Creutzfeldt-Jakob Disease (CJD)

In 1906 a physician, named Alois Alzheimer, cared for a fifty-one year old patient with severe dementia. Upon her death, he was able to examine her brain at autopsy. Dr. Alzheimer was able to take advantage of recent innovations in microscopy and histological techniques that allowed him to study in detail the cellular components in nervous tissue. He found that the brain of his patient had severe cortical atrophy and described the neurofibrillary bundles and plaques that are now the hallmark for definitive diagnosis of what he at that time called “presenile dementia”. An account of his first patient was published in 1907. It is a little ironic that reevaluation this case has lead some to believe that this first patient did not suffer from the Alzheimer’s disease at all. Instead they believe she suffered from a different, rare disease called metachromatic leukodystrophy (Izenberg, 2000).

Classic CJD Creutzfeldt-Jakob disease is a human prion disease. This disease is very progressive and always fatal. Infection with this disease leads to death usually within 1 year of illness. This fatal disorder is believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. CJD occurs worldwide and the estimated incidence in many countries, including the United States,.CJD is classified as a transmissible spongiform encephalopathy (TSE) along with other Prion diseases that occur in humans and animals. In about 85% of patients, CJD occurs as a Irregular disease with no recognizable pattern of transmission. A smaller part of patients (5 to 15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal insomnia. In most CJD patients, the presence of 14-3-3 protein in the cerebrospinal fluid and/or a typical (EEG) pattern, both of which are believed to be diagnostic for CJD .A conformed diagnosis of CJD requires neuropathology or immunodiagnostic testing of brain tissue obtained either at biopsy or autopsy.

Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition falling under the category of Transmissible Spongiform Encephalopathy (TSE) because of having characteristic of spongy degeneration of the brain that it causes and its ability to be transmitted. First it was described in the United Kingdom in March 1996 and it has been connected with exposure to a TSE of cattle known as Bovine Spongiform Encephalopathy (BSE) sometimes called Classical BSE, having been reported first in 1986 in the United Kingdom.

Creutzfeldt Jakob Disease (CJD) is a rapidly progressive form of Dementia. Early diagnosis is important because the underlying cause of Dementia may be treatable. It appears randomly with no apparent reason, about 10% of those who are infected receive the disease through heredity. The use of a computerized axial tomography scan (CT) can help rule out other problems like stroke or brain tumor. The most effective is a Magnetic resonance imaging (MRI), because it can reveal patterns of abnormal brain signals and characteristics of CJD. In rare cases a brain biopsy may need to be performed. A small piece of tissue is removed and it is examined by a Neuropathologist, usually an MRI is sufficient. When a symptom on set happens, individuals may develop confusion, depression, behavioral changes, impaired vision and impaired coordination. As the disease processes there may be a loss of memory, also infected individuals may develop neuromuscular abnormalities. In later stages of the disease, individuals may have further loss of physical, intellectual functions, a coma, and repeated infections of the respiratory tract. Some people have life threatening and life ending complications, that develop less than a year after being

This week may be the last week I write to you. In my last letter to you, you may have noticed that I seemed depressed and not like myself. I wasn’t completely truthful in that letter. I’m not just having a bad day, I’m diseased with an incurable, fatal disease.

Recently in the news has been a story about a patient admitted to a Sydney hospital, suffering from Creutzfeldt-Jakob disease (CJD). You are to write a 2000 word research report on this disease (it can include information on both CJD and VCJD relating to BSE, just make sure you note which one so that I know). In your report you should address the following:

It is a rare, degenerative but fatal brain disorder affecting very a small fraction of persons. The symptoms usually arise at the age of 60 and the person dies within a year. Many researchers believe that this disorder is the result of an abnormal protein known as prion. About 5-10% cases reported in the United States share a genetic basis where this form of dementia is caused by a mutation in the gene for the prion protein. Patients with Creutzfeldt-Jakob disease suffer from the problems associated with muscle coordination, personality changes, impaired memory, judgment making, thinking disability and impaired vision. Other possible symptoms include insomnia and depression. In later stages the persons

Creutzfeldt - Jakob disease is a prion disease characterized by the degeneration of the cerebral cortex, basal ganglia, cerebellum, brainstem and spinal cord. CJD is classified into 4 types: sporadic, familial, iatrogenic, and contaminated meat. CJD represents approximately 85% of all human prion diseases . Although it is common in older ages (50-70), both men and women have an equal chance of acquiring the disease. Recently, there has been a new variant CJD that is thought to be from the ingestion of cattle products in Great Britain. The central characteristic that differentiates vCJD and CJD is that it occurs more commonly in younger people and also has different pathogenic findings. The second variant form is the panencephalopathic form, which is found in Japan.

Creutzfedlt-Jakob Disease(CJD) is a rare, non-treatable, and fatal brain disorder caused by a prion, a pathogen that is smaller than a virus. The causative agent of Creutzfedlt-Jakob Disease is very difficult, almost impossible to destory. The casusative agent is a clear watery fluid that fills the space between the arachnoid membrane and the pia mater. Per year, Creutzfedlt-Jakob Disease effects one in every one million people worldwide, in the United States there are around 300 cases. There are 3 catagories of Creutzfedlt-Jakob Disease; The most common being sporadic CJD(the disease appears even though the person has no common risk factors for CJD), Hereditary CJD, and the least common acquired CJD(caused by exposure to the brain or nervous

Low-dose ketamine is sometimes used in the treatment of complex regional pain syndrome. A 2013 systematic review found only low-quality evidence to support the use of ketamine for CRPS.

Thus we proceed through clinical description and classification, neuropathology, neurophysiology, immunology, and imaging, with a hint of genetics. The continuing critical theme is neuropathology. From the clinician's point of view the development of magnetic resonance imaging has been key to allowing more precise diagnosis as well as surrogate markers for clinical trials. Until very recently there has been no effective treatment, although a wide range of treatments have been used. It is surprising to see that the current use of steroids for acute relapses is quite recent (high-dose intravenous methylprednisolone replacing corticotropin in the early 1980s, following the pattern of usage by rheumatologists), with the first major controlled trial to demonstrate efficacy of the regimen published in 1987. Interferons, discovered in the 1950s, were initially promoted as a treatment for cancer. In 1977 Lawrence Jacobs of Buffalo, NY, was offered a returned supply of interferon (produced from the foreskin of recently circumcized infants). He was initially interested in using this for the rapidly progressive and fatal illness amyotrophic lateral sclerosis (motor neuron disease) but chose to study multiple sclerosis since there were more patients available. The Food and Drug Administration approved the first interferon for treatment of multiple

In the second chapter of the seventh edition Psychology textbook, it discusses biopsychology, neuroscience, and human nature (Zimbardo, Johnson, McCann, 2014). This chapter touches base on the building blocks of the nervous system. Write like one sentence explaining the previous sentence. Within this section, it focuses on demyelinating disorders. Demyelinating disorders occurs when the myelin sheath that protects the central nervous system (CNS) is damaged. Several examples of this type of disorder would include Schilder’s disease, HTLV-1 Associated Myelopathy (HAM), Acute Disseminated Encephalomyelitis (ADEM), Neuromyelitis optica (NMO), Balo’s disease, and Multiple Sclerosis (MS). The most common demyelinating disorder is Multiple Sclerosis

Affecting an estimated 4 million people worldwide (NIH), Parkinson’s is one of the less common nervous system disorders. Despite its rarity, Parkinson’s is largely researched because there is no cure and few effective treatments. It also has a big impact on quality of life for those afflicted. This disease usually shows up after age 40 and occurs when the brain cells that are responsible for motor skills die. Symptoms include tremors, inability to move, and impaired cognitive function, all of which worsen over time. Because this is a condition that affects the brain, if an effective treatment can be established, treatments for other more common conditions such as Alzheimer’s may soon follow. This makes it a critical topic for today’s doctors and scientists.

Alzheimer’s disease is a progressive condition where the neurons degenerate in the brain, while the brain substance shrinks in volume. Alzheimer’s is also the number one cause of dementia. When it was first noticed, Alzheimer’s was thought to be a pre-senile disease, but now it is known to be responsible for seventy-five percent of the dementia cases in people over sixty-five years of age. Alzheimer’s disease usually causes several years of personal and intellectual decline until death. Because there is an increasing number of elderly citizens in the United States, research into the causes and possible cures for the disease is on the rise (1).

CTE is a progressive degenerative disease that is recurring caused by brain trauma and multiple head injuries. The disease attacks the brain through an abnormal protein called Tau which clumps up and spreads throughout the brain killing many brain cells. As Tau kills brain cells, the mass of the brain decreases overtime causing loss of functions. The irreversible deterioration is seen in military veterans and contact sport athletes. Patients experiencing CTE go through depression , aggression, addiction, and memory loss that concludes to other diseases that they are at risk of getting. The most common disease is Alzheimer's which is a form of dementia causing difficulty remembering, confusion, behavior changes,and mood changes. Dementia is a disease causing brain cells to die and get damaged while getting worse overtime showing memory loss. The second most common disease is Parkinson's which includes loss of brain cells that control movement, patients with Parkinson's experience hand shaking tremors and uncontrollable movements. Also Huntington's is a disease that is inherited and passed on by a 50/50 chance of the genes, it is incurable and causes death when their are blood clots due to no