Aicardi-Goutieres Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** A genetically heterogeneous condition characterized by clinical features and onset that may vary significantly. It is characterized in its most severe form by cerebral atrophy, leukodystrophy, intracranial calcifications, chronic cerebrospinal fluid (CSF) lymphocytosis, and increased concentrations of CSF alpha-interferon.