Overview
Cattle can get a disease related to CJD called bovine spongiform encephalopathy (BSE) or “mad cow disease.”
Mad cow disease, or bovine spongiform encephalopathy (BSE), is a disease that was first found in cattle. It’s related to a disease in humans called variant Creutzfeldt-Jakob disease (vCJD). Both disorders are universally fatal brain diseases caused by a prion. A prion is a protein particle that lacks DNA (nucleic acid). It’s believed to be the cause of various infectious diseases of the nervous system. Eating infected cattle products, including beef, can cause a human to develop mad cow 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
Cause
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
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 the 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 this condition 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
There is no known cure for this condition. Different medicines have been tried to slow the disease. These include antibiotics, drugs for epilepsy, blood thinners, antidepressants, and interferon. But none works well.
The goal of treatment is to provide a safe environment, control aggressive or agitated behavior, and meet the person’s needs. This may require monitoring and assistance in the home or in a care facility. Family counseling may help the family cope with the changes needed for home care.
People with this condition may need help controlling unacceptable or dangerous behaviors. This involves rewarding positive behaviors and ignoring negative behaviors (when it is safe). They may also need help getting oriented to their surroundings. Sometimes, medicines are needed to help control aggression.
Persons with CJD and their families may need to seek legal advice early in the course of the disorder. Advance directives, power of attorney, and other legal actions can make it easier to make decisions about the care of the person with CJD.
Source
Courtesy of MedlinePlus from the National Library of Medicine
