An 11 year old boy came with complaints of delayed milestones and behavioural abnormalities since four years of age He has hyperpigmentation since two years, diminution of vision since two months and multiple episodes of convulsions since 15 days.
Confluent symmetric white matter T2/FLAIR hyperintensity is seen in both parieto-occipital and temporal lobes involving deep periventricular white matter. This reaches upto the subcortical U fibres, splenium and posterior body of corpus callosum, posterior limb of bilateral internal capsules and posterior aspect of bilateral external capsules, both cerebral peduncles and extends inferiorly along the corticospinal tracts. There is linear post contrast enhancement along the advancing edge of the white matter hyperintensity.