Which of the following is true of Creutzfeldt Jakob disease
Many different diseases of animals and humans are caused by the pathological form of the prion protein (PrP). In animals, these disorders include scrapie of sheep and goats, "mad cow" disease,
and chronic wasting disease of elk and deer. In humans, these maladies include kuru, Creutzfeldt–Jakob disease (CJD), Gerstmann–Sträussler–Scheinker disease (GSS), and fatal familial insomnia (FFI). Once formed, pathological PrP promotes the misfolding of the normal prion protein into a diseased state. This self-propagating process allows for the exponential increase and accumulation of misfolded PrP in cells, resulting in a disruption of cell function and ultimately cell death. Prion diseases comprise several conditions. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases can affect both humans and
animals and are sometimes spread to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). Prion diseases are rare. About 300 cases are reported each year in the U.S. Types of prion diseases include:
What causes prion disease?Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clump in the brain, causing brain damage. This abnormal accumulation of protein in the brain can cause memory impairment, personality changes, and difficulties with movement. Experts still don't know a lot about prion diseases, but unfortunately, these disorders are generally fatal. Who is at risk for prion diseases?Risk factors for prion disease include:
What are the symptoms of prion diseases?Symptoms of prion diseases include:
How are prion diseases diagnosed?Prion diseases are confirmed by taking a sample of brain tissue during a biopsy or after death. Healthcare providers, however, can do a number of tests before to help diagnose prion diseases such as CJD, or to rule out other diseases with similar symptoms. Prion diseases should be considered in all people with rapidly progressive dementia. The tests include:
How are prion diseases treated?Prion diseases can't be cured, but certain medicines may help slow their progress. Medical management focuses on keeping people with these diseases as safe and comfortable as possible, despite progressive and debilitating symptoms. Can prion diseases be prevented?Properly cleaning and sterilizing medical equipment may prevent the spread of the disease. If you have or may have CJD, do not donate organs or tissue, including corneal
tissue. Living with prion diseasesAs prion diseases progress, people with these diseases generally need help taking care of themselves. In some cases they may be able to stay in their homes, but they eventually may need to move to a care facility. Key points about prion diseases
Next stepsTips to help you get the most from a visit to your healthcare provider:
What is CreutzfeldtCreutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein.
How do you catch CreutzfeldtIs CJD contagious? In theory, CJD can be transmitted from an affected person to others, but only through an injection or consuming infected brain or nervous tissue. There's no evidence that sporadic CJD is spread through ordinary day-to-day contact with those affected or by airborne droplets, blood or sexual contact.
What does CreutzfeldtCJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells. While the abnormal prions are technically infectious, they're very different from viruses and bacteria.
What is the diagnosis of CreutzfeldtThe only way to confirm a diagnosis of CJD is to examine the brain tissue by carrying out a brain biopsy or, more commonly, after death in a post-mortem examination of the brain.
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