Abstract

Aicardi-Goutieres Syndrome (AGS) is an autosomal ressesive genetic disorder, clinically characterized by microcephaly, cerebral atrophy and white matter abnormalities, intellectual disabilty and motor reterdation, increased cerebrospinal fluid (CSF), lymphocytosis and interferon-alpha (IFN) in blood, intracranial calsification especially at basal ganglia. We should keep in mind Aicardi-Goutieres Syndrome for differential diagnosis while investigating a microcephalic newborn presented with intracrianal calsifications and seizure.