Analysis of EEG and CSF 14-3-3 protein as aids to the diagnosis of Creutzfeldt-Jakob disease

Objective: To improve diagnostic criteria for sporadic Creutzfeldt-Jakob di sease (CJD). Methods: Pooled data on initial and final diagnostic classific ation of suspected CJD patients were accumulated, including results of inve stigations derived from a coordinated multinational study of CJD. Prospecti ve analysis for a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoa ssay were conducted. Results: Data on 1,003 patients with suspected CJD wer e collected using a standard questionnaire. After follow-up was carried out , complete clinical data and neuropathologic diagnoses were available in 80 5 cases. In these patients, the sensitivity of the detection of periodic sh arp wave complexes in the EEG was 66%, with a specificity of 74%. The detec tion of 14-3-3 proteins in the CSF correlated with the clinical diagnosis i n 94% (sensitivity). The specificity (84%) was higher than that of EEG. A c ombination of both investigations further increased the sensitivity but dec reased the specificity. Conclusions: Incorporation of CSF 14-3-3 analysis i n the diagnostic criteria for CJD significantly increases the sensitivity o f case definition. Amended diagnostic criteria for CJD are proposed.