Surveillance is how governments learn how many people have prion disease, whether a new form has appeared and whether a cluster needs investigation. This page summarises the systems described by their own operators. It is information for policy readers and not a legal summary. Rules change, so confirm them with the relevant authority.
How does Europe monitor CJD?
The European Creutzfeldt-Jakob Disease Surveillance Network, EuroCJD, was set up in 1993 by seven countries. Its members are experts nominated by national competent bodies in EU and EEA countries. The EU regulated vCJD surveillance in 2000, and responsibility passed to the European Centre for Disease Prevention and Control (ECDC) in 2007.
The network identifies and characterises vCJD cases, standardises diagnosis, tracks risk factors and trends, and looks for links between human and animal prion diseases. Member States report new incident cases to ECDC’s TESSy database, where cases are classed as probable under the EU case definition and confirmation depends on post-mortem neuropathology. ECDC coordinates the network through a contractor, the National CJD Research and Surveillance Unit in Edinburgh. A page for the Edinburgh unit itself could not be opened for this brief, because it redirected to a login.
How does the United States monitor CJD?
The National Prion Disease Pathology Surveillance Center at Case Western Reserve University is described as the only centre of its kind in the country. It coordinates autopsies and neuropathology for suspected cases, offers diagnostic testing, publishes annual national data on referrals and confirmed cases, and is supported by the CDC. The CDC reports about 500 to 600 cases a year.
Reporting duties are set by states. Two examples show the range:
| State | Provider reporting | Source |
|---|---|---|
| Texas | Reportable within one week | Texas DSHS |
| Washington | Providers and facilities within 3 business days; laboratories within 2 business days | Washington Department of Health |
Texas also advises confirming the diagnosis by autopsy where possible, and the CDC encourages listing prion disease on the death certificate when there is no alternative diagnosis. These two states do not show the rule in the other states. A national notifiable-disease status was not established in the pages read.
What is not known?
- How many cases surveillance misses. Neither operator’s page read for this brief gives a completeness figure.
- How case definitions, autopsy rates and legal reporting differ across all countries. Only the European network and two US states were read.
- How data from different systems compare. The EU counts probable cases at report, and the US centre reports referral and confirmed classifications, so figures are not directly interchangeable.
See the surveillance centres list in the Atlas, the evidence for regulators page, the regulations list, data protection and the policy overview.
Sources for this page
- ECDC: European Creutzfeldt-Jakob Disease Surveillance Network (EuroCJD)
- National Prion Disease Pathology Surveillance Center, Case Western Reserve University
- Texas DSHS: Creutzfeldt-Jakob disease reporting
- Washington State Department of Health: Prion disease, notifiable conditions
- CDC: About Creutzfeldt-Jakob disease