What is Creutzfeldt-Jakob disease?
This disease is a debilitating brain disorder that leads to insanity and eventually death. The symptoms of this disease can be similar to abnormalities such as amnesia or Alzheimer’s disease, but the progression of Creutzfeldt-Jakob disease is much faster. Basically, this disease is an increasing and incurable deterioration of the nervous system due to the attack of a type of virus that has been hidden in the body for years. In this article from the section diseasesHello, Dr. Salam, we will thoroughly examine this disease and talk about its symptoms, causes, and ways to diagnose and treat it.
The disease gained public attention in the 1990s, precisely when some people in the UK contracted another form of the disease after eating infected beef, but the classic form of the disease was not linked to contaminated beef. Although the chronic form of this disease is rare, its classic form is somewhat common. Globally, one case of this disease is diagnosed per one million people per year, mostly among adults.
Symptoms of Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease is usually characterized by rapid dementia within a few months, and early symptoms often include:
- Personality changes
- Memory loss
- Loss of ability to think
- Blurred vision or blindness
- Difficulty speaking
- Difficulty swallowing food
- Irregular and sudden movements
As the disease progresses, the symptoms of mental illness worsen. Most of the sufferers of this disease eventually fall into a coma. Heart attack, respiratory arrest, pneumonia and other infections usually cause the death of the affected person, which often happens within a year.
In patients with the classic form of Jacob, symptoms of mental illness may be more pronounced at first, with insanity, loss of ability to think and reason, and memory, which worsen as the illness progresses. Also, compared to the traditional type of this disease, these various symptoms affect the sufferers at a young age, and it seems that its duration is relatively longer, i.e. 12 to 14 months.
This disease and its types are related to a wide group of human and animal diseases known as contagious brain spongy diseases. This name is due to the soft and porous spongy holes that can be seen with a magnifying glass and in the damaged tissues. See the brain grow.
How does the prion protein work?
The cause of this disease and its different types seems to be due to the unusual forms of a type of protein called prion. Usually, these proteins are harmless, but when they are malformed or incomplete, they become infectious and putrefactive and can harm natural biological processes.
Creutzfeldt-Jakob disease risk factors
Most cases of this disease occur for unknown reasons and no risk factor can be identified, but it seems that several factors can be the cause of different types of this disease.
Age: Scattered infection of this disease can occur in old age, usually around 60 years old. The hereditary onset of this disease happens relatively earlier and people get it at a much younger age, usually around the age of 20.
Inheritance: People with hereditary Creutzfeldt-Jakob disease have a genetic change that causes their disease. The disorder is transmitted through asexual chromosomes, which means that you need to inherit an altered gene form from one of your parents to develop the disease. If you also have a gene mutation, the risk of passing it on to your children is 50%.
Genetic analysis of people with the disease due to surgery suggests that hereditary transmission of the prion gene may increase the risk of developing the disease if you are exposed to infected tissue.
Contact with infected tissue: People who have received human growth hormone from human mucous glands or who have had brain tissue transplant surgery may be at risk of developing this disease.
The risk of contracting the disease from eating contaminated beef is difficult to quantify and is greatly reduced if countries are effectively implementing measures to protect public health.
How is Creutzfeldt-Jakob disease transmitted?
The risk of this disease is low and it cannot be transmitted through coughing, sneezing, touching or sexual contact, but it spreads in three ways, which are:
Individually: Most people with the classic form of this disease do not develop this disease for an obvious reason. Spontaneous and individual cases of this disease account for the highest number of infections.
Due to heredity: Less than 15% of patients with Jacob’s disease have a family history of it or have a positive test related to this disease, which is called hereditary Jacob’s disease.
Due to pollution: A small number of people have contracted the disease after coming into contact with contaminated human tissue during a medical procedure such as corneal surgery or skin grafting. Also, because normal disinfection methods do not destroy abnormal prions, a number of people have contracted this disease after brain surgery and contact with contaminated tools.
Patients with Jacob’s disease caused by medical procedures are called surgical patients. Other types of this disease are caused by eating mad cow meat.
side-affects of the disease
Like other causes of insanity, Creutzfeldt-Jakob disease deeply affects the brain and body, although this disease and its variants usually progress more quickly.
People with Creutzfeldt-Jakob disease usually withdraw from friends and relatives and eventually lose the ability to recognize or communicate with them. They also cannot take care of themselves and the fate of many such patients eventually leads to coma and death.
Prevention of Creutzfeldt-Jakob disease
There is no known way to prevent this disease. If you have a family history of neurological diseases, it is better to consult a geneticist so that he can help you about the risks you are facing.
Hospitals and other medical institutions use appropriate methods to prevent this disease due to surgery as follows:
- Exclusive use of synthetic human growth hormone instead of hormone obtained from human mucous glands.
- Disposing of surgical instruments used on the brain or nervous tissues of people who have this disease or are suspected of having this disease.
- Use of disposable devices for back and spine surgeries.
To help ensure the health of the blood supply, people who are at risk of developing Jacob’s disease are not eligible to donate blood, and this includes:
- Those who have had sex with those who have been diagnosed with this disease.
- People who have had brain surgery.
- Those who have received human growth hormone.
- Those who have resided in the UK for at least 3 months from 1980 to 1996.
- People who have spent 5 years or more in Europe since 1980.
- Those who lived for at least 6 months from 1980 to 1990 on US bases located in Northern Europe or from 1980 to 1996 in other parts of Europe.
- Those who have had a blood transfusion in Britain or France since 1980.
- People who have injected bovine insulin since 1980.
The UK and several other countries have also set specific restrictions on donating blood from people who are at risk of developing various types of Jacob’s disease.
Prevention of classic type of Creutzfeldt-Jakob disease
The risk of contracting the disease in the United States is relatively low, and according to the Centers for Disease Control and Prevention, only 3 cases have been reported in the United States that were also of foreign origin, namely 2 in the United Kingdom and 1 in Saudi Arabia. are infected
In the UK, where most of the cases have been reported, less than 200 people have been infected. The outbreak of this disease intensified between 1999 and 2000, but it has decreased since then.
Very few other cases of classical Creutzfeldt-Jakob disease have been reported in other parts of the world.
Development of potential regulation for classical type of Creutzfeldt-Jakob disease
Most countries have taken measures to prevent the entry of contaminated tissues in food as follows:
- Strict restrictions on the import of cattle from countries where Creutzfeldt-Jakob disease is prevalent
- Restrictions on animal feed
- Strict measures regarding the treatment of animals with this disease
- Monitoring and performing the necessary tests to check the health of cattle
- Establishing restrictions on which industries can use cattle meat as food
Creutzfeldt-Jakob disease can be confirmed in a person only after taking tissue from the brain or testing the brain tissue after death, but doctors can diagnose the disease with an accurate diagnosis based on the disease and personal history, neurological tests and special examinations. Do.
These tests are likely to reveal the features of the disease such as muscle contractions and local convulsions, abnormal reactions and coordination problems. Also, people with Creutzfeldt-Jakob disease may have vision problems and changes in seeing objects at a distance.
Doctors also usually use these tests to help diagnose Creutzfeldt-Jakob disease:
Brain test or Electroencephalogram: Using a magnetic pole that is placed on the scalp, this test examines the electrical activity of the brain, and people with various types of Creutzfeldt-Jakob disease show abnormal states.
M.R.I: This imaging method uses radio waves and creates a magnetic field to create clear images of your head and body. This method is especially useful in diagnosing brain disorders because it provides very clear images of the white and gray parts of the brain.
Cerebrospinal fluid tests: Cerebrospinal fluid covers the human brain. In a test called a spinal tap, doctors use a needle to remove a small portion of this fluid for testing, and the presence of a specific protein in this fluid often indicates one of the types of Jacob’s disease.
There is no effective treatment for Creutzfeldt-Jakob disease and its other variants. A number of drugs have been tested, but they have not been useful, and for this reason, doctors have focused their attention on relieving pain and other symptoms to make this disease as bearable as possible for the sufferers.