How to improve the clinical diagnosis of Creutzfeldt–Jakob disease

Abstract

evidence, together with follow-up of the patients still living and those who died without autopsy, revealed a patients initially notified as suspected Creutzfeldt-Jakob disease to a Surveillance Unit in Go ttingen, Germany. Six broad range of other diagnoses. In the younger age groups, the commonest were chronic inflammatory patients were diagnosed as having genetic prion disease by blood analysis and were excluded from the study. After diseases including Hashimoto encephalitis, whilst rapidly progressive Alzheimer's disease was most common in the examination and review of the remaining 358, 193 were classified as probable Creutzfeldt-Jakob disease. How-older age groups. The presence of 14-3-3 protein in the CSF discriminated better between Creutzfeldt-Jakob ever, autopsy revealed that five of the 193 did not have Creutzfeldt-Jakob disease (four cases, Alzheimer's disease and other rapidly progressive dementias than did the EEG pattern or the MRI. The inclusion of this CSF disease; one case, cerebral lymphoma). Of the 54 patients classified as possible Creutzfeldt-Jakob disease, 10 had protein in the criteria of Masters and colleagues (Ann Neurol 1979; 5: 177-88) improves the accuracy and another diagnosis made at autopsy. Two of the 111 cases originally classified as having other diseases were found confidence in the clinical diagnosis of Creutzfeldt-Jakob disease. to have Creutzfeldt-Jakob disease on autopsy. Autopsy