## Abstract Aicardi‐Goutières syndrome (AGS) presents as a severe autosomal recessively inherited neurological brain disease. Clinical and neurological manifestations closely resemble those of congenital viral infection and are generally attributed to a perturbation of innate immunity including a l
✦ LIBER ✦
Interferon and Aicardi-Goutières syndrome
✍ Scribed by PIERRE LEBON; JEAN FRANÇOIS MERITET; ANNE KRIVINE; FLORE ROZENBERG
- Book ID
- 114465026
- Publisher
- Elsevier Science
- Year
- 2002
- Tongue
- English
- Weight
- 123 KB
- Volume
- 6
- Category
- Article
- ISSN
- 1090-3798
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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 Gout
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