What Is Huntington's Chorea?

Huntington's Chorea is a cantral nervous system movement disorder which is extremely disabling for its sufferers.

Dr. George Huntington, a Long Island, N.Y. physician first described this disease in 1872. The most famous sufferer of this disorder was probably the celebrated folk singer Woody Guthrie, whose wife, Marjorie noticed her husband walking lopsidedly one day in the 1950s. She then began to notice that his speech was becoming slurred but it was only when he began to fly into major rages that she realized something was severely wrong with her husband. Eventually, he lost all ability to talk, to read or walk. The only way he could communicate with his wife and children was by waving his arm at cards printed with the words 'Yes' and No'.

The disease was Huntington's Chorea, which is an inherited, degenerative disorder of the Central Nervous System, caused by a dominant gene. This means that everyone who inherits the gene from one of his/her parents WILL develop the disease, and the likelihood of doing so is therefore 50%. The specter of this cruel disease continues to hang over the entire Guthrie family.

Huntington's Chorea is a particularly devastating disease because symptoms normally do not occur until after the age of 35, but can onset later (the earlier the onset, the more severe the disease tends to be). It is principally a movement disorder, with the first observable symptoms manifesting themselves as 'clumsiness', but as the disease progresses the movements become uncontrollable. These movements appear to be very bizarre and include odd bodily postures. Other symptoms are also apparent including forgetfulness and irritability or withdrawing (in the early stages) progressing to dementia with severe memory loss and lack of reasoning.

Patients suffering from Huntington's Chorea show degenerative changes in the basal ganglia structures, which ultimately result in a severely shrunken brain and enlarged ventricles. The caudate and putamen brain structures are particularly affected as they shrink up to half their normal size.

The symptoms of the disease are caused by a significant reduction (volume and activity) of two principal neurotransmitters (naturally occurring chemicals in the brain) - namely Acetylcholine and GABA, in turn affecting the activity of the neurotransmitter Dopamine, which becomes hyperactive. Huntington's Chorea is therefore the 'flip side of the coin' to another movement disorder - Parkinson's Disease where there is dopamine under activity.

Huntington's Chorea is principally characterized by hyperkinesias - abnormal, purposeless, involuntary motor movements that can occur spontaneously or only when the patient is trying to do something. These movements may be repetitive or non-repetitive.

Drug therapies can ease the symptoms of the disease (including the use of dopamine antagonists or neuroleptics) but there may be severe side effects with these drugs. Unfortunately, there is no cure for Huntington's Chorea. However, new techniques involving neural grafting (implanting healthy fetal brain cells into the damaged areas) may offer hope for sufferers in the near future.

The illness may continue relentlessly for 10-20 years once it has been diagnosed, and cause of death is commonly heart disease after a bout of illness, or due to falls, choking or suicide before dementia sets in. New advances in medicine may provide hope for patients suffering from this devastating disease in the future. Research and clinical trials are currently underway.

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