Washington D.C.: U.S. scientists are on high alert following the discovery of an illness exhibiting symptoms similar to Creutzfeldt-Jakob disease (CJD), a rare and rapidly progressing brain disorder that leads to dementia. CJD is part of a group of diseases known as prion disorders, which are known for their severe impact on the brain.
According to the Mayo Clinic, CJD shares several symptoms with Alzheimer’s disease but progresses much more rapidly, often resulting in death within a year. Early symptoms of CJD include personality changes, memory loss, impaired thinking, blurry vision, insomnia, coordination problems, trouble speaking, difficulty swallowing, and sudden jerky movements.
In the variant form of CJD (vCJD), mental changes are more pronounced in the early stages, with dementia developing later. This variant typically affects younger individuals and has a duration of 12 to 14 months. Another related prion disease, variably protease-sensitive prionopathy (VPSPr), mimics other forms of dementia but has a longer progression period of around 24 months.
Causes of Creutzfeldt-Jakob
CJD and related prion diseases are caused by abnormal changes in prion proteins, which are normally produced by the body. These proteins become infectious and abnormally folded, spreading and disrupting normal bodily processes.
Risk Factors of Creutzfeldt-Jakob
While the majority of CJD cases arise without a known cause, certain risk factors are associated with different types of the disease:
Age: Sporadic CJD typically develops around the age of 60, familial CJD at a slightly younger age, and vCJD in individuals in their late 20s.
Genetics: Familial CJD results from a genetic mutation, with a 50% chance of being passed on to offspring.
Exposure to Contaminated Tissue: Risk factors include receiving infected human growth hormone or brain tissue transplants.
The risk of contracting vCJD from contaminated beef is considered very low due to stringent public health measures. Additionally, Chronic Wasting Disease (CWD), a prion disease found in deer and similar animals in North America, has not yet been shown to cause disease in humans.
As the scientific community closely monitors this new illness, efforts are being intensified to understand its origins, transmission mechanisms, and potential impact on public health.
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