Characterising the uncommon corticobasal syndrome presentation of sporadic Creutzfeldt-Jakob disease

sCJD should be suspected in patients presenting with CBS when clinical progression is rapid and accompanied by DWI abnormalities, even without cerebrospinal fluid 14-3-3 protein detection and electroencephalographic periodic sharp wave complexes. We propose the addition of rapid (<12months) progression to akinetic-mutism or death and DWI abnormalities as exclusions in future CBD diagnostic criteria.