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What is Creutzfeldt-Jakob Disease (CJD)

 

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year. In the early stages of disease, people may have failing memory, behavioural changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
 

Symptoms of Creutzfeldt-Jakob Disease (CJD)

 

CJD is characterized by rapidly progressive dementia. Initially, individuals experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these individuals and can lead to death.

Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia.
 

Causes:

Currently, there is no known cause of CJD, however the leading scientific theory is that a form of protein contained in the body spontaneously changes into an infectious form of the same protein and attacks the body, causing neuron loss and brain damage.
 

 

Group

TBC

Short Description

TBC

Contact Details

TBC

Creutzfeldt-Jakob Disease (CJD)

 

Group

CJD Support Network

Web Address

www.cjdsupport.net

 

Contact Details

01630 673973

Group

National CJD Surveillance Unit

Web Address

http://www.cjd.ed.ac.uk/

Contact Details

0131 537 2128

Incidence and Prevalence

 

There are three major categories of CJD:

* In sporadic CJD, the disease appears even though the person

  has no known risk factors for the disease. This is by far the most

 common type of CJD and accounts for at least 85 percent of

 cases.
* In hereditary CJD, the person has a family history of the disease

 and/or tests positive for a genetic mutation associated with CJD

. About 5 to 10 percent of cases of CJD in the United States are

 hereditary.
* In acquired CJD, the disease is transmitted by exposure to brain

or nervous system tissue, usually through certain medical

procedures. There is no evidence that CJD is contagious through

casual contact with a CJD patient. Since CJD was first described

in 1920, fewer than 1 percent of cases have been acquired CJD.
 

 

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