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Disease Name: CJD, vCJD


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Please review the Idaho Reportable Disease Rules (IDAPA 16.02.10) for the most up-to-date information.


Overview / Case Definition

Sporadic CJD (sCJD): Approximately 85% of CJD cases are classified as sporadic. sCJD cases arise spontaneously and the cause is unknown. The U.S. rate of occurrence is ~1 to 1.5 per million population per year.

Familial CJD (fCJD): Approximately 10% of CJD cases are associated with a mutation of the PRNP gene and are hereditary or familial, usually in an autosomal dominant pattern, so other family members with the disease are usually identified.

Iatrogenic CJD (iCJD): Approximately 5% of CJD cases result from accidental medical transmission. Sources include contaminated neurosurgical instruments, cornea or dura mater transplants, and human-derived pituitary growth hormones.

Variant CJD (vCJD, also seen as new variant CJD [nvCJD]): vCJD was first recognized in the United Kingdom in 1996. It has been strongly linked through epidemiologic and laboratory evidence to exposure to bovine spongiform encephalopathy (BSE, or “Mad Cow Disease”), a TSE of cattle. Transmission is thought to occur through the consumption of contaminated meat and animal products; transmission via blood transfusion has also occurred in rare cases.


Restrictions

None


Reporting

Within 3 working days

Reportable by Healthcare and Labs:

Reportable by Food Service Facility:

Suspect Reportable: Yes

Reporting Timeframe: Within 3 working days



Diagnosis / Testing

There is no definitive antemortem test for diagnosis of TSEs, although in rare instances, brain biopsy (not recommended) has been used. Tonsillar biopsy may be used as part of the diagnostic work up for vCJD. Clinical diagnosis of a TSE is based on signs and symptoms, course and progression of illness. Supporting information includes a positive family history, a positive 14-3-3 protein assay and Tau test on CSF, RT-QUIC testing done on CSF samples. EEG and MRI findings and rule-out of competing diagnoses. The only way to confirm CJD, vCJD, or other prion disease is through pathologic examination of the brain, which includes histopathologic and immunohistochemical examinations and the PCR amplification and sequencing of the prion protein gene (PrP).

Every effort should be made to have an autopsy performed in all suspected cases of prion disease to provide confirmation of a diagnosis. Samples must be obtained under specific conditions.  The  Narional Prion Disease Pathology Surveillance Center has specific requirements for submission.  Contact the NPDPSC for specific information.  Charges apply for testing.  The NPDPSC (see link below) can help with autopsy arrangements and financial support of autopsy and body transportation, when necessary. Autopsies are arranged by NPDPSC. The NPDPSC will make an effort try to bring someone into Idaho to perform the autopsy, but depending on staff availability, the autopsy may need to be performed out-of-state in Utah, Oregon, or Washington with prior arrangements made. The NPDPSC will arrange for shipment of the body if an out-of-state autopsy is necessary. Contact the NPDPSC autopsy coordinator at 216-368-0587.

Testing protocols: http://case.edu/med/pathology/centers/npdpsc/protocols.html


Treatment


Additional Information


Click to Call South Central Public Health District

Click to Call the Idaho State Epidemiologist

Click to Call Idaho State Communications