Creutzfeldt Jakob disease

Creutzfeldt-Jakob disease is a type of rare brain disorder which typically leads to dementia and it is also known as CJD. Creutzfeldt-Jakob disease generally comes under the group of human and animal diseases which is commonly known as prion disorders. And then, symptoms of Creutzfeldt-Jakob disease will be similar to those of Alzheimer's disease. Particularly, diseases like Creutzfeldt-Jakob disease symptoms typically get worse faster and it also easily leads to death.

Usually, Creutzfeldt-Jakob disease types are very serious and it is very rare. Among all cases, 1 to 2 cases of Creutzfeldt-Jakob disease are diagnosed 1 million people all over the world each year. This type of disease usually affects older adults.

Symptoms

Creutzfeldt-Jakob disease is encountered by the changes in mental behaviours. This CJD disease symptoms usually gets worse quickly within the range of several weeks to a few months. Symptoms include:

• Personality changes.

• Memory loss.

• Impaired thinking.

• Blurry vision or blindness.

• Insomnia.

• Problems with coordination.

• Trouble speaking.

• Trouble swallowing.

• Sudden, jerky movements.

Because of this disease, death can occur within a year. People who are affected by Creutzfeldt-Jakob disease usually die because of medical issues which are associated with the disease. This disease gives you more troubles which include swallowing, falls, heart issues, lung failure, or pneumonia or other infections.

In the beginning of the disease, people with Creutzfeldt-Jakob disease have changes in mental abilities. In many cases, this disease will lead to dementia  later in the illness. The general symptoms of dementia disease include the loss of the ability to think, reason and remember.

Usually, type called variant CJD affects people at a younger age than the type called CJD. Typically, variant CJD will last upto 12 to 14 months.

Variably protease-sensitive prionopathy (VPSPr) which is another rare form of prion disease.  It can also show other forms of dementia. And it generally causes changes in mental fitness and facing problems with speech and thinking. Usually this type of disease is longer than other types of prion diseases, almost 24 months of range. 

Types of CJD

Generally, in some people, proteins can turn to prions in your body because of various reasons. Those different reasons are usually determined by the type of Creutzfeldt-Jakob disease you have. Type are:

• Sporadic CJD. 

            Sporadic CJD is the most common form of CJD which usually happens in about 85% to 90% of cases. The cause of this type of CJD is unknown.

• Genetic CJD. 

This type of CJD usually happens by gene mutation which you inherit from either one of your parents. It is the second most common cause which causes upto 10% and 15% of CJD cases.

• Acquired CJD. 

           For this type of CJD, you usually get them (acquire) from different sources like medical treatments like organ or tissue transplants and grafts, contaminated surgical equipment, etc.

• Variant CJD (vCJD). 

           This type will happen after eating beef from a cow which is affected by bovine spongiform encephalopathy (BSE). Prions will affect the beef cattle by BSE and then it passes to humans and some other species, causing vCJD.

Causes

Creutzfeldt-Jakob disease and associated conditions caused by changes to a type of protein called a prion. Those proteins are generally produced in the body. When those proteins encounter infectious prions, they fold and form a new shape that’s not general. These affected prions can spread easily  and it affects the process in the body.

Risk Factors

These include:

• Family history 

• Performing or being with at an autopsy procedure who are involved and  exposure to contaminated nervous system tissue

• Surgeon those who are handling contaminated brain tissues

• Those who got transplant like corneal transplant which is clear front part of the eye or meningeal dural tissue transplant that's contaminated

• Those who are involving in preparation of dead body that is contaminated

Diagnosis

Your healthcare professional will diagnose CJD by a combination of methods which includes:

• Physical and neurological exams. In this method, your physician is looking for signs and symptoms of Creutzfeldt Jakob disease and they ask you to do certain tasks, which will help them to identify the problems and they find how your brain functions.

• Diagnostic tests and imaging. In this method, these tests will measure your brain activities or else it creates the images of your brain structure.

• Lab tests. In this step, they analyze the blood or cerebrospinal fluid which surrounds your brain and spinal cord to identify the signs of Creutzfeldt Jakob disease, Particularly helps to identify abnormal proteins and prions. It also involves testing brain tissue after the affected person has died

Treatment

Till now, there are no medications or no curing methods of controlling or slowing its progression. Researchers are looking for several treatment methods for future purposes.

Currently, doctors' goal is to relieve symptoms and make the affected person feel as comfortable as possible.

Medications like Opiate drugs help you to relieve pain. And also, clonazepam and sodium valproate will help you to relieve involuntary movements like muscle twitching.

In the later stages of CJD disease, caretakers will move the affected person often to prevent bedsores. A caretaker will help to drain the affected person's urine, and they will receive proteins and nutrients intravenously.

Prevention

Preventive measure for hospital setting includes:

• You must sterilize all the medical equipment

• Should not accept any cornea donations from people CJD disease

Caretaker of Creutzfeldt Jakob disease should follow some preventive steps:

• You must cover the open wounds, cuts, and abrasions on the skin

• If you handling tissue, blood, or fluid,  you must wear gloves

• You may also wear a disposable gown or clothing

• When there is a risk of splashing contaminated fluid, you should use a face shield, eye protection, or a mask.

• If the sterilized equipment used on or it placed near the person

• You should use disposable bed clothes or else you have to soak the linens in a chlorine solution for at least 1 hour

Researchers continue their research investigating how Creutzfeldt Jakob disease affects the brain and their effort to develop effective treatments.

Frequently Asked Questions

Q1. Is CJD from mad cow disease?

ANs: CJD is not the same as mad cow disease or CWD. All three diseases are in the TSE family and can cause related illnesses and brain lesions.

Q2. How is CJD transmitted?

Ans: In theory, CJD can be transmitted from an affected person to others, but only through an injection or consuming infected brain or nervous tissue. 

Q3. How is CJD prevented?

Ans: There's no known way to prevent sporadic CJD.

Q4. Does CJD show on MRI?

Ans: MRI is a helpful imaging technique for CJD. It usually shows an abnormal signal in the putamen and head of the caudate.

Q5. Can stress trigger CJD?

Ans: Further data indicated that stressful events seem to influence not only the onset of CJD but also the progression of the disease.