Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease.

Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis.

Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%, 58.6%, and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP) test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%, 91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%).

Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information.