Variant Creutzfeldt – Jakob Disease

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Variant Creutzfeldt – Jakob Disease

Introduction

Variant Creutzfeldt-Jakob disease (vCJD) is an entirely new, fatal, and uncommon illness, usually categorized as Transmissible Spongiform Encephalopathy (TSE). Variant Creutzfeldt-Jakob disease is a prion ailment that was initially described in March 1996 in the United Kingdom (Brandel & Knight, 2018). In addition, vCJD is a form of Creutzfeldt-Jakob disease (CJD) described by cognitive decline and early psychiatric symptoms. All forms of CJD, including vCJD, are part of an uncommon family of progressive neurodegenerative diseases affecting animals and humans. Variant CJD is mainly caused by consuming meat and beef products contaminated with the contagious agent of bovine spongiform encephalopathy (BSE). Also, prion, an abnormal infectious protein, is another cause of vCJD. These prions lead to irreparable harm to the brain’s nerve cells when they reach high levels of accumulation in that brain. Although the aberrant prions technically carry an infectious disease, they are not like viruses or bacteria.

According to Brandel and Knight (2018), Variant Creutzfeldt-Jakob disease usually affects the brain causing brain damage that worsens over time. The spongiform encephalopathy caused by CJD slowly destroys the brain cells and develops microscopic holes in the brain. Variant CJD may remain dormant in a person’s body for a long time before causing illness by destroying the brain cells. Individuals suffering from vCJD face difficulties managing their body motions, enduring changes in speech and gait, and developing dementia. Variant CJD is deadly within a short time, often no more than eight months. Only a few individuals live for one or two years after being infected with the disease. The most common causes of death are heart failure, infections, or respiratory failure.

Statistical Information – Prevalence in the U.S / NYC

Globally, the prevalence of variant Creutzfeldt-Jakob disease is approximately one person per million per year (Sitammagari & Masood, 2018). The prevalence of variant CJD is about 1 to 1.5 cases per 1 million population annually in the United States. This is approximately 350 new cases diagnosed with variant CJD in the United States. However, rates up to two cases per million are not unusual though they are rare. In the Ney York City specifically, the annual incidence and prevalence of variant Creutzfeldt-Jakob disease are approximately 1 per million populations. This indicates that variant CJD has been responsible for the deaths of around twenty people every year, on average, in the New York City (New York State, 2017).

Common Signs and Symptoms of Variant Creutzfeldt – Jakob disease

Variant Creutzfeldt-Jakob disease (vCJD) invariably leads to the patient’s death. On average, the duration of the disease is 14 months. Variant CJD is more likely to strike persons in their early years, with an approximate age of onset of roughly 28 years, in contrast to sporadic CJD, which is more likely to strike individuals in their middle years or later in life. Although it is unknown how much time passes between exposure and the appearance of signs and symptoms, experts believe it might be anywhere from years to decades. After the first sign and symptoms appear, the average person can expect to live for another 13 months. In the early stages, individuals suffering from variant CJD may exhibit the following initial signs and symptoms; painful sensations, behavioral changes such as changes in personality, and major psychiatric problems such as withdrawal, frequent depression, and anxiety (NIH, 2022).

Moreover, during the later stages of variant CJD, infected individuals may exhibit the following signs and symptoms; loss of balance and coordination, involuntary movements such as loss of muscle tone, sudden jerking movements, also known as myoclonus, and twisting, slow, or continued movements such as athetosis, chorea, or dystonia. In addition, according to Brandel and Knight (2018), in later stages of variant CJD an individual might show signs of dementia which is the impaired ability to think, remember, or make decisions that interfere with doing daily activities. Lastly, individuals suffering from variant CJD may exhibit the delayed onset of neurologic disorders, such as ataxia, which might occur within months or weeks.

Standard Diagnostic Measures of Variant Creutzfeldt-Jakob Disease

Neuropathology, which is often performed at autopsy and sometimes performed by brain biopsy, is necessary for a definitive diagnosis of variant CJD. On the other hand, based on the clinical symptoms and the discovery of the pulvinar signs on cerebral magnetic resonance, a clinical diagnosis with a fair degree of plausibility is feasible (Brandel & Knight, 2018). Besides, new tests are being developed, including blood tests that show promise in terms of their specificity and sensitivity for vCJD. These tests which help diagnose variant CJD include; Electroencephalography (EEG), a technique that captures the electrical pattern of the brain and may be very helpful since it reveals a particular sort of abnormality that is present in significant CJD cases but not in all cases. Another test used to diagnose variant CJD is magnetic resonance imaging (MRI). A strong signal in the pulvinar region of the brain is shown in around 90% of cases of variant Creutzfeldt–Jakob disease. Lastly, cerebrospinal fluid-based tests are used to help diagnose variant CJD, which shows very high specificity and sensitivity of vCJD (NIH, 2022).

However, according to Ramanathan (2017), the only way to confirm with certainty that a patient has variant CJD is by examining the tissues of the brain either during life by means of brain biopsy or, more usually, after death through autopsy, the post-mortem examination of the brain. During a brain biopsy, a little tissue sample is taken from the individual’s brain and removed by a neurosurgeon so that a neuropathologist may analyze it. Though this treatment poses a potential risk to the person, it is not guaranteed that tissue will be obtained from the afflicted region of the brain during the surgery. Since an accurate diagnosis of variant CJD does not aid the patient, a brain biopsy should be avoided unless it is required to rule out an illness that can be treated.

Curative Measures of Variant Creutzfeldt-Jakob Disease

Currently, no curative treatment is available for variant Creutzfeldt-Jakob disease (Brandel & Knight, 2018). Although there is now no medication that can control or cure variant CJD, research on a wide range of pharmaceuticals is currently being conducted in order to find one. The current therapy for variant CJD focuses on reducing the severity of a person’s symptoms and providing patients with as much comfort as possible. For example, opiate medicines may help relieve pain, while the medications sodium valproate and clonazepam could help relieve myoclonus symptoms. When the condition has progressed to a later stage, it may also be necessary to provide intravenous fluids and artificial nutrition.

Moreover, symptomatic treatment, palliative treatment, supportive care, or supportive therapies are the only available medical therapy for variant CJD. The goal of symptomatic treatment is typically to lessen the patient’s signs and symptoms to improve their comfort and general well-being. However, symptomatic treatment may also be helpful in reducing the organic repercussions and sequelae of the disease that are caused by these signs and symptoms. Even though there is no current curative measure for variant CJD, to minimize the risk of transmitting variant CJD from one individual to another, individuals who are confirmed or suspected of vCJD or are at a higher risk following their family history of the illness should not be allowed to donate blood components, blood, and tissues. This is because blood components, organs, blood, and tissues donated by infected individuals could transmit infectious prions, thus causing variant CJD (Thomas etal., 2022).

References

Brandel, J. P., & Knight, R. (2018). Variant Creutzfeldt–Jakob disease. In Handbook of Clinical Neurology (Vol. 153, pp. 191-205). Elsevier. https://doi.org/10.1016/B978-0-444-63945-5.00011-8Creutzfeldt-Jakob Disease Fact Sheet | National Institute of Neurological Disorders and Stroke. Ninds.nih.gov. (2022). Retrieved 3 October 2022, from https://www.ninds.nih.gov/creutzfeldt-jakob-disease-fact-sheet.

New York State. Creutzfeldt-Jakob Disease. Health.ny.gov. (2017). Retrieved 4 October 2022, from https://www.health.ny.gov/diseases/communicable/creutzfeldt-jakob/fact_sheet.htm.

Sitammagari, K. K., & Masood, W. (2018). Creutzfeldt Jakob Disease.

Thomas, S., Katz, M., Slower, A. M., Coelho, E., Mallinson, G., Paediatric Components Working Group of the Advisory Committee on the Safety of Blood, Tissues, and Organs, … & Field, S. (2022). Importation of plasma and use of apheresis platelets as risk reduction measures for variant Creutzfeldt‐Jakob disease: The SaBTO review. Transfusion Medicine, 32(1), 24–31. https://doi.org/10.1111/tme.12840Ramanathan, A. (2022). The new method accurately detects prions in blood—Retrieved 3 October 2022, from https://www.nih.gov/news-events/nih-research-matters/new-method-accurately-detects-prions-blood.