Colorado surveillance program for chronic wasting disease transmission to humans - Lessons from 2 highly suspicious but negative cases

Objective: To describe 2 patients with rapidly progressive dementia and risk factors for exposure to chronic wasting disease (CWD) in whom extensive testing negated the possible transmission of CWD. Design/Methods: We describe the evaluation of 2 young adults with initial exposure histories and clinical presentations that suggested the possibility of CWD transmission to humans. Patients: A 52-year-old woman with possible laboratory exposure to CWD and a 25-year-old man who had consumed meat from a CWD endemic area. Interventions: Clinical evaluation, neuropathological examination, and genetic testing. Results: Neuropathological and genetic assessment in the 2 patients proved the diagnoses of early-onset Alzheimer disease and a rare genetic prion disease. Conclusion: No convincing cases of CWD transmission to humans have been detected in our surveillance program.