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Research Frontiers Part 4
Neurology at Washington University
There are 400 people who work in the neurology department at Washington University. Dr. Tabbal, one of 40 clinicians at the Center, is also founder of the first HD clinic at the University of Arkansas. He is proud of the university's prestige in the field: Washington University receives between 6 and 7 percent of the annual funding given by the National Institutes of Health (NIH), or roughly 22-23 million dollars.
Remarkably, the Positron Emission Tomography (PET) scanner was invented at Washington University. Although the original scanners were enormous, the ones used today are only slightly larger than the subjects that go in them, usually people and larger animals. PET scanners are a major research tool at the Center, something that distinguishes Washington University from other research labs.
Before PET was invented, researchers could only infer information about the brain through dissections after death and animal studies. PET allows researchers to track the function of the brain of a living, alert subject. To do this, a very small amount of a radioactive substance that is similar to a substance already found in the body is injected. As any radioactive substance decays, it emits particles called positrons. The scanner then picks up these positrons as the radioactive marker in the subject's brain decays.
The specific kind of marker used depends on the area or function of the brain being studied. For example, a researcher studying glucosemetabolism in the brain will inject a substance similar to a normal glucose molecule, but with an added radioactive fluorine atom, called TRIUMF. To study blood flow to various areas of the brain, radioactive water or 6-F-dopa is injected. The amount of radiation a subject is exposed to is typically less than he or she would be exposed to during an X-ray. Unlike X-rays, however, which produce only a shadow image, PET scans allow doctors and researchers to track changes in the subject's brain as various cognitive tasks are performed.
Information gained from PET scans and genetic testing have contributed immensely to the growing body of knowledge on HD. Since 1992, when genetic testing became available for HD, scientists have been "learning exponentially" about the condition. Dr. Tabbal cautions that this knowledge must be used wisely.
"[Pharmaceutical companies] have a library of compounds," says Dr. Tabbal, "Many are not FDA regulated. They are also expensive and ineffective, and we have little knowledge of their long-term effects. The difference between Western medicine and sorcery is that with Western medicine, we do research and experimentation to find out if there is validity to a drug's proclaimed effect. Unfortunately, most of these drugs are more witchcraft than medicine."
A drug that has recently gained publicity is Coenzyme Q-10. Q-10 supposedly prevents changes in nerve cells' oxidation-phosphorylation, a normal metabolic process. These changes are believed to contribute to apoptosis of nerve cells, or nerve cell death. In clinical trials, only doses of 1200 mg have been shown to have any marked effect on persons with HD. Even then, the benefits are few. Dr. Tabbal points out that many people still pay a lot of money for doses of 50-100 mg.
According to Dr. Tabbal, the research conducted at the Washington University neurology department focuses on phenomenology, which means putting a great emphasis on understanding the process of disease rather than merely recording results. Otherwise, treatment becomes a shot in the dark.
Dr. Tabbal jokes, "There are more supporting staff [like Melinda] than doctors in the Movement Disorders department." This high ratio of supporting staff to doctors is for a good reason. Like Melinda, Dr. Tabbal believes that HD is mainly a psychiatric disease which requires counseling as much as, if not more than, medical intervention to treat.
Dr. Tabbal is a friendly, upbeat doctor who has come close to experiencing the effects of HD firsthand. Every summer, he participates in a five-day camp for people with HD at Columbia University. Everything at the camp caters to people living with HD: the cabins are ergonomically designed and the daily schedule is highly structured and detailed.
"When you are surrounded by people with Huntington's, you become the odd one," Dr. Tabbal says. "It is the only way you can really understand what's it's like. You understand why it takes so long to dress, how they fall."
Dr. Tabbal believes that a good support system, physical therapy, relevant medicines, and the right attitude should be the foundation of treatment. He believes that there is hope after a diagnosis of HD. People at risk for HD should not be afraid of what the doctor might say because, after all, the diagnosis is the first step in treatment. Dr. Tabbal adds his final piece of advice with a smile, "Make sure you see a good doctor."
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