US scientists are on high alert due to the discovery of an illness with symptoms resembling Creutzfeldt-Jakob disease (CJD). CJD, a rare and rapidly progressing brain disorder, leads to dementia and belongs to a group of diseases known as prion disorders.
According to Mayo Clinic, CJD symptoms are similar to those of Alzheimer's disease but worsen much faster, often leading to death within a year. Early signs include personality changes, memory loss, impaired thinking, blurry vision, insomnia, coordination problems, trouble speaking, swallowing, and sudden jerky movements. In variant CJD (vCJD), mental changes are more pronounced early on, with dementia developing later. vCJD typically affects younger individuals and lasts 12 to 14 months. Another prion disease, variably protease-sensitive prionopathy (VPSPr), mimics other dementias but progresses over 24 months.
Causes
CJD and related conditions are caused by changes in prion proteins. Normally produced in the body, these proteins become infectious and abnormally folded, spreading and disrupting bodily processes.
Risk Factors
Most CJD cases arise for unknown reasons, but some factors are associated with different CJD types:
- Age: Sporadic CJD usually develops around age 60, familial CJD slightly earlier, and vCJD in the late 20s.
- Genetics: Familial CJD is caused by a genetic mutation with a 50% chance of passing 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 very low due to effective public health measures. Chronic wasting disease (CWD), a prion disease in deer and similar animals in North America, has not yet caused human disease.
