We describe the clinical and neuro radiological features of eleven
patients with Aicardi-Goutieres syndrome, a rare and severe progressive
encephalopathy with onset in the first year of life. The syndrome is
autosomal recessive with varying clinical presentation and course. Our
patients were studied by CT and MR imaging and findings were in
agreement with literature reports. Calcification of the basal nuclei was
found in 100\% of cases and six patients presented a progressive
increase in the number and size of the calcifications which were
bilateral and largely symmetrical. White matter changes were seen in
76\% of cases without a specific pattern of distribution. Early
neuroradiological diagnosis of suspect Aicardi-Goutieres syndrome is
established by ruling out other pathological processes and the site and
features of the calcifications rather than the white matter changes is
important to then search for typical CSF changes
