières syndrome
What is Aicardi-Goutières syndrome?
A rare inherited subacute encephalopathy characterized by the variable association of basal ganglia calcification, leukodystrophy, cerebrospinal fluid (CSF) lymphocytosis and evidence of enhanced type I interferon signaling in blood and CSF.
Other condition names
- Encephalopathy with basal ganglia calcification
- Encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal fluid
Inheritance type
Autosomal dominant, Autosomal recessive
Prevalence
- Europe: 1-5 in 10,000
- Worldwide: Unknown
Age of Onset
- Infancy
- Neonatal
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.