Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, and fatal brain disorder. It is said to affect one person out of every million people world wide each year. In the United States, alone, it affects about 200 people each year, according to the National Institute of Neurological Disorders and Strokes.

Creutzfeldt-Jakob disease (CJD) will normally begin to appear later on in life and will progress rapidly. The onset of symptoms will normally begin when a person is about 60 years old. Up to ninety percent of people who have Creutzfeldt-Jabok disease (CJD) will die within a year on the onset of symptoms.

There are three major categories of Creutzfeldt-Jakob disease (CJD). The first is known as sporadic Creutzfeldt-Jakob disease, or sporadic CJD. In this type of CJD, the person will have the disease even though they do not have any known risk factors of the disease. Sporadic Creutzfeldt-Jakob disease or sporadic CJD is the most common form of this disease and occurs in up to eighty-five percent of all Creutzfeldt-Jakob disease (CJD) cases.

The second major category of Creutzfeldt-Jakob disease (CJD) is called hereditary Creutzfeldt-Jakob disease, or hereditary CJD. In this type, the person has tested positive for the gene mutation that is known to cause Creutzfeldt-Jakob disease. Five to ten percent of all cases of Creutzfeldt-Jakob disease cases are hereditary.

The third major category of Creutzfeldt-Jakob disease (CJD) is known as acquired Creutzfeldt-Jakob disease, or acquired CJD. This is the least common form of Creutzfeldt-Jakob disease, (CJD) affecting less than one percent of people with CJD. When acquired CJD is present, it is transmitted by exposure to the brain or nervous system tissue that can happen through certain medical procedures. There is no evidence that shows Creutzfeldt-Jakob disease (CJD) to be contagious through casual contact with a person who has the disease.

Creutzfeldt-Jakob disease (CJD) is a type of human and animal disease, known as TSEs or transmissible spongiform encephalopathies. The word "spongiform" describes what an infected brain looks like. An infected brain becomes filled with holes and will resemble a sponge when examined under a microscope. Creutzfeldt-Jakob disease (CJD) is the most common type of TSE known in humans.

A person with CJD will have dementia that progresses very quickly, according to MedicineNet.com. At first, the person will have trouble with muscle coordination, personality changes, and problems with their vision. Personality changes to look for would be things such as trouble with memory, judgment, and thinking. A person with CJD will likely experience trouble sleeping (insomnia), depression, and/or unusual sensations throughout their body. The person is likely to develop involuntary muscle jerking known as cyclones, and the person may go blind. Eventually, a person with Creutzfeldt-Jakob disease (CJD) will be unable to move or speak, and will go into a coma. This disease does not cause any flu-like symptoms in the person who has it, but once they go into a coma, they can easily get pneumonia or other infections that can cause death.

There are variations of Creutzfeldt-Jakob disease (CJD), and each variation can have slightly different symptoms. One particular variation that is found in Great Britain and France begins with psychiatric symptoms, is known to affect younger people, and has a longer duration from onset to death. Yet another variation is known in Japan. This variation has symptoms that progress over several years. What causes these variations is unknown.

Some of the symptoms of Creutzfeldt-Jakob disease (CJD) are similar to other neurological disorders such as Huntington's disease or Alzheimer's disease. The difference can be seen in the brain tissue upon autopsy because Creutzfeldt-Jakob disease (CJD) causes very unique changes in the brain tissue. Another difference is that Creutzfeldt-Jakob disease (CJD) usually causes faster deterioration in a person's mental and physical abilities than other types of dementia.

There is currently no single test to diagnose Creutzfeldt-Jakob disease (CJD). If a doctor thinks that a person may have it, they will first rule out other types of dementia that are treatable. A neurological exam will be done and the doctor will probably talk to other doctors about the case. A spinal tap; taking some of the person's spinal fluid, may be done to rule out more common types of dementia. An encephalograph (EEG) may be done to record the brains electrical pattern to detect specific abnormalities that are known to show in people with Creutzfeldt-Jakob disease (CJD). A computerized test that shown the tomography of the person's brain, or magnetic resonance imaging (MRI) may be used to show any characteristic patterns of brain degeneration are also tests used to diagnose Creutzfeldt-Jakob disease (CJD). These tests can all help form a diagnosis, but they are not always conclusive. The only way to definitively diagnose Creutzfeldt-Jakob disease (CJD) is by a brain biopsy or autopsy. Brain biopsy can be very dangerous for the person because a small part of the brain is actually removed to be looked at under a microscope by a neuropathologist. The biopsy itself may not always be accurate because it is possible that the portion that is taken is not affected by the disease. Brain biopsy can also be very dangerous for the person. Even if a brain biopsy shows that the person has Creutzfeldt-Jakob disease (CJD), the patient cannot be helped because there is currently no effective treatment, therefore; a brain biopsy is not recommended unless it is being used to rule out any treatable condition. In an autopsy, the entire brain is examined after the person dies. Both brain biopsy and autopsy pose a small risk to the person who handles the brain tissue. In other words, the person who handles the brain tissue is at a small, but definite risk of getting acquired Creutzfeldt-Jakob disease. To minimize their risk, they use special surgical and disinfection procedures.

There is currently no available treatment or cure for Creutzfeldt-Jakob disease (CJD). The focus is on lessening the symptoms and making the person as comfortable as possible. New drugs are being tested and studied, but as of right now, these drugs have shown no consistent benefit for humans.

The risk of spreading Creutzfeldt-Jakob disease (CJD) from one person to another is very small, but there are ways to make the risk even smaller. For instance, if it is suspected, or confirmed that a person has Creutzfeldt-Jakob disease (CJD); the person should never donate blood, tissues, or organs. This is also true if a person has an increased risk of acquiring the disease because of family history, or any other risk factor.

According to MedicineNet.com, Creutzfeldt-Jakob disease (CJD) is believed to be cause by abnormal, infected prion proteins in the body. These prions cannot be destroyed by normal sterilization methods such as cooking, washing, and/or boiling. When working with a person that has the disease, there are special precautions to take to minimize the risk of acquiring the disease.

First, always wash your hand and any exposed skin before eating, drinking, smoking, putting anything in your mouth, or touching any of your mucous membranes.

Second, be sure to cover any open wounds you may have with a waterproof dressing.

Third, wear surgical gloves whenever you are handling the patient to avoid contaminating yourself with bodily fluids or tissues.

Fourth, Avoid cutting or sticking yourself with any instrument that could be contaminated.

Fifth, use face protection to avoid any bodily fluids splashing in your face.

Sixth, soak any instruments used on the patient in chlorine bleach for at least an hour, then, put them in a pressure cooker, or autoclave, for at least an hour at a temperature of 132-134 degrees Centigrade. This will sterilize the instruments. If possible, use disposable instruments and be sure to dispose of them properly, in a sharp's container that is closed. Seal the container well before disposing of it.

In conclusion, Creutzfeldt-Jakob disease, or CJD is a fatal neurological disease that has rapid progression of dementia and other problems. Most people will live less than a year after the onset of symptoms. It can be acquired, hereditary, or sporadic. It is caused by infected prions in the body. Diagnosis can only be definitively made through a brain biopsy (if infected tissue is taken) or autopsy. There is a small risk of acquiring the disease through contact with contaminated brain tissue or neurological tissue or fluid. It is not believed to be contagious through casual contact, but special precautions should be taken by any one who cares for a person with the disease. There is no cure for Creutzfeldt-Jakob disease at this time.

Information in this article was obtained from http://www.medicinenet.com and http://www.ninds.nih.gov.