Typical magnetic resonance imaging (MRI) patterns of intermediate Alexander disease

In the intermediate (juvenile) form of Alexander disease, there are significant abnormalities in the supratentorial and infratentorial spaces (features of cerebral and bulbospinal disease or type I and type II). The frontal white matter is involved (A-D, arrows) along with the basal ganglia (B, arrowheads) and periventricular rim (not shown). Mass lesions (C, D, arrowheads) and/or atrophy are present in the brainstem. Cerebellar abnormalities may also be present involving the white matter (arrow) as well as the dentate nuclei. Various structures enhance post-gadolinium.