Cause: Prions, or “proteinaceous infectious particles” in which normal cellular prion proteins in the brain fold into abnormal, pathologic forms, causing a fatal neurodegenerative disease.
Illness and treatment: About 85% of CJD cases are sporadic (sCJD) while 15% are inherited. Sporadic CJD is characterized by rapidly progressing dementia, poor balance, visual changes and/or muscle jerks. Treatment is supportive.
Sources: The cause of sporadic CJD is not known. In 1996, a new variant of CJD (vCJD) recognized in the United Kingdom was associated with cattle infected with a related infection (“mad cow disease”). To date, no cases of vCJD have been acquired in the United States.
Prevention: There are no specific precautions.
Recent Washington trends: During 2011-2020, 9-19 cases were reported each year.
Purpose of Reporting and Surveillance
- To monitor trends in the epidemiology of human prion diseases in Washington State.
- To maximize laboratory confirmation of suspected cases and facilitate testing
- To promote awareness of available resources.
- To detect the emergence of variant Creutzfeldt-Jakob disease or novel prion diseases in the United States.
- To prevent potential iatrogenic transmission.
Legal Reporting Requirements
- Health care providers and Health care facilities: notifiable to local health jurisdiction within 3 business days.
- Laboratories: notifiable to local health jurisdiction within 2 business days; submission on request – specimen associated with positive result, within 2 business days.
- Local health jurisdictions: notifiable to the Washington State Department of Health (DOH) Communicable Disease Epidemiology (CDE) within 7 days of case investigation completion or summary information required within 21 days.
Infection Control Key Points (PDF)
Information for Medical Providers (PDF)
Funeral/Crematory Practitioner Recommendations (PDF)
Human Prion Disease Annual Surveillance Data (PDF)
Human Prion Disease 10 Year Surveillance Data (PDF)