Term Paper on CJD
Creutzfeldt–Jakob Disease (CJD) refers to a type of neurodegenerative disorder that usually occurs and progresses rapidly. According to Al-Ansari and Robertson, this illness is characterized by the conformational alteration of prion protein and assumes an abnormal form that eventually spreads itself to affect the parts of the human brain (1029). The condition is known to have devastating effects as it destroys the brain cells by causing some tiny holes. In most cases, persons with this illness experience ataxia, or the inability to control the movement of their bodies, leads to the formation of an enormous gait, affects speech, and causes dementia (Kraft par 1). Further, Kraft asserts that CJD is a fatal condition considering that it has no cure (par 2). It is estimated that CJD affects at least one in every million persons yearly (Kraft par 3). Though it may appear to be a rare condition, it is vital to understand the history, symptoms, and prognosis of this illness since it has severe implications on human health.
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History and Prevalence of CJD
The history of this condition dates back to 1990s. According to Mayo Clinic, this condition came to the limelight of the public at this period followed the outbreak of variant CJD (vCJD) in the United Kingdom (par 2). The condition is understood to have developed after the victims had consumed meat from diseased cattle. However, Mayo Clinic asserts that “classic” CJD disease has not been entirely linked to contaminated beef (par 2).
Next, the condition could be classified as rare following its limited prevalence worldwide. The illness affects one person in every million yearly, which also includes the US population (Kraft par 3). It is also understood that people aged above sixty are the most vulnerable to the disease. This makes it rare to occur in people aged below thirty (Kraft par 4). However, despite the rare occurrence, the latest studies indicate that there has been a gradual increase in the condition. According to Mackenzie and Will, more case of mortality from the illness has been reported in the UK and in many other nations that conduct a systematic analysis of CJD (4). The changes in the prevalence rate may be attributed to alterations in the population demographics, the introduction of more sensitive diagnostic investigations, and improved case ascertainment following the high levels of awareness regarding the condition (Mackenzie and Will 6). After the symptoms of the CJD have appeared, it is hard for the victim to survive for more than a year. Thus, considering that only one in a million people could be affected yearly, the condition is rare.
Signs and Symptoms of CJD
Though the condition tends to appear later in life, the approximate age when the onset of the symptoms could be detected is fifty-five to seventy-five (Vacca 36). After the appearance of the first symptoms, the patient can only live a median of 4.5 months. The early signs of the condition are the development of personality and behavioral changes (Vacca 36). One may also experience a failing memory, unstable gait, myoclonus, and difficulties with vision (Vacca 36). With myoclonus, a characteristic feature of sporadic CJD (sCJD), the patient is likely to experience some muscular jerk-like episodes, which occur irregularly and last for short durations.
Prognosis/Causes of CJD
The condition occurs typically when a prion protein, a type of abnormal amyloid protein, causes other proteins to develop some abnormalities. As a result, the buildup of these abnormalities will eventually lead to the malformation of prions in the brain cells, which results in fatal damage to the brain (Kraft par 10). The causes may be sporadic, inherited, or acquired. According to Kraft, sporadic CJD is the most common condition, representing 85% of the reported cases (par 12). This particular type of CJD has no apparent risk factors. Besides, inherited CJD only accounts for 5-10% of the reported instances globally (Kraft par 13). The process may occur through mutation of egg or sperm cells, which makes the offspring vulnerable to the condition. In acquired CJD, no particular evidence has proved that the illness can be passed from one person to another (Kraft par 17). However, procedures like corneal transplant, electrode implants, dura mater graft, and use of human growth hormones are understood to transmit this condition (Kraft par 18).
Treatment and Prevention of the Disease
Currently, there is no known cure to CJD. However, studies are ongoing to at the National Prion Clinic to establish a possible cure to the illness (NHS UK par 1). The viable treatment options available at the moment are keeping the patients comfortable and minimizing the conditions’ symptoms with medicines. For example, sedatives and antidepressants may be utilized when treating those patients who have developed psychological symptoms like anxiety and depression (NHS UK par 3). Medicine like clonazepam, may be used to treat those patients suffering from muscle jerks or tremors. The pain from CJD may be suppressed with powerful opiate-based painkillers.
Again, there is no known way of preventing the occurrence of sporadic CJD. If one realizes that his/her family has a history of suffering from this condition, it is advisable for such a person to visit a genetic counselor for further guidance (Mayo Clinic par 20). However, iatrogenic CJD can be prevented by following explicit policies in hospitals and medical institutions. For example, it is vital for such institutions to exclusively use synthetic human genome and destroy all surgical instruments used on the brain or any other nervous tissues (Mayo Clinic par 22). Lastly, vCJD may be prevented by imposing tight restrictions on importation of cattle from nations prone to the condition, introducing some restrictions on animal feeds, and implementing testing methods that could tract cattle health.
To summarize, CJD is a neurodegenerative disorder that generally occurs and progresses rapidly. It is a severe illness since it can kill the victims in less than a year. However, the condition is rare following its limited prevalence worldwide. The disease occurs when a prion protein causes other proteins to develop some abnormalities. Though there is no known treatment, good health practices for the patients are always recommended. Besides, some preventive measures like following explicit medical policies and tight restrictions on importation of cattle may help to curb its spread. Therefore, though CJD is rare, it is vital to understand the history, symptoms, and prognosis since it has severe implications on human health.
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Al-Ansari, Aseel, and N P Robertson. “Creutzfeldt-Jacob Disease: New Directions in Diagnosis and Therapeutics.” Journal of Neurology, vol. 264, no.5, 2017, pp. 1029-1031.
Kraft, Sy. “Creutzfeldt-Jakob Disease (CJD): Symptoms, Causes, and Treatment.” Medical News Today, 26 Apr. 2017, www.medicalnewstoday.com/articles/185884.php. Accessed 15 July 2019.
Mackenzie, Graeme, and Robert Will. “Creutzfeldt-Jakob Disease: Recent Developments.” F1000Research, vol. 6, no.2053, 2017, pp. 1-9.
Mayo Clinic. “Creutzfeldt-Jakob Disease – Symptoms and Causes.” Mayo Clinic, 4 Oct. 2018, www.mayoclinic.org/diseases-conditions/creutzfeldt-jakob-disease/symptoms-causes/syc-20371226. Accessed 15 July 2019.
National Health Service UK. “Creutzfeldt-Jakob Disease – Treatment.” Nhs.uk, 23 Oct. 2017, www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/treatment/. Accessed 15 July 2019.
Vacca, Vincent M J. “CJD: Understanding Creutzfeldt-Jakob Disease.” Nursing, vol. 46, no.3, 2016, pp. 36-42