MRI of Heidenhain variant Creutzfeldt-Jakob disease

Head MRI of a 65-year-old patient with biopsy-proven sporadic CJD (Heidenhain variant).

(Image A) A T2 MRI that shows essentially normal signal intensity within the basal ganglia and throughout the brain.

(Image B) A proton density MRI that shows increased cortical signal intensity within the right frontal lobe and bilateral medial occipital lobes.

(Image C) A DWI that reveals more conspicuous involvement of the right frontal lobe and more extensive hyperintensities of the bilateral occipital lobes with no involvement of the basal ganglia.

(Image D) A DWI rostral to the basal ganglia that shows extensive cortical involvement throughout the brain with more prominence posteriorly, as may be expected with the Heidenhain variant.