Creutzfeldt-Jakob disease (YAH-kobe at KROITS) (CJD) is a degenerative disease of the brain that leads to dementia and ultimately death. The symptoms of Creutzfeldt-Jakob disease can be similar to those of other dementia-like diseases of the brain, such as Alzheimer’s disease. However, Creutzfeldt-Jakob disease usually progresses much faster.
- CJD gained public attention in the 1990s when some people in the UK developed a form of the disease – variant CJD (vCJD) – after eating the meat of sick cattle. However, the “classic” Creutzfeldt-Jakob disease has not been associated with contaminated beef. All types of CJD are severe but very rare. Around one to two cases of CJD are diagnosed per million people worldwide each year, most commonly in the elderly.Creutzfeldt-Jakob disease Treatment Hyderabad
Creutzfeldt-Jakob disease is usually characterized by rapid mental deterioration within a few months. The first signs and symptoms are usually:Creutzfeldt-Jakob disease Treatment Hyderabad
- Personality changes
- Memory loss
- Changed thinking
- Blurred vision or blindness
- In coordination
- Difficulty speaking
- difficulties swallowing
- Abrupt and jerky movements
Images comparing a normal prion with a diseased prion
How prions open the popup dialog
Creutzfeldt-Jakob disease and its variants belong to a large group of diseases in humans and animals known as transmissible spongiform encephalopathies (TSEs). The name is derived from the spongy holes that are visible under a microscope and develop in the affected brain tissue.
- Creutzfeldt-Jakob disease and other TSEs appear to be caused by abnormal versions of a type of protein called a prion. Usually these proteins are produced in our body and are harmless. However, when deformed, they become infectious and can disrupt normal biological processes.Creutzfeldt-Jakob disease Treatment Hyderabad
Autosomal dominant inheritance model
Autosomal Dominant Inheritance PatternOpen Context Dialog
Most cases of Creutzfeldt-Jakob disease occur for unknown reasons and no risk factors can be identified. However, some factors appear to be linked to different types of CJD:Creutzfeldt-Jakob disease Treatment Hyderabad
- Age. Sporadic CJD tends to develop later in life, usually around the age of 60. Familial CJD begins a little earlier, and vCJD has affected people at a much younger age, usually in their late twenties.
- Genetically. People with familial CJD have a genetic mutation that causes the disease. To develop familial CJD, a child must have a copy of the mutated gene that is inherited from both parents. If you have the mutation, the chance of passing it on to your children is 50%.
- Exposure to contaminated tissue. People who have received infected man-made human growth hormone or who have had the infected tissue covering the brain (dura) transplanted may be at risk for iatrogenic CJD.
There is no known way to prevent sporadic Creutzfeldt-Jakob disease (CJD). If you have a family history of neurological disorders, speaking with a genetic counselor may benefit you. He or she can help you clarify the risks associated with your situation.
- Prevention of iatrogenic CJD
Hospitals and other medical institutions follow clear guidelines to prevent iatrogenic CJD. These measures include:
- Exclusively using artificial human growth hormone in place of the human pituitary derived type
Destruction of surgical instruments used on the brain or nervous tissue of someone with or with suspected CJD
Disposable kits for lumbar punctures (lumbar punctures).Creutzfeldt-Jakob disease Treatment Hyderabad