Aicardi-Goutières syndrome.

  title={Aicardi-Gouti{\`e}res syndrome.},
  author={Françoise Gouti{\`e}res},
  journal={Brain & development},
  volume={27 3},
Aicardi-Goutieres syndrome is a familial progressive early onset encephalopathy with basal ganglia calcifications, chronic CSF lymphocytosis and high level of interferon-alpha in CSF. Cutaneous necrotic lesions and the neuropathological aspect of microangiopathy and microinfarctions suggest a vascular process in relation to elevated interferon-alpha. A genetic defect in the regulation of its synthesis may be the causal factor of the disorder.