Creuzfeldt-Jakob disease or CJD is known as fatal neurodegenerative disease that has no cure or treatments; can be referred to as transmissible spongiform encephalopathy (TSE) also known as prion disease.
Prion diseases are a group of rare brain disorders that causes "spongy" appearance in the brain and can be viewed under a microscope.
CJD is caused by an abnormal form of protein, the protein is converted into an infectious form (proteinaceous infectious particle), the infectious prion protein takes on an abnormal shape and misfolds, the abnormal prion protein can kill the brain. There are no antibodies produced in the response of TSE and there is no inflammatory response observed during infection, so the body can not combat against the mutation of the proteins.
It takes a long time before the symptoms of CJD can appear. The brains of people or animals with the prion disease undergo damage called "spongiform change" or "spongiosis" because when the tissue is examined under it resembles a sponge, and contains abnormal deposits of infected prion protein. There are multiple forms of Prion diseases related to CJD:
(1) Sporadic CJD: the most common form of Creutzfeldt-Jakob disease is sporadic CJD. It is a spontaneous development within individuals. The disease appears even though the person has no known risk factors. Sporadic CJD has no known causes.
(2)Familial Heredity: that is caused be changes in the prion protein gene. The changes are dominant making the gene inheritable from a parent, if there is a history of the disease, or positive tests for the genetic mutation related to CJD it will be passed on.
(3) Infectious CJD : a rare form of CJD that results from exposure to an external sources. The two most common outside sources are:
It can be transferred from human to human through medical treatments such as blood transfusions, cornea transplants, and dura matter transplants. Medical procedures involving instruments used in neurosurgery, and growth hormone obtained from the pituitary gland of infected individuals.
Meat or other products from cattle infected with bovine "Mad Cow Disease", a form of CJD found within animals. Scientists traced the new type of CJD to beef from cattle whose feed included processed brain tissue from other animals, including sheep with scrapies.
It can also be passed on through ingestion of contaminated beef or prion containing material. The prions can pass through the intestinal wall rapidly and enter the lymph nodes, where they can grow. Abnormal prions are picked up by the nerves and transported to the spinal cord and brain, where they infect the brain tissue. Prions may accumulate in the spleen, tonsils, and the lymph nodes where white blood cells are produced.
Site of infection , pathology and disease symptoms:
TREATMENT:
There are no treatments that can slow or stop the brain cell destruction caused by CJD. Current therapies focus in treating symptoms and supporting individuals and families coping with CJD.
Doctors may prescribe painkillers to treat pain . Muscle twitching and stiffness my be treated with muscle-relaxing medications or anti-seizure drugs
In later stages of CJD individuals become completely dependent on others for their daily needs and comfort.
Affected individuals lose their ability to move or speak and require full time care to meet their daily needs. As the condition progress, people with CJD will need significant nursing care and practical support. As well as help with feeding, washing, and mobility. Some people may need help with urinating. Many people will also have problems swallowing, so they may have to be given food through feeding tube. It may be possible to treat people with CJD at home, but it depends on the condition.
The spongiform effects brain tissue and protein deposits in the brain by forming plaque, and undergoes damage called spongiform change or spongiosis because the tissue forms holes. Once they appear the abnormal prion protein clumps together.
Creutzfeldt-Jakob disease progresses rapidly and can cause dementia. it can also result in problems with muscular coordination, personality changes, impaired memory, judgement, thinking, and impaired vision.
People with this disease may also experience insomnia, depression, or unusual sensations. As the illness progresses mental impairment becomes severe, individuals may develop involuntary muscle jerks and go blind. Eventually they lose the ability to move or speak and go into a coma. Creutzfeldt-Jakob disease causes a more rapid deterioration of a persons abilities than alzheimers. There are no antibodies produced in response to TSE, no inflammatory response observed during infection, and symptoms progress rapidly.