Variant Creutzfeldt–Jakob disease

Not to be confused with Creutzfeldt–Jakob disease.

Variant Creutzfeldt–Jakob disease' (vCJD) is a type of brain disease within the transmissible spongiform encephalopathy family, characterized by abnormally folded proteins known as prions that affect the brain's structure and function. It is distinct from classic Creutzfeldt–Jakob disease, although both are prion diseases. The primary route of transmission is believed to be the consumption of beef infected with bovine spongiform encephalopathy (BSE), more commonly known as "mad cow disease."

Symptoms

vCJD manifests with a range of symptoms, including psychiatric problems such as depression and anxiety, behavioral changes, and painful sensations. The disease's progression can lead to severe neurological symptoms, such as muscle stiffness, twitching, and eventually, severe physical disability.

Etiology

vCJD is caused by prions, which are misfolded proteins that can induce normal proteins to also misfold and aggregate in the brain, leading to brain damage and the characteristic symptoms of the disease. The primary mode of transmission is believed to be through consumption of BSE-infected beef, although potential transmission through contaminated blood products or surgical equipment has also been suggested.

Diagnosis

Diagnosis of vCJD can be challenging and often relies on a combination of clinical criteria and diagnostic tests. The gold standard for diagnosis is a brain biopsy, although this is typically only performed post-mortem due to the invasive nature of the procedure. A clinical diagnosis may be made based on the presence of typical symptoms, coupled with supporting evidence from MRI brain scans and certain blood tests.

Treatment

There is currently no cure for vCJD, and treatment is focused on providing supportive care to relieve symptoms and improve quality of life. This may include medications for pain management, psychological support for mood disturbances, and physical therapy to assist with mobility issues.

Epidemiology

As of 2012, around 170 cases of vCJD have been recorded in the United Kingdom, primarily due to an outbreak in the 1990s, with an additional 50 cases reported in the rest of the world. The incidence of the disease has declined since 2000. The disease typically affects individuals under 30 years of age, with an average life expectancy of 13 months following the onset of symptoms. The disease was first identified in 1996 by the National CJD Surveillance Unit in Edinburgh, Scotland.

Prevention

Preventing the spread of vCJD is a multifaceted effort that focuses on monitoring prion diseases in animals and ensuring the safety of food, blood products, and medical instruments.

In many countries, measures have been implemented to eliminate the feeding of meat-and-bone meal to animals, which can propagate BSE. There are also strict regulations and controls for handling and disposing of BSE-infected cattle.

In healthcare settings, single-use instruments are recommended for surgeries involving high-infection-risk tissues. For reusable surgical instruments, recommended decontamination procedures should be strictly followed.

As for blood transfusions, countries like the UK have measures in place such as excluding donors who have a history of blood transfusion and importing plasma for fractionation from countries with a low risk of vCJD.

Future Research

Research into vCJD and other prion diseases continues, with the goal of better understanding these diseases and eventually finding a cure. Studies are being conducted to understand the exact mechanisms of prion propagation and neurodegeneration, the factors influencing prion strain diversity, and the species barrier.

In addition, efforts are ongoing to develop and improve diagnostic tests for vCJD, particularly those that can confirm the diagnosis during the early stages of the disease. There is also a need for therapeutic interventions that can slow or halt the progression of the disease, and these are areas of active research.

See also

• Jonathan Simms, a person who died from vCJD
• Mepacrine

References

Variant Creutzfeldt–Jakob disease Resources
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