Aicardi-Goutières syndrome

What is Aicardi-Goutières syndrome?

An inherited, subacute encephalopathy characterised by the association of basal ganglia calcification, leukodystrophy and cerebrospinal fluid (CSF) lymphocytosis.

Other condition names

  • Encephalopathy with basal ganglia calcification
  • Encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal fluid

Inheritance type

Autosomal dominant, Autosomal recessive


  • Europe: 1-5 in 10,000
  • Worldwide: Unknown

Age of Onset

  • Infancy
  • Neonatal
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