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Creutzfeldt-jakob Disease

Alternate Names

  • subacute spongiform encephalopathy
  • Brain structures

Definition

The classic form of Creutzfeldt-Jakob disease, or CJD, is disease that causes a rapid progressive dementia resulting in death in a matter of weeks to months.

What is going on in the body?

Both classic and new variant CJD are two examples of several known prion diseases. These types of diseases can be inherited, occur sporadically, or secondary to infection after exposure to prions.
In general, prion diseases are disorders of protein folding. In other words, a prion is an abnormally folded protein. This abnormally folded protein can propagate or multiply a process that is not yet fully understood.
A build up of prions results in damage and ultimately death of brain cells resulting in progressive and relentless dementia and death.

Risks

What are the causes and risks of the disease?

There are three main causes for classic CJD.
Inherited CJD runs in families and is caused by a mutation in the gene coding for the normal prion protein.
Sporadic CJD has no known genetic or infectious cause.
Creutzfeldt-Jakob disease can be transmitted to others. An individual may be infected through direct or indirect contact with body tissues of an infected person.
Following are examples of how this transmission has occurred:
  • after surgery where contaminated surgical instruments were used
  • after corneal transplantation, where eye tissue was taken from an infected person
  • following surgery, where grafts of brain membranes were derived from an infected individual
  • after injection of human growth hormone from the pituitary of an infected cadaver
  • following the ingestion of beef from a cow with a prion disease commonly known as mad cow disease

At the present time, infected cattle are found mostly in Europe, particularly the United Kingdom although a few infected animals have been discovered in the United States and Canada.

Prevention

What can be done to prevent the disease?

Travelers to the UK, an area with a relatively high risk for mad cow disease, can limit their risk for new variant CJD by avoiding beef and beef products. If the person chooses to eat meat, he or she should select solid pieces of muscle meat, such as a steak or roast. Sausage and hamburger should be avoided.
Public health measures are important such as educating beef farmers about the disease, screening cattle herds by testing for prion disease in "downers" (cattle that are too sick to stand), and then eliminating infected cattle thereby preventing them from getting into the food supply.

Diagnosed

How is the disease diagnosed?

Diagnosis of CJD begins with a medical history and physical exam. A spinal tap can be performed to look for abnormalities consistent with CJD.
In addition an EEG, or recording of the brain waves, may show abnormalities. A brain scan such as magnetic resonance imaging (MRI) of the brain may help in diagnosis.
A definitive diagnosis can only be made with a biopsy of the brain. This involves taking a small piece of brain tissue with a special needle. The piece of tissue can then be examined with a microscope.

Long Term Effects

What are the long-term effects of the disease?

CJD causes progressive brain deterioration and death.

Other Risks

What are the risks to others?

CJD does not seem to spread from person to person. However, it may be spread through direct or indirect contact with infected body tissue, particularly any tissue relating to the spinal cord or brain.

Treatments

What are the treatments for the disease?

There is no treatment for CJD. Medications may used to treat psychiatric problems or relieve the symptoms resulting from CJD.

Side Effects

What are the side effects of the treatments?

Medications may cause allergic reactions and stomach upset.

After Treatment

What happens after treatment for the disease?

CJD causes progressive brain damage and death. The classic form usually causes death within 6 to 12 months of the initial symptoms. Someone who has new variant CJD usually dies within 1 to 2 years of the initial symptoms.

Monitor

How is the disease monitored?

The individual may have periodic visits with the healthcare provider to monitor the disease. Any new or worsening symptoms should be reported to the provider.

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