Please report all clinical cases diagnosed by a neurologist or confirmed by tissue pathology of human TSEs including:

• Sporadic CJD (sCJD), also known as Classic Creutzfeldt-Jakob Disease (CJD)
• Familial CJD (fCJD) or inherited CJD
• Iatrogenic CJD (iCJD)
• Variant Creutzfeldt-Jakob Disease (vCJD)
• Gerstmann-Straussler-Scheinker Syndrome (GSS)
• Fatal Familial Insomnia (FFI)

Case Notification

The National Prion Disease Pathology Surveillance Center (NPDPSC) based at Case Western Reserve University performs diagnostic testing for CJD.

A positive 14-3-3 and tau protein result in CSF is NOT definitive for the diagnosis of prion disease. A positive result can occur in non-prion diseases or in a sample that is contaminated by blood.

When you receive a positive CSF test result for the 14-3-3 and tau protein, contact the ordering physician and ascertain whether a neurologist thinks that the patient may have CJD. If an exact diagnosis has not been made, but the patient has fully recovered, a prion disease has been ruled out. If the neurologist no longer suspects CJD, no report is necessary. If, however, the diagnosis of CJD is still being entertained follow up of the suspect case should continue.

If the patient is still alive, the local health department can play an important role in getting a definitive diagnosis by alerting clinicians that free shipping and testing of autopsy tissue can be arranged through the NPDPSC.

If you are notified of a neurologist-diagnosed CJD case, you receive an autopsy/biopsy confirmed lab report from the National Prion Disease Pathology Surveillance Center, or receive a death certificate listing CJD as a cause of death, a CJD case report form should be filled out.

Reporting a Case

The case report form is available on the CA Department of Public Health Communicable Disease Control form’s website. The case report form should be sent to CDPH’s Surveillance and Statistics section or filled out in CalREDIE. If available, hospital discharge summaries, neurology notes, MRI and EEG reports, CSF and brain biopsy/autopsy lab results, and a copy of the death certificate should be attached.

Paper copies of case report forms should be mailed to

California Department of Public Health
Center for Infectious Diseases
Division of Communicable Disease Control
Infectious Diseases Branch
Surveillance and Statistics Section
MS 7306, P.O. Box 997377
Sacramento, CA 95899-7377