Prion Disease

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.