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Lessons From 2 Highly Suspicious but Negative Cases

C. Alan Anderson, MD; Patrick Bosque, MD; Christopher M. Filley, MD; David B. Arciniegas, MD; B. K. Kleinschmidt-DeMasters, MD; W. John Pape, BS; Kenneth L. Tyler, MD

Arch Neurol. 2007;64(3):439-441.

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.

Author Affiliations: Departments of Neurology (Drs Anderson, Bosque, Filley, Arciniegas, Kleinschmidt-DeMasters, and Tyler), Psychiatry (Drs Anderson, Filley, and Arciniegas), Pathology (Dr Kleinschmidt-DeMasters), Medicine (Dr Tyler), Microbiology (Dr Tyler), and Immunology (Dr Tyler); University of Colorado School of Medicine, Denver; Denver Veterans Affairs Medical Center, Denver (Drs Anderson, Filley, Arciniegas, and Tyler); Denver Health Medical Center, Denver (Dr Bosque); and Colorado Department of Public Health and Environment, Denver (Mr Pape).

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