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: notifiable to local health jurisdiction within 3 business days.
  • Health care facilities: notifiable to local health jurisdiction within 3 business days.
  • Laboratories: no requirements for reporting.
  • 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.