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Creutzfeldt – Jakob Disease

Atefa Noorain
Atefa Noorain
July15/ 2016


Creutzfeldt – Jakob disease (CJD) is a rare neurodegenerative disorder. It is also called as fatal brain disorder. Creutzfeldt – Jakob disease (CJD) cases are very rare as 1 person in every one million people is known to be effected by it globally. In few places like United States about 300 cases are reported per year. Creutzfeldt – Jakob disease (CJD) mainly affects the people who are above 60 years of age. The survival rate of CJD is less than a year as the patients suffering from Creutzfeldt – Jakob disease (CJD) die within 1 year of onset of infection.

In early stages of disease people experience behavioural changes, lack of coordination, memory loss and visual disturbances. The severity of disease can be understood with pronounced mental deterioration, involuntary movements, extreme weakness, blindness and coma.

Creutzfeldt – Jakob disease (CJD) is a form of transmissible spongiform encephalopathies (TSEs). CJD and TSEs both are caused due to the prion proteins.

Based on severity the disease can be categorized into 3 categories
1.Sporadic Creutzfeldt – Jakob disease (CJD) – Sporadic CJD is the most common type and accounts for almost 85 % of cases.
2.Hereditary Creutzfeldt – Jakob disease (CJD) – 5-10% of Creutzfeldt – Jakob disease (CJD) cases are hereditary. It is seen in people who have a family history of disease and those who are positive for genetic mutation test results.
3.Acquired Creutzfeldt – Jakob disease (CJD) – People who does not have a family history and are reported negative for genetic mutation test have acquired Creutzfeldt – Jakob disease (CJD). Acquired CJD is caused due to medical procedures.

Symptoms

The most common symptoms of Creutzfeldt – Jakob disease (CJD) are

  • Progressive dementia
  • Muscle coordination problems
  • Impaired memory
  • Impaired vision
  • Memory failure
  • Personality changes
  • Involuntary muscle jerks
  • Loss of speaking ability

Symptoms of Creutzfeldt – Jakob disease (CJD) are similar to other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease.

Causes

Creutzfeldt – Jakob disease (CJD) is caused by prion proteins.  Some researchers believe that Creutzfeldt – Jakob disease (CJD) is caused by a slow virus or other organisms. The exact cause of CJD is unknown. Prions that are assumed to be the causative agent change into an abnormal form to cause infection. Abnormal prion proteins aggregate and clump together which leads to the neuron loss and other damages in the brain of CJD patients. Prions are misfolded protein that occurs both in normal form and an infectious form. It is difficult to kill the causative agent whether in a microbe or a protein form. The causative agent does not contain any genetic information in the form of genetic material like nucleic acids (DNA or RNA). Symptoms of Creutzfeldt – Jakob disease (CJD) appear after a long period of time as the causative agent has a long incubation period.

Transmission

Creutzfeldt – Jakob disease (CJD) cannot be transmitted from one person to other through air or by casual contact. Transplanted tissues play a major role in transmitting the disease from person to person. Grafts of dura mater, transplanted cornea, electrode implantation in brain and injections of contaminated hormone are the key factors to transmit the disease into a healthy individual.

Prevention

You need to be careful while undergoing a transplantation surgery of blood tissues.  People who are suspected to have Creutzfeldt – Jakob disease (CJD) should never donate blood tissues or organs. Heath care workers are at high risk of Creutzfeldt – Jakob disease (CJD) as they need to assist the CJD patients. The following precautions need to be taken while dealing with CJD patients.

  • Wear surgical gloves while handling patient’s tissues and fluids.
  • Wear gloves while dressing the open wounds of the CJD patients.
  • Use disposable bedclothes for contact with the patients. A regular cloth soaked in chlorine can be used in absence of disposable materials.
  • Use face protection if there is a risk of splashing of infected fluids like blood and cerebrospinal fluid.

Atefa Noorain
Atefa Noorain

Atefa Noorain, (Microbiologist) former Head of the Department of Life Sciences, Assistant Professor of Microbiology at Sujatha Degree College for Women. She worked as an Associate Research Analyst at Thomson Reuters and an Associate Editor of two research journals. She has presented her research work in 3 International Conferences. She has written articles for national journals and international journals. She had been felicitated as a Young Speaker in the last conference. She has worked on Nanoparticles as a part of her research project and won the Best Scientist of the Day award.

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