Two patients with CJD whose deficits are attributable to damage to selective cognitive mechanisms are described. One patient presented with 'pure' word deafness with severe anomia and progressed to Global aphasia. EEG and SPECT showed left cortical abnormalities. The other presented with progressive left neglect and digressive speech with meager interpretive content, but never developed aphasia. EEG showed right cortical abnormalities. These cases provide evidence that CJD can begin focally and unilaterally. Several cases of progressive aphasia due to CJD have been described, but this may be the first reported case of progressive right hemisphere dysfunction due to CJD.