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National Prion Disease Pathology Surveillance Center

UCSF Memory and Aging Center

For Health Care Providers | For Local Health Departments | Background | How is CJD Diagnosed? | Resources

Health care providers:

Help us effectively monitor for cases of TSE and perform follow-up investigation on CJD cases with possible public health importance, confirm clinical cases through arranging autopsies and clearly indicate CJD diagnoses on death certificates.


Report all human cases of CJD or TSE to your local health department.

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 (see Background) and the results of other diagnostic tests (see How is CJD diagnosed?), none are confirmatory and the diagnosis is difficult to make. Because these diseases are rare and difficult to diagnose, the CA Department of Public Health’s (CDPH) surveillance case definition requires that the diagnosis of CJD be made either by a neurologist or confirmed by biopsy or autopsy.

If you are unsure of the diagnosis (See background information for more on epidemiology and diagnosis) you may want to consult a neurologist. The UCSF Memory and Aging Center is the only center with an ongoing treatment trial for CJD.


Alert us for suspected vCJD or iatrogenic CJD:

Contact us by email, cjd@ceip.us. We are particularly interested if you are caring for a CJD patient with any of the following:

• Younger than 55 years of age

• Suspected diagnosis of variant CJD (see WHO vCJD diagnostic criteria)

• History of neurosurgical procedure or receiving cadaver-derived human pituitary growth hormone, dura mater grafts, or corneal grafts (Note: for these possible iatrogenic cases, your local public health department must also be notified.)

• History of deer or elk meat consumption from states endemic for chronic wasting disease among deer and elk (CO, WY, NE, NM, IL, KS, NY, SD, UT, WI, or WV)


Arrange for an autopsy on all suspected CJD cases:
Confirming the histopathology diagnosis of CJD cases will improve the specificity of these diagnoses and help better understand this prion disease. The National Prion Disease Pathology Surveillance Center (NPDPSC) can assist in coordinating autopsies.


Clearly indicate the diagnoses of CJD on death certificates:
By having the CJD ICD-10 code A81.0 (or ICD-9 code 046.1) on the death certificate, you will help improve our surveillance efforts through mortality data review.


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