Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease. Bovine spongiform encephalopathy is a prion disease that affects cows; there is evidence that this illness can be transmitted to humans, producing vCJD. This illness also is often called “mad cow disease.”
Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection; instead, the illness is related to progressive accumulation of prion proteins. The central nervous system is progressively damaged as these prion proteins accumulate.
Exposure to prion protein is the primary risk factor. Eating beef from infected cows is a risk factor. There is also evidence that human growth hormone (HGH) injections prior to the mid-1980s was a risk factor, but changes in the preparation of HGH in the mid-1980s eliminated this risk.
Psychiatric symptoms predominate:
Neurologic symptoms predominate:
- Gait
- Ataxia
- Involuntary movements: muscle jerks and stiffness
- Cognitive decline:
impaired speech
Median duration from first symptoms to death is 13 months ( range 6 to 39 months).
The clinical history and physical exam are the primary diagnostic tools. If your physician suspects vCJD, additional tests including
lumbar puncture,
CT
or
MRI
of the brain, and
electroencephalogram (EEG), may be necessary.
Biopsy
of tonsil tissue has been used to confirm vCJD in some cases. In many cases, final diagnosis requires autopsy and pathologic studies.
Currently, there is no cure for vCJD. Treatment is primarily supportive, maximizing level of function and minimizing discomfort.
About 150 worldwide cases of vCJD have occurred to date, nearly all associated with beef consumption in the United Kingdom. There is a great deal of controversy regarding safety of US beef. Two cases of bovine spongiform encephalopathy have been detected in the US. No cases of vCJD attributable to consumption of US beef have been detected.
To minimize risk, it is generally recommended that you avoid beef products, particularly processed meat (eg, sausage and hotdogs), or beef items containing brain, spinal cord, or bone marrow.
Last reviewed March 2007 by Roshni N. Patel, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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