Health managers of the County of Hood River, Oregon, study three cases of a rare and fatal brain disease known as Creutzfeldt-Jakob's disease.
Two people died and a third person has symptoms compatible with the disease. The disease was confirmed in one of the deceased by an autopsy; The other two cases are considered probable, according to a press release from the County Department of Hood River.
The three cases have been diagnosed in the past eight months. County health officials refused to provide details on individuals, such as their age, sex or city of residence.
“Currently, there is no identifiable link between these three cases,” said a press release from the county of Hood River. The county has a population of around 24,000 inhabitants.
Creutzfeldt-Jakob's disease is a neurodegenerative disorder caused by poorly folded proteins called prayer. These prayers lead to rapid deterioration of the brain, resulting in serious neurological symptoms and death. Although the disease is known for its sporadic event, the clusters arouse concerns among public health officials concerning potential environmental or food exposure.
Symptoms include problems of memory, walking, coordination, speech and behavior changes, according to experts. It does not spread in air, water, contact or social contact, according to the health officials of the county of Hood River.
The disease is considered incurable and is still fatal. About 350 cases are diagnosed in the United States each year, According to the National Institutes of Health.
The disease is rare in people, affecting around 1.4 people per million. However, as the disease takes years to develop, the chance of anyone to develop the disease is closer to 1 in 5,000 or 6,000, said Michael Geschwind, professor of neurology at UC San Francisco in the Center for Memory and Aging.
The disease is similar to chronic waste of waste, or CWD, which is also a disease powered by Prion, and has been detected for the first time in wilderness in California and Washington last year.
CWD was the first Reported in 1967, in a captive deer in Colorado. He has spread since deer in 36 states. There is no known case of disease in Oregon fauna.
For decades, CWDs were feared to be able to move through human populations by ingestion of contaminated meat.
Indeed United Kingdom, France and elsewhere had become infected with crazy cows or a spongiform bovine spongiform.
Since then, public health officials of Centers for Disease Control and Prevention and several states have paid particular attention to the clusters of Creutzfeldt -Jakob's disease – investigating epidemics to determine if deer, wapitis or infected moose meat was involved.
They – and researchers from other agencies, such as the National Wildlife Health Center of the US Geological Survey – also monitored the populations of wild deer and have kept an eye on the hunters who could be exposed.
Although geographically widespread in the United States and Canada, the disease is considered relatively rare in the wild populations of deer, wapitis and moose, said Brian Richardson, the coordinator of the emerging disease of the USGS Wildlife Center.
“It may be (in Oregon), but it is difficult to find rare events,” he said.
To date, there is no known incident of people who acquire Prion disease by consuming deer, elk or original meat, said Geschwind, the UCSF teacher.
He said that around 85% to 90% of Creutzfeldt-Jakob cases are considered to be sporadic, without cause or source of infection identified. In 10% to 15% of cases, the disease seems to be genetically inherited – some people acquiring mutations associated with the disease.
However, there have been a few cases in which sources of infection or contamination were identified, and almost all came from a medical accident.
Prayers are notoriously difficult to inactivate or destroy – withstand standard sterilization techniques – And can remain intact for months and years on a surface, said Geschwind.
In a small number of cases, he said, people have acquired the disease following contaminated and poorly cleaned surgical equipment. In some other cases, it was acquired by people who used products – like the growth hormone, or which have received corneal transplants – inadvertently derived from infected corpses.
It is the sustainability and longevity of these proteins that are worried by many researchers. Studies have shown that deer shelters the disease can pass infectious prayers to other deer by saliva, blood, urine and excrement.
“So, if the animal licks a plant or licks a licking of salt, for example, and that another animal presents itself and lick that the plant or salt licked, then it could be a way to propagate the disease,” said Geschwind.
In addition, the body decaying a dead deer of the disease can infect the surrounding environment where the animal has expired – potentially contaminating plants, seeds, fungi and soil, said Richardson.
He said that not only is the question of surface contamination, but research has also shown that proteins can “be discussed by rootlets and deposited in air plant tissues. If these plants contribute to the transmission of chronic disease and what type of risk pose that humans remain open questions. ”
Geschwind noted that the work carried out by federal researchers to better understand the disease, to provide diagnostic autopsies on alleged cases, to monitor fauna and investigate the clusters provided a level of protection to the American public, which could be destabilized by cuts proposed to federal agencies.
“The idea of reducing the funding of the government of rare diseases is very myopic, because even if the CJD is a rare disease, what we have learned from Prion diseases has implications for all neurodegenerative diseases,” he said, noting Alzheimer's disease, front-staked dementia, Parkinson's disease and multiple atrophy.
“All these diseases act in a manner like a prion in which normal proteins have misplaced, and these poorly folded proteins bring cells not to work in part and to lead to the disease,” he said. “But the basic mechanism we have learned from this very rare disease applies to diseases that are thousands of more common times. To get rid of research? It would be a very serious mistake. “
