Diagnosis and management of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD) is rare, occurring in one per million people. It is difficult to eradicate from contaminated instruments, and so is important to recognise for infection control reasons. As well, there is much interest in possible changes in the epidemiology of this disease, and so familiarity is necessary among all physicians. Sporadic CJD presents in the young-old with a rapidly progressive dementia, while variant CJD presents in younger patients, initially with psychiatric symptoms, Electroencephalography, MRI and 14-3-3 protein testing are all helpful in the diagnostic process. There is no recognised therapy as yet.