Aicardi-Goutières syndrome (AGS).

@article{Stephenson2008AicardiGoutiresS,
  title={Aicardi-Gouti{\`e}res syndrome (AGS).},
  author={John B.P. Stephenson},
  journal={European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society},
  year={2008},
  volume={12 5},
  pages={
          355-8
        }
}
In 1984, Jean Aicardi and Françoise Goutières described 8 children showing both severe brain atrophy and chronic cerebrospinal fluid lymphocytosis, with basal ganglia calcification in at least one member of each affected family. The course was rapid to death or a vegetative outcome. Aicardi and Goutières correctly predicted that the disorder would be genetic, but emphasised that "some features, especially the pleocytosis, may erroneously suggest an inflammatory condition". The increased… CONTINUE READING
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