Incidence of Sporadic Creutzfeldt-Jakob Disease in the Emergency Department

IntroductionCreutzfeldt-Jakob disease (CJD) is a fatal prion disorder marked by rapidly progressive dementia and neuropsychiatric features. Epidemiological data in Latin America are limited, and no incidence studies exist in Mexico. This study sought to quantify CJD incidence in a neurological reference center.MethodsA cross-sectional study was conducted at the National Institute of Neurology and Neurosurgery, Mexico City, during 2023-2024. Clinical and paraclinical findings were reviewed. Incidence was calculated per 1000 emergency visits, with relative risk (RR) and 95% confidence intervals.ResultsAmong 19 003 patients, 15 fulfilled probable CJD criteria. Incidence was 0.55/1000 in 2023 and 1.01/1000 in 2024 (RR 1.83; 95% CI 0.63-5.35; <i>P</i> = .309). Cognitive and behavioral symptoms predominated (psychosis in 68%). MRI revealed abnormalities in all cases, most often cortical ribboning (93%).ConclusionThis first incidence study highlights the presence of CJD in specialized Mexican centers, underscoring the need for systematic surveillance and improved diagnostic access.