Creutzfeldt-jakob Disease

Creutzfeldt-Jakob disease, pronounced as KROITS-felt YAH-Kobe, is a regressive brain disorder that can cause dementia and death.

The symptoms of Creutzfeldt-Jakob disease are similar to those of other dementia-like conditions. Nonetheless, Creutzfeldt-Jakob disease typically progresses more rapidly.

Creutzfeldt-Jakob disease got public attention in the ’90s when few individuals in the UK developed the disease after eating meat from infected cattle.

Despite how severe CJD is, it is still considered rare, and the variant Creutzfeldt-Jakob disease is the least form of the disease. Globally, there’s been an estimated single case of CJD.

Although, severe cases of CJD are uncommon, and variant CJD is still the least common form.

Symptoms

Creutzfeldt-Jakob disease is identified by rapid mental deterioration that occurs typically within a few months.

Early signs and symptoms can include:

  • Memory loss
  • Impaired thinking
  • Blurred vision or blindness
  • Insomnia
  • Difficulty speaking
  • Personality changes
  • Anxiety
  • Depression
  • Difficulty swallowing
  • Sudden jerky movements

The severity of the disease can worsen the mental symptoms, and it’s not unusual to slip into a coma.

Several individuals who suffer from Creutzfeldt-Jakob disease may experience pneumonia, respiratory failure, heart failure, and other infections that eventually lead to death. Death in affected persons usually occurs within a year.

Psychiatric symptoms may be more noticeable in the early stages of individuals suffering from the rarer form of Creutzfeldt-Jakob disease with dementia developing later.

Additionally, the variant form of the disease affects younger people than the Classic form of the disease. It’s also been discovered to have a longer duration ranging from 12 to 15 months.

Causes

CJD and its variants fall under an extensive group of human and animal diseases that are known as transmissible spongiform encephalopathies (TSEs).

This name is derived from the spongy holes that are observable under a microscope. These holes develop in the brain tissue of an affected person.

The cause of CJD and potentially other TSEs appears to be irregular versions of some protein known as a prion. Generally, these proteins would cause no health risk until they are misshapen. They then become infectious that they affect our biological processes.

How is CJD transmitted?

It’s been recorded that the risk of getting infected with the disease is low. It can’t be transmitted through sneezing or coughing, sexual contact, or physical touch.

However, the three ways Creutzfeldt-Jakob disease can be contracted could be:

  • By inheritance: not less than 15% of people with CJD may have a family history of the condition. It is also probable that they tested positive for a genetic mutation linked with the disease. This type is known as familial CJD.
  • Sporadically: several individuals with the classic form of the disease become infected for no apparent reason. Sporadic CJD or spontaneous CJD accounts for many cases.
  • By contamination: few people develop CJD when exposed to infected human tissue during. This could happen during medical procedures such as skin or cornea transplants. Contamination can also occur because standard sterilization methods don’t destroy abnormal prions. This can invariably expose a few people to CJD after undergoing brain surgery involving contaminated instruments.

Few cases of Creutzfeldt-Jakob disease that are surgically related are known as iatrogenic CJD. In comparison, variant CJD is connected to the consumption of infected beef.

Risk factors

Most reports of CJD happen for many unknown reasons, hence making it difficult to identify any risk factors.

Nonetheless, few factors linked with several kinds of CJD can’t be overlooked, and some of them include:

  • Age: CJD may develop in a person typically around the ages of 60 to 65. Signs of familial CJD can manifest a bit earlier, and variant CJD has shown to affect more younger people, usually those in their 20s.
  • Contaminated tissue: Individuals who have received growth hormone from affected people stand the risk of exposure to iatrogenic CJD. However, it is hard to ascertain if the contraction is possible by the consumption of contaminated beef. Generally, if effective health measures are being implemented, then the condition would be easier to control.
  • Genetics: it’s been studied that individuals suffering from familial CJD could be as a result of genetic mutation. This means the disease can be inherited from either parent. Also, if you are infected, the chances of passing it down to your child are about 50%.

Complications

Similar to other dementia, CJD affects the brain, which also affects the body. It’s not uncommon for people who have Creutzfeldt-Jakob disease to withdraw form family and friends before they lose their ability to identify or relate with them.

The disease can also make the sufferer lose their ability to take care of themselves before many slip into a coma. CJD can be fatal and can lead to death.

Prevention

There is presently no known method to prevent CJD. It would be helpful to discuss with a medical doctor or a genetic counselor if you have a family member with any history of neurological complications.

This may help to minimize the chances of developing the condition.

Preventing iatrogenic CJD

Most medical facilities follow strict policies that help to prevent iatrogenic CJD. Some of these measures have included:

  • Selective use of synthetic human growth hormone
  • Proper disposal of surgical tools that have been used on the nervous tissue or brain of an affected person
  • One-time use of kits used for spinal taps

To be sure the safety of blood supply is intact, people with the risk of exposure to vCJD or CJD aren’t eligible to donate their blood. This includes individuals who have:

  • a family member who has been diagnosed with Creutzfeldt-Jakob disease
  • gotten a human growth hormone
  • had a procedure that involved a dura mater brain graft
  • been on bovine insulin since the 80s
  • stayed in parts of Europe for five years or more since the 80s
  • lived in the UK for at least 90 days from 1980 – 1996

The United Kingdom, including many other countries, has specific restrictions concerning blood donations, especially when the donors are people with the risk of exposure to vCJD or CJD.

Preventing variant CJD

The chances of contracting vCJD in the US remains very low. According to the Centers for Disease Control and Prevent, the cases reported so far are just three. It is strongly believed that the cases were gotten abroad.

The United Kingdom has recorded over 200 cases of vCJD, with CJD cases becoming more prominent between 1998 and 2000. Fortunately, there has been a decline since then.

  • A minimal number of other vCJD cases have been reported in different countries nationwide
  • Controlling possible sources of vCJD
  • Several countries have taken measures to prevent BSE-infected tissue from entering the food supply, including:
  • Tight restrictions on the importation of cattle from countries where BSE is common
  • Restrictions on animal feed
  • Strict procedures for dealing with sick animals
  • Testing and surveillance methods for tracking the health of cattle
  • Limitations on the parts of cattle that can be processed for food

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