Huntington's Disease Quotes

James Parkinson. George Huntington. Robert Graves. John Down. Now this Lou Gehrig fellow of mine. How did men come to monopolize disease names too?

James Parkinson. George Huntington. Robert Graves. John Down. Now this Lou Gehrig fellow of mine. How did men come to monopolize disease names too?” I blink and my mother blinks back, and then she is laughing and so am I. Even as I crumple inside.

Huntington’s disease.

There is a significant hereditary contribution to ADD but I do not believe any genetic factor is decisive in the emergence of ADD traits in any child. Genes are codes for the synthesis of the proteins that give a particular cell its characteristic structure and function. They are, as it were, alive and dynamic architectural and mechanical plans. Whether the plan becomes realized depends on far more than the gene itself. It is determined, for the most part, by the environment. To put it differently, genes carry potentials inherent in the cells of a given organism. Which of multiple potentials become expressed biologically is a question of life circumstances. Were we to adopt the medical model — only temporarily, for the sake of argument — a genetic explanation by itself would still be unsuitable. Medical conditions for which genetic inheritance are fully or even mostly responsible, such as muscular dystrophy, are rare. “Few diseases are purely genetic,” says Michael Hayden, a geneticist at the University of British Columbia and a world-renowned researcher into Huntington’s disease. “The most we can say is that some diseases are strongly genetic.” Huntington’s is a fatal degeneration of the nervous system based on a single gene that, if inherited, will almost invariably cause the disease. But not always. Dr. Hayden mentions cases of persons with the gene who live into ripe old age without any signs of the disease itself. “Even in Huntington’s, there must be some protective factor in the environment,” Dr. Hayden says.

People with Parkinson’s are very lucky to have L-dopa. There is no equivalent therapy for other neurodegenerative conditions like Alzheimer’s, Huntington’s, or Lou Gehrig’s disease. Whatever its limitations, L-dopa turned Parkinson’s from a condition in which victims experienced a rapid slide toward immobility and death into a chronic disease with a gradual trajectory of decline. A

ASPARTAME AND MSG: EXCITOTOXINS Aspartame is, in fact, an excitotoxin, one of a group of substances, usually acidic amino acids, that in high amounts react with specialized receptors in the brain, causing destruction of certain types of neurons. A growing number of neurosurgeons and neurologists are convinced that excitotoxins play a critical role in the development of several neurological disorders, including migraines, seizures, learning disorders in children, and neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and amyotrophic lateral sclerosis (ALS).1 Glutamate and aspartate are two powerful amino acids that act as neurotransmitters in the brain in very small concentrations, but they are also commonly available in food additives. Glutamate is in MSG, a flavor enhancer, and in hydrolyzed vegetable protein, found in hundreds of processed foods. Aspartate is one of three components of aspartame (NutraSweet, Equal), a sugar substitute. In higher concentrations as food additives, these chemicals constantly stimulate brain cells and can cause them to undergo a process of cell death known as excitotoxicity—the cells are excited to death.

There are a small number of debilitating conditions with a strong genetic basis, such as muscular dystrophy or Huntington’s disease. These are rare, affecting about one person in ten thousand or even fewer. They do not pose a significant threat to the survival of the species. If, however, we add up the numbers of people plagued by depression or ADD or the other common psychological problems people in this society struggle with, including alcoholism and anxiety, we will have identified no less than a third of the North American population. Genetic explanations for these conditions assume that after millions of years of evolution, nature would permit a very large number of disordered genes, handicapping a third of humankind, to pass through the screen of natural selection — a highly unlikely proposition.

Of course there is no doubt that some diseases, like Huntington’s chorea, beta thalassemia, and cystic fibrosis, can be blamed entirely on one faulty gene. But single-gene

In the years to come, some of our best minds will try to dig deeper into that computer program, to figure out its individual lines of code (the IF-THENS that we call genes), the products of those lines (what we call proteins), how all those lines of biological code fit together, and how they make room for nurture. In the long run, the effects on society will be profound. Take, for example, the advances that our increasing understanding of genes will lead to in medicine. Because, as we have seen, the brain is built like the rest of the body, it is also amenable to many of the same types of treatment. For example, stem cell therapies originally developed for leukemia are being adapted to treat Parkinson's disease and Huntington's disease. Gene therapies developed for cystic fibrosis may someday help treat brain tumors. Both work by harnessing the body's own toolkit for development.

Despite the availability of testing, at least half of the population at risk for Huntington’s disease still has children without making use of the new technologies. Even some of the people who have prenatal testing for Huntington’s still have a profound reluctance to learn their own status. Couples who try preimplantation genetic diagnosis may even conceive a child and choose not to find out if the parent at risk has the mutation. Deciding

According to at least one study, that would be the wrong decision. Researchers followed a sample of young adults who had a 50 percent chance of getting Huntington’s disease and who agreed to take the genetic test. The participants completed measures of depression and psychological well-being before they knew the results of the genetic test, right after they got the results, six months later, and one year later. Those who got the bad news were, of course, initially devastated, reporting considerably more distress and depression than did those who got the good news. At the six-month and one-year points, however, the two groups were indistinguishable—those who knew that they would die at a relatively young age were no more depressed, and expressed just as much well-being, as did those who knew that they were disease-free. The participants who learned they had the gene received the worst news one can get, and yet within six months they were as happy as anyone else. Even more striking were the results of a third group—those for whom the test was inconclusive or who had chosen not to take the test. At the beginning of the study, before any genetic testing had begun, this group was as happy and well-adjusted as the others. But as time went by, this group did the worst: at the one-year mark, they exhibited significantly more depression, and lower well-being, than those in the other two groups—including the ones who had found out that they had inherited the Huntington gene. In other words, people who were 100 percent sure that they would get the disease and die prematurely were happier and less depressed than people who were 50 percent sure that they were healthy and disease-free.

Tomorrow is promised to no one so live your life fully everyday.

You never know what's going to happen in life.

Our genes are essentially an instruction manual written in a four-letter alphabet: C (cytosine), A (adenine), T (thymine), and G (guanine). Each word is made up of three letters. The word CAG codes for the amino acid glutamine and calls for it to be inserted into a protein when that protein is being synthesized. In Huntington’s disease, a portion of the mutant gene repeats the word CAG again and again, resulting in the insertion of too many glutamines. This expanded string of glutamines causes the protein to clump inside the neuron, killing the cell. We all have multiple CAG repeats in this portion of the huntingtin gene, but a person who inherits a mutated version of this gene and, as a result, has more than 39 CAGs will develop Huntington’s disease (fig. 7.5

We have the technology right now to effectively eradicate Huntington's disease from the planet, along with many other genetic disorders. But the messy realities of human existence--of economics, emotions, politics, and the rest--override the technological possibilities.

The risk of Parkinson’s is increased if you have a mutation in a gene called GBA, which codes for one of the digestive enzymes involved in autophagy. Parkinson’s is accompanied by ‘Lewy bodies’, clumps of a protein called alpha-synuclein that are toxic to brain cells. The problematic, sticky form of alpha-synuclein is normally degraded by autophagy, but even a small impairment caused by a minor GBA mutation is enough to slow its breakdown, increase its levels and thus increase the risk of getting Parkinson’s. Impaired autophagy is also associated with Alzheimer’s and Huntington’s disease, arthritis and heart problems.

in making neurotransmitters, the pathways that neurons travel on. The major breakthrough is the fact that the brain becomes insulin resistant. A substance in coconut oil and palm oil called MCT. When the oil is metabolized, it created ketones which could protect the brain from Alzheimer's, it may reverse the disease. It has been tested as possible treatments for Parkinson's Disease, Huntington's Disease, Multiple Sclerosis and amotrophic lateral sclerosis (also known as ALS, or Lou Gherig's disease). There have been some positive results with coconut oil. With further research, there may be a cure for disease that takes so much away from those stricken with the diseases.

Researchers followed a sample of young adults who had a 50 percent chance of getting Huntington’s disease and who agreed to take the genetic test. The participants completed measures of depression and psychological well-being before they knew the results of the genetic test, right after they got the results, six months later, and one year later. Those who got the bad news were, of course, initially devastated, reporting considerably more distress and depression than did those who got the good news. At the six-month and one-year points, however, the two groups were indistinguishable—those who knew that they would die at a relatively young age were no more depressed, and expressed just as much well-being, as did those who knew that they were disease-free. The participants who learned they had the gene received the worst news one can get, and yet within six months they were as happy as anyone else. Even more striking were the results of a third group—those for whom the test was inconclusive or who had chosen not to take the test. At the beginning of the study, before any genetic testing had begun, this group was as happy and well-adjusted as the others. But as time went by, this group did the worst: at the one-year mark, they exhibited significantly more depression, and lower well-being, than those in the other two groups—including the ones who had found out that they had inherited the Huntington gene. In other words, people who were 100 percent sure that they would get the disease and die prematurely were happier and less depressed than people who were 50 percent sure that they were healthy and disease-free.