LAUSR

Key Points Question How sensitive and specific are the 2017 revised International Creutzfeldt-Jakob disease Surveillance Network diagnostic criteria for the diagnosis of sporadic Creutzfeldt-Jakob disease? Findings In this diagnostic study of 647 individuals conducted by a multinational team of surveillance experts, the revised diagnostic criteria, which incorporate cerebrospinal fluid real-time quaking-induced conversion assay and cortical ribboning on brain magnetic resonance imaging, were significantly more sensitive than previous criteria with no loss of specificity. Meaning These results suggest that the revised diagnostic criteria achieve superior diagnostic sensitivity and maintain a high degree of specificity compared with previous criteria, enhancing diagnostic accuracy for clinical care and surveillance.