Overview Of Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease in movement and loss of mental function.
Commonly Associated With
Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease
Causes Of Creutzfeldt-Jakob Disease
CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other proteins’ ability to function.
CJD is very rare. There are several types. The classic types of CJD are:
- Sporadic CJD makes up most cases. It occurs for no known reason. The average age at which it starts is 65.
- Familial CJD occurs when a person inherits the abnormal prion from a parent (this form of CJD is rare).
- Acquired CJD includes variant CJD (vCJD), the form related to mad cow disease. Iatrogenic CJD is also an acquired form of the disease. Iatrogenic CJD is sometimes passed through a blood product transfusion, transplant, or contaminated surgical instruments.
- Variant CJD is caused by eating infected meat. The infection that causes the disease in cows is believed to be the same one that causes vCJD in humans.
- Variant CJD causes less than 1 percent of all CJD cases. It tends to affect younger people. Fewer than 200 people worldwide have had this disease. Almost all cases occurred in England and France.
CJD may be related to several other diseases caused by prions, including:
- Chronic wasting disease (found in deer)
- Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual)
- Scrapie (found in sheep)
- Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia
Symptoms Of Creutzfeldt-Jakob Disease
CJD symptoms may include any of the following:
- Dementia that gets worse quickly over a few weeks or months
- Blurred vision (sometimes)
- Changes in gait (walking)
- Confusion, disorientation
- Hallucinations (seeing or hearing things that aren’t there)
- Lack of coordination (for example, stumbling and falling)
- Muscle stiffness, twitching
- Feeling nervous, jumpy
- Personality changes
- Sleepiness
- Sudden jerky movements or seizures
- Trouble speaking
Exams & Tests
Early in Creutzfeldt-Jakob disease, a nervous system and mental examination will show memory and thinking problems. Later in the disease, a motor system examination (an exam to test muscle reflexes, strength, coordination, and other physical functions) may show:
- Abnormal reflexes or increased normal reflex responses
- Increase in muscle tone
- Muscle twitching and spasms
- Strong startle response
- Weakness and loss of muscle tissue (muscle wasting)
- There is a loss of coordination and changes in the cerebellum. This is the area of the brain that controls coordination.
- An eye exam shows areas of blindness that the person may not notice.
Tests used to diagnose Creutzfeldt-Jakob disease may include:
- Blood tests to rule out other forms of dementia and to look for markers that sometimes occur with the disease
- CT scan of the brain
- Electroencephalogram (EEG)
- MRI of the brain
- Spinal tap to test for a protein called 14-3-3
- The disease can only be confirmed with a brain biopsy or autopsy. Today, it is very rare for a brain biopsy to be done to look for this disease.
Treatment Of Creutzfeldt-Jakob Disease
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