Tuesday, 13 Nov 2018

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Patients with rare brain disease; abnormal proteins found in their skin

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Samia haque: 24 November, 2017: Prions- the abnormal proteins widely believed to cause a rare brain-destroying disease. According to a study published on Wednesday, scientists found this protein in the skin of 23 patients who had died from this brain disease.

The discovery proposed that skin samples might be used to improve detection of the disorder, Creutzfeldt-Jakob disease, which now is usually diagnosed with much more difficult procedures, like brain biopsies or autopsies.

The presence of prions in the skin also raises the unsettling questions about whether the medical instruments could be contaminated even during surgery that does not involve the brain. And then spread the disease to other patients. The prions stick to stainless steel and are really hard to destroy.

 According to National Institute of Health, Creutzfeldt-Jakob disease affects one person in a million worldwide. 300 cases a year in the United States. 60 is the typical age when this disease usually begins to show. It is cruel and rapidly fatal: Most patients die within a year of becoming ill.

They deteriorate mentally, weaken, move uncontrollably, and may become blind and unable to speak. The findings do not mean that Creutzfeldt-Jakob disease can be transmitted by touch or casual contact, said the senior author of the study, Dr. Wen-Quan Zou, at Case Western University School of Medicine. Patients are not dangerous, he emphasized.

Most cases of Creutzfeldt-Jakob, about 85 percent, are “sporadic,” meaning they strike out of the blue in people with no risk factors or family history. Another 5 percent to 10 percent of cases are hereditary, caused by a genetic mutation.

Kurt Giles, at the University of California, San Francisco, said that if this finding led to a new means of diagnosing the disease, that would be valuable. He said, there is now no treatment, but if one were to become available, it will be important to correctly identify patients as early as possible.