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By, Anu Edwin Josefreeda Group III

What is Creutzfeldt-Jakob disease?


CreutzfeldtJakob disease or CJD is a degenerative

neurological disorder that is incurable and invariably fatal.

It is the most common among the types of transmissible spongiform encephalopathy found in humans.

Types of CJD
The three main categories of CJD are sporadic CJD, which occurs for no known reason hereditary CJD, which runs in families acquired CJD, which occurs from contact with infected tissue, usually during a medical procedure

TRANSMISSION

The defective protein can be transmitted by contaminated

harvested human growth hormone (hGH) products, Immunoglobulins (IVIG), corneal grafts, dural grafts or electrode implants (acquired or iatrogenic form: Icjd) The disease has also been shown to result from usage of HGH drawn from the pituitary glands of cadavers who died from CreutzfeldtJakob Disease,. Consuming beef or beef products containing prion particles can also cause the development of classic CJD Cannibalism has also been implicated as a transmission mechanism for abnormal prions, causing the disease known as kuru, found primarily among women and children of the Fore tribe in Papua New Guinea. While the men of the tribe ate the body of the deceased and rarely contracted the disease, the women and children, who ate the less desirable body parts, were 8 times more likely to contract the disease from infected tissue.

CAUSE OF CJD
Transmissible spongiform encephalopathy diseases are

caused by prions. The diseases are thus sometimes called prion diseases. The prion that is believed to cause CreutzfeldtJakob exhibits at least two stable conformations. One, the native state, is water-soluble and present in healthy cells. As of 2007, its biological function is presumably in transmembrane transport or signaling. The other conformational state is very poorly water-soluble and readily forms protein aggregates. People can also acquire CJD genetically through a mutation of the gene that codes for the prion protein (PRNP). This only occurs in 510% of all CJD cases.

CAUSE
The CJD prion is dangerous because it promotes refolding of

native proteins into the diseased state. The number of misfolded protein molecules will increase exponentially,and the process leads to a large quantity of insoluble prions in affected cells. This mass of misfolded proteins disrupts cell function and causes cell death. Mutations in the gene for the prion protein can cause a misfolding of the dominantly alpha helical regions into beta pleated sheets. This change in conformation disables the ability of the protein to undergo digestion. Once the prion is transmitted, the defective proteins invade the brain and are produced in a selfsustaining feedback loop, causing exponential spread of the prion, leading to death within a few months, although a few patients have lived as long as two years.

RISK FACTORS
Most cases of Creutzfeldt-Jakob disease occur for unknown reasons, and no risk factors can be identified. However, a few factors seem to be associated with different kinds of CJD. Age. Genetics Exposure to contaminated tissue.

SYMPTOMS
Rapidly developing delirium or dementia (over

the course of a few weeks or months) Blurred vision (sometimes) Changes in gait (walking) Muscle twitching Hallucinations Lack of coordination (for example, stumbling and falling) Muscle stiffness

SYMPTOMS
Myoclonic jerks or seizures
Nervous, jumpy feelings Personality changes Profound confusion, disorientation Sleepiness Speech impairment

COMPLICATIONS
Infection
Heart failure Respiratory failure Loss of ability to function or care for oneself Loss of ability to interact with others Death

TESTS AND DIAGNOSIS


The exam is likely to reveal such characteristic symptoms as muscle twitching and spasms, abnormal reflexes, and coordination problems. People with CJD also may have areas of blindness and changes in visual-spatial perception. In addition, doctors commonly use these tests to help detect CJD Electroencephalogram (EEG). Magnetic resonance imaging (MRI). Spinal fluid tests. lumbar puncture popularly known as a spinal tap Tonsil biopsy.

TREATMENT
There is no treatment that can cure or control CJD. Researchers

have tested many drugs, including amantadine, steroids, interferon,acyclovir, antiviral agents, and antibiotics. Studies of a variety of other drugs are now in progress. However, so far none of these treatments has shown any consistent benefit in humans. Current treatment for CJD is aimed at alleviating symptoms and making the patient as comfortable as possible. Opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus. During later stages of the disease, changing the person's position frequently can keep him or her comfortable and helps prevent bedsores. A catheter can be used to drain urine if the patient cannot control bladder function, and intravenous fluids and artificial feeding also may be used.

PROGNOSIS
The outcome is usually very poor. Within 6 months or

less of the onset of symptoms, the person will become incapable of self-care. The disorder is fatal in a short time, usually within 8 months, but a few people survive as long as 1 or 2 years after diagnosis. The cause of death is usually infection, heart failure, or respiratory failure.

PREVENTION
People should never donate blood, tissues, or organs if they have

suspected or confirmed CJD, or if they are at increased risk because of a family history of the disease, a dura mater graft, or other factor. Wash hands and exposed skin before eating, drinking, or smoking. Cover cuts and abrasions with waterproof dressings. Wear surgical gloves when handling a patient's tissues and fluids or dressing the patient's wounds. Avoid cutting or sticking themselves with instruments contaminated by the patient's blood or other tissues. Use face protection if there is a risk of splashing contaminated material such as blood or cerebrospinal fluid. Soak instruments that have come in contact with the patient in undiluted chlorine bleach for an hour or more, then use an autoclave (pressure cooker) to sterilize them in distilled water for at least one hour at 132 - 134 degrees Centigrade.