They eventually lose the ability to move and speak, and enter a coma. Pneumonia and other infections often occur in these individuals and can lead to death.
Variant CJD begins primarily with psychiatric symptoms, affects younger individuals than other types of CJD, and has a longer than usual duration from onset of symptoms to death.
However, CJD causes unique changes in brain tissue which can be seen at autopsy. Current scientific consensus maintains that abnormal forms of normal cellular proteins called prions cause CJD in people and TSE in animals. The normal, harmless prion is usually designated PrP C C stands for cellular and the abnormal, infectious form which causes the disease is PrP Sc Sc stands for prototypical prion disease—scrapie.
Proteins are long chains of amino acids that have to fold together into a unique shape or conformation to gain function in the cells. Research findings indicate that the infectious prion originates from a normal protein whose conformation has changed to one that causes the disease. The normal prion protein is found throughout the body but is most abundant in the nervous system. Its overall role is not fully understood. It is believed that the harmless to infectious protein conformational change is common to the all major forms of human prion disease, including CJD.
In the acquired form of the disease, the PrP Sc comes from the outside the body, for example, through contaminated meat as is seen in vCJD. It then clings to and changes the conformation of the normal prion protein of the host and progressively spreads in domino-like fashion toward the brain where it causes lesions.
As the mutated PrP C replicates itself, it spontaneously changes shape into the infectious form. Prions themselves do not contain genetic information and do not require genes to reproduce themselves.
Several different mutations in the prion gene have been identified. The particular mutation found in each family affects how frequently the disease appears and what symptoms are most noticeable. However, not all people with mutations in the prion protein gene develop CJD. In the sporadic form, the infectious prions are believed to be made by an error of the cell machinery that makes proteins and controls their quality.
These errors are more likely to occur with aging, which explains the general advanced age at onset of CJD and other prion diseases. Once they are formed, abnormal prion proteins aggregate, or clump together. Investigators think these protein aggregates lead to the nerve cell loss and other brain damage seen in CJD.
However, they do not know exactly how this damage occurs. CJD cannot be transmitted through the air or through touching or most other forms of casual contact.
Spouses and other household members of people with sporadic CJD have no higher risk of contracting the disease than the general population.
Read more about treating Creutzfeldt-Jakob disease. The vCJD Trust assesses claims and pays compensation to victims and their families. Page last reviewed: 07 September Next review due: 07 September Symptoms of CJD Symptoms of CJD include: loss of intellect and memory changes in personality loss of balance and co-ordination slurred speech vision problems and blindness abnormal jerking movements progressive loss of brain function and mobility Most people with CJD will die within a year of the symptoms starting, usually from infection.
What causes CJD? Familial or inherited CJD Familial CJD is a very rare genetic condition where one of the genes a person inherits from their parent the prion protein gene carries a mutation that causes prions to form in their brain during adulthood, triggering the symptoms of CJD.
It affects about 1 in every 9 million people in the UK. How CJD is treated There's currently no cure for CJD, so treatment aims to relieve symptoms and make the affected person feel as comfortable as possible. Some people will need nursing care and assistance with feeding. In addition to the classical form of the disease, which is found worldwide, there is a new variant of the disease that has been found almost exclusively in the United Kingdom.
Cases of nv-CJD have slightly different clinical signs, symptoms, and laboratory features from those of the classical form of CJD. Monitoring functions for these diseases in humans and animals collectively called transmissible spongiform encephalopathies or TSEs are shared by federal, state and local health and agricultural officials.
The Florida Department of Health regularly reviews death certificates and tracks the numbers of deaths attributed to CJD. This age group is where experts feel nv-CJD is most likely to occur. We also take reports from medical providers and the public on clusters of disease. Agricultural officials are responsible for looking for BSE in livestock. Information on these programs can be obtained from the links that follow. The free Acrobat Reader may be required to view these files.
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