Diagnostic Criteria for Variant Creutzfeldt-Jakob Disease in the United States

Definite Variant CJD

Neuropathologic examination of brain tissue is required to confirm a diagnosis of variant CJD. The following confirmatory features should be present.

• Numerous widespread kuru-type amyloid plaques surrounded by vacuoles in both the cerebellum and cerebrum - florid plaques.
• Spongiform change and extensive prion protein deposition shown by immunohistochemistry throughout the cerebellum and cerebrum.

Suspected Variant CJD

• Current age or age at death <55 years (a brain autopsy is recommended, however, for all physician-diagnosed CJD cases).
• Psychiatric symptoms at illness onset and/or persistent painful sensory symptoms (frank pain and/or dysesthesia).
• Dementia, and development ≥4 months after illness onset of at least two of the following five neurologic signs: poor coordination, myoclonus, chorea, hyperreflexia, or visual signs. (If persistent painful sensory symptoms exist, ≥4 months delay in the development of the neurologic signs is not required).
• A normal or an abnormal EEG, but not the diagnostic EEG changes often seen in classic CJD.
• Duration of illness of over 6 months.
• Routine investigations of the patient do not suggest an alternative, non-CJD diagnosis.
• No history of receipt of cadaveric human pituitary growth hormone or a dura mater graft.
• No history of CJD in a first degree relative or prion protein gene mutation in the patient.

NOTE:

• If a patient has the typical bilateral pulvinar high signal on MRI scan, a suspected diagnosis of variant CJD requires the presence of a progressive neuropsychiatric disorder, d, e, f and g of the above criteria, and four of the following five criteria: 1) early psychiatric symptoms (anxiety, apathy, delusions, depression, withdrawal); 2) persistent painful sensory symptoms (frank pain and/or dysesthesia); 3) ataxia; 4) myoclonus or chorea or dystonia; and 5) dementia.
• A history of possible exposure to bovine spongiform encephalopathy (BSE) such as residence or travel to a BSE-affected country after 1980 increases the index of suspicion for a variant CJD diagnosis.

Preventing vCJD
Notice to readers: The information below was published in Health Information for International Travel: 2005-2006. The case information and other statistics may have changed since the date of publication.
To reduce any risk of acquiring vCJD from food, travelers to Europe or other areas with indigenous cases of BSE may consider either avoiding beef and beef products altogether or selecting beef or beef products, such as solid pieces of muscle meat (rather than brains or beef products like burgers and sausages), that might have a reduced opportunity for contamination with tissues that may harbor the BSE agent. Milk and milk products from cows are not believed to pose any risk for transmitting the BSE agent.
Treatment
Notice to readers: The information below was published in Health Information for International Travel: 2005-2006. The case information and other statistics may have changed since the date of publication.
As of September 2004, treatment of prion diseases remains supportive; no specific therapy has been shown to stop the progression of these diseases.