Huntingdons Chorea

Huntingdon’s Chorea biology gcse/a-level

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Huntingdon's Chorea
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 nonrepetitive.
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 1020 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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