Back to Health A to Z. Creutzfeldt-Jakob disease CJD is a rare and fatal condition that affects the brain. It causes brain damage that worsens rapidly over time. Most people with CJD will die within a year of the symptoms starting, usually from infection. This is because the immobility caused by CJD can make people with the condition vulnerable to infection.
Read more about the symptoms of Creutzfeldt-Jakob disease and diagnosing Creutzfeldt-Jakob disease. CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells.
While the abnormal prions are technically infectious, they're very different from viruses and bacteria. These are deposits scattered throughout the brain, which are surrounded by spongiform change. There is no treatment at present for CJD. However, there are a number of drugs, which can relieve the symptoms and make the patient more comfortable. These include valproate and Clonazepam for jerking movements. The patient and their carers will also need much help from social services and nursing services.
In addition to surveillance and research they can organise intensive support for the person with CJD and their family. Is Alzheimer's disease hereditary? Is there a test that can predict Alzheimer's disease? How is Alzheimer's disease diagnosed? Diagnosis of dementia Disclosure of the diagnosis Facing the diagnosis Taking care of yourself Developing coping strategies Maintaining a social network Attending self-help groups Accepting help from others Dealing with feelings and emotions Changing roles and how you see yourself On a more positive note Organising family support Dealing with practical issues Financial and administrative matters Driving Safety issues Employment issues Healthy eating Contact and communication Speaking, listening and communication Signs, symbols and texts Personal relationships Talking to children and adolescents Changing behaviour Lack of interest in hobbies Disorientation Managing everyday tasks Keeping an active mind Services Caring for someone with dementia The onset of the disease Diagnosis: should the person with dementia be told?
Dealing with emotions Arranging who will be responsible for care Determining to what extent you can provide care How will Alzheimer's disease affect independent living? What progress so far? Acknowledgements References Intercultural care and support Introduction Understanding dementia and help seeking Diagnosis, assessment and treatment.
Support and care Professional and informal carers Conclusions References Members of the expert working group Terms and concepts Dementia as a disability? Why PharmaCog? Who are the PharmaCog partners? Kuru is considered an acquired prion disease. To date, about cases of vCJD, mostly in the United Kingdom, have been reported related to consuming beef but none in which the disease was acquired in the U. Other TSEs are found in specific kinds of animals.
These include BSE, mink encephalopathy, feline encephalopathy, and scrapie, which affects sheep and goats. Chronic wasting disease CWD affects elk and deer and is increasingly prevalent in certain areas in the United States. To date no transmission of CWD to humans has been reported. Although sporadic TSE includes five distinct subtypes of sporadic CJD and sporadic fatal insomnia sFI , overall they are characterized by rapidly progressive dementia.
Initially, individuals experience problems with muscle coordination, personality changes including impaired memory, judgment, and thinking , and impaired vision.
People with the disease, especially with FFI, also may experience insomnia, depression, or unusual sensations. They often develop involuntary muscle jerks called myoclonus , and they may go blind. 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. Platelet Components. Regulatory Resources for Transfusion Services.
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