Cruetzfeldt Jacob Syndrome (CJD) is a fatal disease affecting humans and is caused by infectious proteins called prions. It belongs to transmissible spongiform encephalomyelitis group of diseases. The disease occurs from the accumulation of prions PrSc that damages the brain cells. CJD can occur in a hereditary form or as an acquired form. The hereditary form results from a dominant mutation affecting prion protein gene (PrP). CJD is a communicable disease. Acquired infection occurs through iatrogenic transmission from hospital. Contaminated instruments used in surgery’s are an important cause for transmission in iatrogenic infection. The disease can also be transmitted through contaminated tissue transplantation. More than 85% of the CJD cases are sporadic in occurrence. Presently, there is no evidence for transmission of disease through contact or from pregnant mother to the fetus. There is also no evidence to support the occupational risk to the disease. (Stonebrook, 2007)
The diagnosis of CJD is confirmed through neuropathological examination, following the autopsy or biopsy. It other causes of subacute dementia are excluded, the electroencephalograph, the presence of 14-3-3 protein in CSF, MRI and Real Time-Quaking Induced Conversion (RT-QUiC), can be diagnostic significance. (Stonebrook, 2007)
People are grouped into two categories based on their risk of CJD: symptomatic patient and asymptomatic patient. Symptomatic patients are those that are actively considered in the diagnosis of CJD, based on the symptom they possess. Asymptomatic risk group, involves those who had undergone a recent brain surgery or transplantation. As per the current evidences, routine noninvasive examination in the clinic, does not pose a risk for CJD. However, those patients undergoing CJD have a risk of acquiring CJD. Handwashing, sterilization of surgical instrument, wearing protective clothing while handling body fluids and tissue and laundry of contaminated bedding linen as recommended by the Department of Health, can help to prevent iatrogenic cases of CJD. (Ruiz-Tovar, 2015)
The following precautions are recommended for prevention of CJD: the trained health care personal must remain aware of the risk of CJD while carrying out invasive procedures and use of body fluids. The staffs are expected to wear protective clothing like an apron, gloves and mask while performing invasive procedure or while transferring fluids. Disposable instruments should be used for procedures like lumbar puncture. Use of disposable instruments is recommended for invasive procedures. The manual handling of contaminated instruments can be avoided. Likewise, minimum number of staff should be employed for performing invasive procedures in the high risk patient. Decontamination of surfaces can be done with 20,000 ppm available chlorine of sodium hypochlorite for 1 hour. 2M sodium hypochlorite for 1 hour is partially effective in disinfecting the surface. The infectious prion protein is highly resistant to decontamination. (Ruiz-Tovar, 2015)
Following the death of CJD patient, the cadaver should be placed in a cadaver bag and labelled as Biohazard, with information on the infectious nature of the cadaver. Infected tissue and body fluid of the infected patient, has the potential to transmit information. As the prions are resistant to disinfection, they tend to persist in the environment for long. For non-disposable instruments used in research, the instruments are reprocessed according to the best practice and return to use standards. Once infected with CJD, there is no cure for the disease. The treatment options that are currently available, aims to alleviate symptom and pain. Paralysis and myoclonus are also noticed towards the later stages of diseases. The symptoms of CJD, may show similarity with diseases like Alzheimer’s, Huntington’s disease and other forms of dementia. Research is still underway to determine routes of prion infectivity and its effective treatment. (Ruiz-Tovar, 2015)
References
Ruiz-Tovar, M. (2015). Review of guidelines for prevention of Creutzfeldt–Jakob disease transmission in medical settings in EU Member States and Norway (1st ed.). Norwaw: ECDC.
Stonebrook, M. (2007). Creutzfeldt-Jakob disease. New York: Nova Biomedical Books.
