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Late MR imaging study of a patient with presumed juvenile Alexander disease, obtained at the age of 10 years.
A and B, There is extensive white matter involvement with frontal preponderance (A). The basal ganglia are dark and atrophic on T2-weighted images (A). A thin periventricular rim of low signal intensity is just visible (arrows, A). After contrast administration, enhancement of the entire cerebellar surface and dentate nucleus is seen (B).
