Variant Creutzfeldt-Jakob Disease

Q

What is vCJD?
How is it transmitted?
What are the symptoms of vCJD?
How long does it take for symptoms to occur?
How do I know if I have vCJD?
How is it treated?
Is there a vaccine for vCJD?
Why screen donated blood for vCJD?

A

What is vCJD?

Variant Creutzfeldt-Jakob Disease (vCJD) is a rare, degenerative, fatal brain disorder in humans. There is no known treatment of vCJD and it is invariably fatal.

vCJD is thought to be linked to bovine spongiform encephalopathy (BSE), or mad-cow disease. BSE is a progressive neurological disorder of cattle that results from infection by an infectious protein particle, or prion.

A

How is it transmitted?

It is believed that the people who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE.

Although experience with this new disease is limited, there has been no evidence to date of vCJD being transmitted through direct contact of one person with another.

In at least one case, however, blood from a donor who was later identified as having vCJD was transfused to a patient who had subsequently developed vCJD. The case suggest the possibility that vCJD may be transmitted through transfusion.

A

What are the symptoms of vCJD?

Symptoms include:

  • Prominent psychiatric/behavioral symptoms
  • Painful dyesthesiasis (distortion of sense of touch)
  • Delayed neurologic signs, including:
    • Ataxia (inability to coordinate muscular movements)
    • Dementia (impaired intellectual functioning)
    • Myoclonus (shock-like muscle contractions)
A

How long does it take for symptoms to occur?

The incubation period for vCJD is unknown because it is a new disease. However, it is likely that this incubation period will be measured in terms of many years or decades. In other words, whenever a person develops vCJD from consuming a BSE-contaminated product, he or she likely would have consumed that product many years or a decade or more earlier.

A

How do I know if I have vCJD?

vCJD can be confirmed only through examination of brain tissue obtained by biopsy or at autopsy, but a "probable case" of vCJD can be diagnosed on the basis of clinical criteria developed in the United Kingdom.

A

How is it treated?

No specific therapy has been shown to stop the progression of vCJD.

A

Is there a vaccine for vCJD?

No, there is no vaccine and no cure for vCJD.

A

Why screen donated blood for vCJD?

In at least one case, blood from a donor who was later identified as having vCJD was transfused to a patient who had subsequently developed vCJD. The case suggest the possibility that vCJD may be transmitted through transfusion.

Because the incubation period could be years or decades, a blood donor could be infected with vCJD for years before symptoms appear.

 

References

  1. Centers for Disease Control and Prevention Website - vCJD Page. Accessed Aug. 12, 2005.
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