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Huntington's disease is a condition of programmed deterioration of nerve cells in certain areas of the brain. Caused by gene that codes proteins to "fold" incorrectly, this condition causes a steady progression of symptoms that lead to death.

The symptoms of Huntington's disease

The earliest symptoms of this condition are fatigue and depression. There can be mood swings, and difficulty learning new things. As the condition progresses, victims of this disease may become unable to drive, unable to dress themselves, unable to feed themselves. The movements of the arms and legs are described as "chorea," a jerky kind of uncoordinated motion that can resemble an awkward dance.

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Eventually there will also be problems with swallowing and breathing. The rate at which Huntington's progresses varies from person to person, but the disease typically gets worse and worse with only brief periods of partial remission.

A neurodegenerative genetic disorder

Huntington's disease is hereditary. A single copy of the gene for the disease inherited from either parent will cause the illness. Not every child of a person who has Huntington's, however, will get the disease, and not every sibling of a person has the gene will get the disease.

Anyone who has Huntington's, however, has at least one parent who carried the gene for the disease. Some people who have the gene die of other causes before they are diagnosed.

The malformed protein that causes the symptoms of Huntington's disease exists from birth. For the first three or four decades of life, this huntingtin protein simply floats in the watery cytosol inside brain cells. At this stage it is harmless.

As other influences cause aging in the brain, however, the huntingtin protein begins to interfere with the various "organelles" or activity centers within the brain cell. It stops the transport of nutrients from the bloodstream to parts of the cells that need them. It encapsulates the storage vesicles inside the cell that give it reserve energy. It begins to interfere with the energy-making mitochondria of the cell.

Diet and Huntington's disease

The huntingtin protein is made of glutamate. This is the same glutamate that is often associated with the use of aspartame and foods made with MSG, also known as an excitotoxin.

The few holistically oriented physicians who treat this condition reason that since the damage caused by Huntington's is unleashed by glutamate, eliminating glutamate from the diet should be the number one nutritional priority. This means not eating any food containing MSG (monosodium glutamate) or hydrolyzed vegetable protein, and also avoiding red meat, aged cheese, and pureed tomatoes. It is impossible, of course, to test the impact of diet on Huntington's disease since any study would require at least half of the participants to eat the foods that are potentially harmful to them, and no researcher would consider this ethical.

Holistically oriented physicians also advise that the effects of aspartame (Nutrasweet) are cumulative, so the sweetener is best avoided altogether. Fortunately, recent research has also found that certain beneficial practices are also of cumulative benefit.

Use it or lose it, brain function in Huntington's disease. Scientists now know that the rate at which Huntington's disease progresses depends a great deal on brain activity. Although the gene for Huntington's disease eventually causes proteins in the brain to stick together so that brain tissue is destroyed, keeping the brain at the right level of electrical activity slows the development of the disease.

Physicians specializing in this condition have long recognized that mental impairment precedes physical impairment, and that keeping mentally active, by reading, doing crossword puzzles, and so on, also helps slow down loss of motor abilities. Australian researchers Dr. Jess Nithianantharajah and Dr. Anthony Hannan have found in their studies of mice with a biochemical equivalent of Huntington's disease that an information-rich environment stopped the development of dementia and slowed memory loss. The researchers found that staying active preserved the hippocampus, the part of the brain that is involved in making memories. The more mentally stimulated the mice were, the more certain, identifiable chemicals in the hippocampus protected its proteins from degradation.

In human beings, chronic diseases of neurological deterioration affecting the hippocampus usually first result in an inability to articulate a memory. Asked to recall a happy event or the name of a family member, the affected person may reply with a bland "I don't know," without any upset over the loss of the memory—because the emotional and factual components of the memory do not come together. If loved ones who are no longer remembered persist in questioning, however, then the person with the memory deficit may become upset, but not because the memory is lost.

A cure for Huntington's on the horizon?

Relying on doing the crossword puzzles, of course, is not and never will be a complete cure for Huntington's. Researchers have also known for a long time that keeping the neurons in the brain "busy" helps preserve them from the huntingtin protein, but the production of chemicals outside the neurons in the watery fluids of the brain hurts them.

Working with cultures of brain cells incubated with the cell-damaging protein huntingtin, two researchers have learned that the drug Memantine may keep brain cells working without generating damaging chemicals outside them. Canadian researcher Michael R. Hayden, M.D., Ph.D., University Killam professor in the department of Medical Genetics at the University of British Columbia, and director of the Centre for Molecular Medicine and Therapeutics at the Child & Family Research Institute, and California researcher Stuart A. Lipton, M.D., Ph.D., director of the Del E. Webb Center for Neuroscience, Aging and Stem Cell Research, have found that protecting brain cells from the excitotoxic effects of glutamate, while also keeping them active, slows the rate of deterioration.

But Memantine is not the only drug in development. Memantine preserves brain cells from the outside in, and another drug seems to preserve brain cells from the inside out. The experimental drug Dimebon (latrepirdine), in clinical trials sponsored by Medivation and Pfizer, stabilizes the energy-making mitochondria inside the neuron. In concert with Memantine, Dimebon may create the first effective treatment for this ancient progressive neurodegenerative disease.

  • Burnham Institute (2009, November 16). Potential treatment for Huntington's disease. ScienceDaily. Retrieved June 14, 2010
  • Howard Florey Institute (2008, January 29). Mental And Physical Exercise Delays Dementia In Fatal Genetic Disease. ScienceDaily. Retrieved June 14, 2010
  • Photo courtesy of Proxy Design by Flickr : www.flickr.com/photos/proxyarch/5921131112
  • www.sciencedaily.com/releases/2009/11/091115134134.htm
  • www.sciencedaily.com/releases/2008/01/080124092540.htm

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