MRI findings in CJD. (A,B) A patient with probable variant CJD and three common MRI patterns in sporadic CJD: (C,D) predominantly subcortical, (E,F) both cortical and subcortical, and (G,H) predominantly cortical. Note that in sporadic CJD the abnormalities are always (C,E,G) more evident on DWI than (D,F,H) on FLAIR images. (A,B) A 21-year-old woman with probable variant CJD with MRI showing bilateral thalamic hyperintensity in the mesial pars (mainly dorsomedian nucleus) and posterior pars (pulvinar) of the thalamus, the so-called “double hockey stick sign.” Also note the “pulvinar sign,” with the posterior thalamus (pulvinar) being more hyperintense than the anterior putamen. The three sporadic CJD cases are pathology-proven. (C,D) A 52-year-old woman with MRI showing strong hyperintensity in bilateral striatum (solid arrows, both caudate and putamen) and slight hyperintensity in mesial and posterior thalamus (dotted arrow). (E,F) A 68-year-old man with MRI showing hyperintensity in bilateral striatum (note anteroposterior gradient in the putamen, which is commonly seen in CJD), thalamus, right insula (dotted arrow), anterior and posterior cingulate gyrus (arrow, L > R), and left temporal-parietal-occipital junction (arrow). (G,H) A 76-year-old woman with MRI showing diffuse hyperintense signal mainly in bilateral parietal and occipital cortex, right posterior insula (dashed arrow) and left inferior frontal cortex (arrow), but no significant subcortical abnormalities. CJD, Creutzfeldt-Jakob disease; MRI, magnetic resonance imaging; DWI, diffusion-weighted imaging; FLAIR, fluid-attenuated inversion recovery.