The California Creutzfeldt-Jakob Disease (CJD) Surveillance Project,
in collaboration with the U.S. Centers for Disease Control and
Prevention (CDC) and the California Department of Public Health
(CDPH), conducts enhanced surveillance for CJD, variant CJD (vCJD)
and other Transmissible Spongiform Encephalopathies (TSE) in the
state of California. As of June 2007 CDPH added TSEs, including
CJD, to the list of reportable diseases. We support these surveillance
efforts, with additional follow up effort on cases afflicting
patients less than 55 years old.
A definitive diagnosis of a TSE can only be made by neuropathological
examination of brain tissue obtained through biopsy or autopsy.
Although CJD may be suspected on the basis of clinical symptoms
and the results of other diagnostic tests, none are confirmatory
and the diagnosis is difficult to make. Because these diseases
are rare and difficult to diagnose, the CDPH’s
surveillance case definition requires that the diagnosis
of CJD be made either by a neurologist or confirmed by biopsy
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CJD epidemiologic data to learn more about these illnesses,
monitor trends and detect novel forms of disease or transmission.
• Monitor ongoing CJD testing in collaboration
with the National Prion Disease Pathology Surveillance Center
(NPDPSC) at Case Western Reserve University and the CDC.
• Support CDPH reporting efforts.
• Review clinical and neuropathology records in
persons younger than 55 years old with symptoms and test results
suggestive of CJD.
• Contact medical providers and public health officials
in cases with possible environmental or unusual sources of
• Be an educational resource for clinicians, local
health departments and communities in California.
• Improve the accuracy of diagnoses through increased
autopsy and biopsy rates in cases of suspected TSE in California.