At present, researchers offer various hypotheses about the cause of Creutzfeldt–Jakob disease (CJD). According to the generally accepted assumption, CJD is triggered by an infectious agent consisting of the host prion protein that subsequently creates beta-pleated amyloid (Baker, Zhi Yun, & Manuelidis 2004, p. 29). Some neuropathologists, for instance, Laura Manuelidis (2013) object to this assumption and argue that this disorder is caused by a virus. Still, the prion hypothesis remains the most widespread explanation.
It is believed that CJD does not elicit innate or adaptive immune responses. Furthermore, as a rule, no inflammatory reactions are detected (Cyngiser, 2008, p. 201). These prions are not recognized by the immune system as foreign bodies (Baker, Zhi Yun, & Manuelidis 2004, p. 29). To some degree, this lack of innate or adaptive immune responses can be explained by the fact that prions can exist in normal and infectious forms (Cyngiser, 2008, p. 200). In turn, the immune system may not distinguish them.
The entry of the prion into the body can take various forms. For instance, an individual can be infected with this disease through contact with immunoglobulins or corneal transplants (Cyngiser, 2008, p. 201). Nevertheless, it is the rarest form of transmission. Additionally, some people can inherit this disease. In part, this result can be explained by the defect of the gene which is responsible for the formation of the prion protein. Finally, there is a sporadic form of CJD which means that the patients were not exposed to any risks associated with CJD. The infectious form of the prion protein causes damage to the brain tissue. The body is unable to decompose the accumulation of prions and they disrupt the functioning of a cell (Cyngiser, 2008, p. 200). It is believed that this disease can be transmitted in several ways. For example, one can mention blood transmission or contact with animals with the bovine form of Creutzfeldt–Jakob disease. Moreover, artificial insemination should is also believed to be a possible route of transmission.
There are several clinical manifestations of the disease. In particular, one can speak about the impairment of short-term memory and the inability of a person to perform even minor complications (Gencer et al. 2011, p. 122). Additionally, one should remember about sleep disturbances and paralysis. Moreover, it is critical to remember that CJD is fatal. A patient can die within several months.
There are several procedures that are involved in the diagnosis of CJD. For instance, one can mention cranial MRI since the increased intensity of the signal cerebral cortex is typical of CJD (Gencer et al. 2011, p. 122). Furthermore, the examination of cerebrospinal fluid can also be used to diagnose this disease (Gencer et al. 2011, p. 122). In particular, the detection of neuron-specific enolase is also typical of CJD. Yet, one should note that these diagnostic tools are still elaborated. The spread of this disease is limited in different ways. In this case, one can mention blood donor restrictions (Puopolo et al. 2011, p. 1557). For instance, those individuals, who received blood transmission in the early eighties, are not allowed to be donors.
References
Baker, C, Zhi Yun, L, & Manuelidis, L 2004, ‘Early induction of interferon-responsive mRNAs in Creutzfeldt-Jakob disease’, Journal Of Neurovirology, vol.10. no. 1, pp. 29-40.
Cyngiser, T 2008, ‘Creutzfeldt-Jakob disease: a disease overview’, American Journal Of Electroneurodiagnostic Technology, vol. 48. no. 3, pp. 199-208.
Gencer, A, Pelin, Z, Kucukali, C, Topcuoglu, Ă–, & Yilmaz, N 2011, ‘Creutzfeldt-Jakob disease’, Psychogeriatrics, vol. 11. no. 2, pp. 119-124.
Manuelidis, L 2013, ‘Infectious particles, stress, and induced prion amyloids’, Virulence, vol. 4. no. 5, pp. 373-383.
Puopolo, M, Ladogana, A, Vetrugno, V, & Pocchiari, M 2011, ‘Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases’, Transfusion, vol. 51. no. 7, pp. 1556-1566.