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.
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.
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.