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MRI of the brain demonstrates extensive increased T2 signal in the white matter extending from the centrum semiovale, down the posterior limb of the internal capsule and through the anterolateral pons, as well as the white matter of the cerebellar hemispheres.
Additionally low signal is seen in the deep grey matter structures, particularly involving the thalami, globus pallidi and dentate nuclei, as well as the red nuclei and substantia nigra.
Widespread cerebral and cerebellar volume loss is also present with marked thinning of the corpus callosum, without T2 signal change.