What causes Creutzfeldt-Jakob disease?

The cause of CJD and other TSE diseases is due to the abnormality of a protein called a prion.
The cause of CJD and other TSE diseases is due to the abnormality of a protein called a prion.

Creutzfeldt-Jakob disease belongs to a broad group of human and animal diseases called transmissible spongiform encephalopathies (TSE). The cause of CJD and other TSE diseases is due to the abnormality of a protein called a prion. Normally prions are harmless, but when they become misshapen, they can become infectious and affect normal biological processes in the body. The disease is not transmitted through casual touching, sexual contact, coughing, or sneezing

Some possible ways of developing CJD are:

  • Spontaneous: In most cases, the exact cause of CJD or prion disability is not known, and the disorder can arise spontaneously. This is also called sporadic CJD.
  • Genetics: Familial CJD occurs due to genetic mutation associated with CJD that may be inherited.
  • Contamination: Exposure to an infected human or animal tissue during a medical procedure, such as brain surgery, blood transfusion, corneal or skin transplant, or consuming contaminated meat, especially beef. CJD has been associated with eating beef infected with mad cow disease (bovine spongiform encephalopathy).
  • Age: The risk of CJD increases with age, especially after 60 years of age. Familial CJD usually presents at a younger age, usually in the 20s.

What are the signs and symptoms of Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease is characterized by rapid mental deterioration within a few months. The symptoms worsen rapidly. Patients eventually have the failure of multiple organ systems, such as the heart and lungs. CJD can cause fatal complications leading to death. Early signs and symptoms typically include:

How is Creutzfeldt-Jakob disease treated?

There is no cure for CJD or treatments available to control the disorder or slow or stop the progression of the disease. Treatment mainly involves providing symptom reliefs and providing comfort to the patient.

Opiate drugs may be administered to relieve the pain. Medications like Sodium valproate, an antiseizure medication can help relieve or reduce involuntary movements, such as muscle jerking and twitching. In the later stages of the disorder, the patient may be bedridden and unable to perform normal activities or care for themselves on their own. Patients who are bedridden for long periods develop complications like bedsores, which need treatment and preventive measures. Most patients require a caregiver since they aren’t self-sufficient in the later stages. A catheter placed in the bladder will drain the patient’s urine to help the person urinate without having to go to the bathroom. A bedpan may be used by the caregiver to help the patient defecate. Since patients can develop difficulty swallowing, nutrition may be administered through a tube (Ryle’s tube), which is passed through the nose and into the food pipe, a tube may be placed directly into the stomach, or nutrition can also be provided intravenously.

How to prevent Creutzfeldt-Jakob disease?

The following measures can help reduce the risk of acquiring and spreading of CJD:

  • Getting medical treatment in a reputed clinic or hospital where the medical equipment is thoroughly sterilized.
  • Avoiding accepting corneal and skin donations for cornea and skin transplantation, respectively, without knowing the cause of death of the donor.
  • Covering open wounds, cuts, and abrasions on the skin.
  • Wearing gloves, face shield, protective eyewear, disposable gown, or apron while handling human or animal tissue, blood, and fluids.
  • Sterilizing equipment, utensils, or fabrics that are used by CJD patients.
  • Using disposable bed sheets and linens for CJD patients.

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Medically Reviewed on 10/13/2020
References
https://www.cdc.gov/prions/cjd/index.html

https://www.cdc.gov/prions/cjd/about.html

https://rarediseases.org/rare-diseases/creutzfeldt-jakob-disease/

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