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  • Title: Aicardi-Goutières syndrome.
    Author: Goutières F.
    Journal: Brain Dev; 2005 Apr; 27(3):201-6. PubMed ID: 15737701.
    Abstract:
    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.
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