Cases of aphasia or neglect due to Creutzfeldt-Jakob disease

Two patients with CJD whose deficits are attributable to damage to selectiv e cognitive mechanisms are described. One patient presented with 'pure word deafness' with severe anemia and progressed to Global aphasia. EEG and SPE CT showed left cortical abnormalities. The other presented with progressive left neglect and digressive speech with meager interpretive content, but n ever developed aphasia. EEG showed right cortical abnormalities. These case s provide evidence that CJD can begin focally and unilaterally. Several cas es of progressive aphasia due to CJD have been described, but this may be t he first reported case of progressive right hemisphere dysfunction due to C JD.