Creutzfeldt-Jakob disease facts for kids
Creutzfeldt–Jakob disease (pronounced KROITS-felt YAH-kohb) or CJD is a serious brain disease. It slowly gets worse over time. Sadly, there is no cure for CJD, and it always leads to death. CJD is sometimes called a human form of "mad cow disease" (which is called bovine spongiform encephalopathy, or BSE). But CJD and BSE are not the same disease. BSE is actually one rare cause of a specific type of CJD.
CJD is caused by something called a prion. Prions are special proteins that are folded incorrectly. These misfolded prions can make other normal proteins fold incorrectly too. CJD makes the brain tissue unhealthy very quickly. As the disease harms the brain, it creates tiny holes. This makes the brain's texture change, becoming like a kitchen sponge.
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Types and Causes of CJD
There are different ways people can get CJD:
Variant CJD (vCJD)
- This type of CJD might be caused by eating food that has prions in it. An example is meat from cows with BSE ("mad cow disease"). However, this is a very rare way to get CJD.
Sporadic CJD (sCJD)
- This is the most common type of CJD. About 85% of all CJD cases are sporadic CJD. Scientists do not know what causes sCJD. It seems to happen randomly, without a clear reason.
Familial CJD (fCJD)
- Most of the other 15% of CJD cases are familial CJD. This form of CJD can be passed down through families. It means it runs in the family's genes.
Iatrogenic CJD
- This type of CJD usually happens because of a medical procedure. A person might get it if they receive blood or tissue from someone who has CJD. For example, if a person gets a blood transfusion or a cornea transplant from someone with CJD, they could get iatrogenic CJD.
Signs and Symptoms
The first sign of CJD is usually a quick worsening of dementia. This means a person's memory and thinking skills get worse very fast. They might also have changes in their personality and see or hear things that are not real.
Other common mental signs can include:
- Feeling very worried (Anxiety)
- Feeling very sad (Depression)
- Feeling suspicious of others (Paranoia)
- Having thoughts or actions they cannot control (Obsessive-compulsive symptoms)
- Having confused thoughts or losing touch with reality (Psychosis)
Physical signs of CJD often include:
- Trouble speaking clearly
- Sudden, jerky movements of the body (myoclonus)
- Trouble keeping their balance (ataxia)
- Difficulty walking steadily
- Shaking or feeling stiff
- Problems with their Vision
- Trouble swallowing, which can make eating very hard
- Trouble coughing, which can lead to lung infections like pneumonia
- Movements that the person cannot control (dyskinesia)
Most people with CJD pass away within six months after their first symptoms appear. Often, they die from pneumonia because they cannot cough well. About 15% of patients live for two or more years. Some patients have lived 4–5 years with mostly mental symptoms. But then the disease gets worse, causing more physical problems. Once this happens, people usually pass away within a year.
The symptoms of CJD happen because more and more nerve cells in the brain die. When scientists look at brain tissue from a CJD patient under a microscope, they see many tiny holes. These holes are where large groups of nerve cells have died.
Diagnosis
Doctors might suspect CJD when a person has certain symptoms. For example, memory problems usually get worse slowly. But if memory and thinking skills get worse very quickly, it is unusual. This, along with symptoms like jerky movements, might make doctors think of CJD.
Tests can then be done to see if the person has CJD. These tests include:
- Electroencephalography (EEG): This test checks the electrical activity in the brain. Doctors can often see changes on the EEG that are common in people with CJD. The type of changes depends on the CJD type and how far along the disease is.
- Lumbar puncture (spinal tap): This test involves taking a small sample of cerebrospinal fluid. This fluid surrounds the brain and spinal cord. Doctors look for a specific protein called "14-3-3 protein" in the fluid.
- MRI of the brain: This test uses a very strong magnet to take detailed pictures of the brain.
- Biopsy: In a biopsy, a surgeon takes a tiny piece of tissue from the body using a needle. Doctors then look at it under a microscope. Variant CJD (vCJD) can be diagnosed with a biopsy of the tonsils. For all other types of CJD, a brain biopsy is the only way to be completely sure. However, a brain biopsy can cause brain damage. So, it is usually not done if other tests already show that a person likely has CJD.
Treatment
As of 2016, there is no treatment that can cure CJD or even slow down its effects. Many experiments are being done to try and find new treatments.
Today, the only treatments for CJD are medicines that help with the symptoms. These medicines make patients more comfortable. For example, patients who have seizures might be given anticonvulsant medications. Other medicines, like Benzodiazepines, can help reduce muscle jerks.
Patients might also choose to have medical procedures to help with difficult symptoms. For example, CJD can make it so hard to swallow that a person cannot eat. Some people with CJD choose to have a feeding tube put in when they can no longer eat. This tube goes into the stomach. Special liquid food can then be given directly into the stomach to provide nutrition.
Related pages
See also
In Spanish: Enfermedad de Creutzfeldt-Jakob para niños
