Creutzfeldt-Jakob (KROITS-felt YAH-kobe) disease, also known as CJD, is a rare brain disorder that leads to dementia. It belongs to a group of human and animal diseases known as prion disorders. Symptoms of Creutzfeldt-Jakob disease can be similar to those of Alzheimer’s disease. But Creutzfeldt-Jakob disease usually gets worse much faster and leads to death.
All types of CJD are serious but are very rare. About 1 to 2 cases of CJD are diagnosed per million people around the world each year. The disease most often affects older adults.
Cause:
CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells. While the abnormal prions are technically infectious, they’re very different from viruses and bacteria.
Symptoms:
Creutzfeldt-Jakob disease is marked by changes in mental abilities. Symptoms get worse quickly, usually within several weeks to a few months.
Early symptoms include:
– Personality changes.
– Memory loss.
– Impaired thinking.
– Blurry vision or blindness.
– Insomnia.
– Problems with coordination.
– Trouble speaking.
– Trouble swallowing.
– Sudden, jerky movements.
Death usually occurs within a year. People with Creutzfeldt-Jakob disease usually die of medical issues associated with the disease. They might include having trouble swallowing, falls, heart issues, lung failure, or pneumonia or other infections.
In people with variant CJD, changes in mental abilities may be more apparent in the beginning of the disease. In many cases, dementia develops later in the illness. Symptoms of dementia include the loss of the ability to think, reason and remember.
Variant CJD affects people at a younger age than CJD. Variant CJD appears to last 12 to 14 months.
Prevention:
There’s no known way to prevent sporadic CJD. If you have a family history of neurological disease, you may benefit from talking with a genetics counselor. A counselor can help you sort through your risks.
Preventing Creutzfeldt-Jakob disease related to medical procedures
– Hospitals and other medical institutions follow clear policies to prevent CJD related to medical procedures, known as iatrogenic CJD. These measures have included:
– Using only human-made human growth hormone. This is used instead of taking the hormone from human pituitary glands.
– Destroying surgical instruments that may have been exposed to CJD. This includes instruments used in procedures that involve the brain or nervous tissue of someone with known or suspected Creutzfeldt-Jakob disease.
– Single-use kits for spinal taps, also known as lumbar punctures.
To help ensure the safety of the blood supply, people with a risk of exposure to CJD or vCJD aren’t eligible to donate blood.
This includes people who:
Have a blood relative who has been diagnosed with familial CJD. Blood relatives include parents, aunts, uncles, grandparents and cousins.
Have received a dura mater brain graft. Dura mater is the tissue that covers the brain.
Have received human growth hormone from cadavers.
