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Creutzfeldt-Jakob Disease

What is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob disease is a rare, degenerative, invariably fatal brain disorder.

Creutzfeldt-Jakob disease is very rare. It affects about one person in every one million people per year worldwide. In the United States there are about 200 cases per year.

Creutzfeldt-Jakob disease usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year.

Symptoms of Creutzfeldt-Jakob disease

In the early stages of disease the most common symptoms are:

  • rapidly progressive dementia
  • behavioral changes
  • lack of muscle coordination
  • impaired memory, judgment and thinking
  • impaired vision
  • insomnia

As Creutzfeldt-Jakob disease progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

Some symptoms of Creutzfeldt-Jakob disease are similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. However, Creutzfeldt-Jakob disease causes unique changes in brain tissue which can be seen at autopsy.

Types of Creutzfeldt-Jakob disease

There are three major categories of Creutzfeldt-Jakob disease:

  1. Sporadic Creutzfeldt-Jakob disease
    • In sporadic Creutzfeldt-Jakob disease, the disease appears even though the person has no known risk factors for the disease.
    • This is the most common type of Creutzfeldt-Jakob disease and accounts for at least 85 percent of cases.
  2. Hereditary Creutzfeldt-Jakob disease
    • Inherited from parents
    • About 5 to 10 percent of cases of Creutzfeldt-Jakob disease in the United States are hereditary.
  3. Acquired Creutzfeldt-Jakob disease
    • Transmitted by exposure to brain or nervous system tissue.
    • Not contagious through casual contact with a Creutzfeldt-Jakob disease patient.

How is Creutzfeldt-Jakob disease Transmitted?

Creutzfeldt-Jakob disease cannot be transmitted through the air or through touching or most other forms of casual contact.

In some cases, Creutzfeldt-Jakob disease has spread to other people from grafts a tissue that covers the brain, transplanted corneas, implantation of inadequately sterilized electrodes in the brain, and injections of contaminated pituitary growth hormone derived from human pituitary glands taken from cadavers.

How is Creutzfeldt-Jakob disease Treated?

Unfortunately, there is no treatment that can cure or control Creutzfeldt-Jakob disease. Currently, treatment for Creutzfeldt-Jakob disease is aimed at alleviating symptoms and making the patient as comfortable as possible.

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