What Is A Creutzfeldt Jakob Disease?

March 22 20:47 2019 Print This Article

It is defined as a disorder in which there is a viral infection caused by the involvement of nerves. It involves the brain and is also known as mad cow disease. It cannot be cured and may lead to death. It is quite common transmissible spongiform encephalopathy. It is found in humans.

What are the signs and symptoms of Creutzfeldt jakob disease?

It includes the loss of memory, dementia and the changes in personality. It may also lead to hallucinations. There are physical problems also such as the problem in speech, coordination as well as balance, changes in the gait, posture as well as seizures. There are jerky movements also. The duration of disease is not constant and it can lead to death within few weeks or months. The symptom can continue for a long time. There are involuntary movements and one can do take electro encephalogram for diagnosis. It may lead to the death of nerve cells in the brain. The abnormal proteins are formed. When the brain is examined with the help of a micro scope it shows small holes in which the nerve cells have died. The spongiform denotes to the spongy look of brain.

What are the causes of Creutzfeldt jakob disease?

It includes the main causative agent which is the prion. They are also known as prion disease. The prion has stable conformations. It is water soluble and has a healthy state and some of the state is not water soluble and the protein accumulates. The other causative factor can be the mutation occurring in the gene. It is seen in one tenth of cases. The prion can lead to refolding of the native protein. The protein which is not stable increases in the size and there is a formation of large number of prions which are not soluble. They affect the function of cell and may lead to the death of cells. The mutation in the gene can also lead to this type of trouble. With the transmission of prion there is invasion of protein in the brain and it leads to feedback loop which is self sustained. It leads to the spread of prion and leads to death in few months. A few patients survive for few years.

How Creutzfeldt jakob disease can be diagnosed?

It includes the use of visual examination and a patient history. There are involuntary movements and one can do take electro encephalogram for diagnosis. It may lead to the death of nerve cells in the brain. The diagnosis is done with the help of signs as well as symptoms. One can take cerebero spinal fluid for analysis. The MRI of brain must also be done. One can also go for the diffusion weight images which are the most sensitive. They show the cortical as well as sub cortical problems. In few cases thalamus is also involved. The diagnosis can also be established by the symptoms along with a physical examination.

The tumor marker shows an increase in the case of this disorder. It is neuron specific enolase. There is a deposition of the prion protein which is found in the spleen as well as skeletal muscle. One can confirm the biopsy of this tissue which involves the brain. Biopsy is not the only criteria which confirm the diagnosis. In the microscope one sees changes in the histology with the presence of round vacuoles which are large in number. They are present in the neutrophil. It is present in all the layers of cortex. There is a loss of neurons and there is an accumulation of the plaque in new form of this disease. The vacuoles are formed in the other conditions also. The small vacuoles have the ability to combine and form large vacuoles.

How Creutzfeldt jakob disease can be treated?

There is a medicine known as pentosan poly sulfate which is used to treat interstitial cystitis. The drug is infused in the patient in the lateral side of brain. This drug does not stop the process of disease but it loses the brain function as well as the tissue. The treatment increases the survival period and this has been established in the case of studies done in animals. The progress of disease can also be checked by the interference in RNA. It hampers the production of protein and the prions are formed. The other useful drugs in this case can be amphotrecin B and doxorubicin. But the evidence has not been proved yet. There are few anti malarial drugs which have been found to be quite effective.

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