WASHINGTON, July 28 (UPI) -- The U.S. government laboratory credited with conducting groundbreaking work on human brain disorders similar to mad cow disease soon will be shut down, United Press International has learned.
The lab -- known officially as the Laboratory for Central Nervous System Studies at the National Institutes of Health in Bethesda, Md. -- has been relatively inactive for the past several years and will come to a complete halt at the end of the week. That is when the last remaining member, Dr. Paul Brown, will retire after 40 years of dealing with these mysterious and fatal diseases, known collectively as transmissible spongiform encephalopathies.
TSEs, which include mad cow disease and its human equivalent, Creutzfeldt Jakob disease, destroy the brain and are agonizing for both the unlucky victims and their family members who can only stand by helplessly as loved ones are consumed by the incurable disorder.
The NIH's decision to shut down the lab is confusing to some experts in the field -- including Brown -- particularly so because these diseases are not fully understood and there is no therapy or cure in sight.
"It's been difficult for me to understand," said Brown, who serves as medical director of the CNSS lab. "They're (the NIH administration) simply going in a different direction and want to spend their money elsewhere and they just, for inexplicable reasons, are not interested in promulgating CJD in-house research," he told UPI.
"CJD simply comes under the umbrella of preferring not to deal with it," Brown said, emphasizing that his comments were not meant to be considered sour grapes or criticism. "I'm much surprised, viewed in the interest and importance of this group of diseases, that NIH would choose to go in the direction they've gone, but it is their decision to make."
A panel of 12 experts assembled by the Institute of Medicine agreed with Brown on the importance of keeping an in-house TSE lab operating at the NIH. The panel recommended in a report released late last year (but not officially published until April 2004) that the NIH re-establish a lab similar to the LCNSS, because it would aid in the development of diagnostics and drugs to treat the TSEs, which many scientists think are caused by abnormal proteins called prions.
The institute's 288-page report, "Advancing Prion Science: Guidance for the National Prion Research Program," notes that "the infrastructure for TSE research in the United States is small, aging, and funded at a level below that needed to achieve the research goals ... expeditiously." It adds, "Efforts to re-establish an intramural NIH laboratory on human prion diseases should be encouraged," because "it could better handle issues of infrastructure investment, sustained funding, and investigator security than extramural program in universities and private institutes."
Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the agency is considering the possibility of re-establishing another lab like CNSS, but at present they think they can achieve their TSE research goals by funding outside laboratories at universities.
"I don't think we're losing anything," Major told UPI. "The institute continues to support and consider prion science a very important area," he added.
The NIH supports TSE research internally at the Rocky Mountain Lab in Hamilton, Mont., but this lab tends to focus on the animal TSEs, such as scrapie in sheep and chronic wasting disease in deer and elk, rather than the human forms.
The CNSS lab was instrumental in the 1960's in showing a rare and fatal brain disease known as kuru that occurred in the Fore people in New Guinea was infectious. The work won the lab's first director, Carleton Gajdusek, a Nobel prize in 1976 and helped pave the way for discovering that prions are involved in causing TSEs.
Brown's research helped demonstrate the nearly indestructible nature of prions. In one controversial experiment, he took a sample of prion-containing tissue home with him and buried it in a flower pot in his backyard. The agent was still infectious when he dug it up three years later.
In another study, Brown showed prions could remain infectious even after going through temperatures as high as 1,110 degrees Fahrenheit (600 degrees Celsius). On the preventative side, Brown developed an ultra-high pressure method of inactivating prions in processed meat, which could help ensure it is safe from contamination with mad cow disease, but this technique has not yet been employed by meat companies.
At the same time the NIH is moving to shut down the CNSS lab, new discoveries about TSEs are being made in other parts of the world.
Although it appeared the outbreak of variant CJD -- the form of CJD humans can contract by consuming meat infected with the mad cow pathogen -- was declining in the United Kingdom, a recent study indicated as many as 3,800 people may be unwittingly infected, but not yet showing symptoms. It also was reported recently that a second person had acquired vCJD through a blood transfusion, raising concerns about how many additional people worldwide received infected blood and might be at risk of having contracted the disease.
TSEs also are presenting problems in the United States. The first confirmed case of mad cow disease was detected in Washington state last December and U.S. Department of Agriculture officials have said they expect to find additional cases as they ramp up the number of cows tested for the disorder. Also, chronic wasting disease is spreading in deer and elk in the midwestern United States and Canada and researchers are concerned it could infect humans --although no confirmed cases have been detected so far.
Dr. Richard Johnson, a neurologist at the Johns Hopkins University School of Medicine in Baltimore and chair of the committee that developed the recommendations in the Institute of Medicine report, said the time-limited grants from the NIH -- they generally cover three-year increments -- and the limited funding available for TSE research is "a very strong disincentive for people to go into this work," because prion research is expensive and it can take several years to run one experiment due to the long incubation time of these diseases.
"The solution for that would be for the government to establish a lab within NIH that would concentrate on human diseases," similar to the CNSS lab, Johnson told UPI. He noted the Centers for Disease Control and Prevention's Rocky Mountain lab in Hamilton, Mont., is not a suitable surrogate, because it traditionally has concentrated on the animal forms of these diseases.
Dr. Bruce Chesebro, chief of the Laboratory of Persistent Viral Diseases at the Rocky Mountain lab, said he agrees "completely" with the recommendations in the institute's report for maintaining an in-house lab at the NIH headquarters in Bethesda, Md.
This would help foster clinical research for possible treatments, Chesebro said. The Rocky Mountain lab is interested in the human side of these diseases but they don't have a hospital, so a facility in Bethesda, where there is a hospital and clinical research experts, would help encourage that type of research, he said.
At the moment, that type of research is sorely needed. An NIH database of ongoing clinical trials for experimental therapies does not contain a single study for CJD, an indication there is no promising therapy on the horizon for this fatal disease.
Steve Mitchell is UPI's Medical Correspondent. E-mail firstname.lastname@example.org