Aicardi-Goutieres syndrome in siblings

Two siblings with familial encephalopathy, calcification of the basal gangl ia, and cerebrospinal fluid lymphocytosis, constituting the triad of Aicard i-Goutieres syndrome, are reported. This syndrome resembles congenital intr auterine infections, which must be meticulously excluded. Aicardi-Goutieres syndrome is extremely rare and is being reported from the Arab world for t he first time to our knowledge.