HUNTINGTON’S CHOREA

An uncommon hereditary disorder, which can have disastrous effects not only on the sufferer but also on the whole family, is known as Huntington’s chorea.

It is caused by a dominant gene, so the children of a sufferer have a one in two chance of inheriting the disease. Unfortunately, it may not appear until a person is in his forties, so the sufferer has usually married and reproduced before being aware that he has it. It affects men and women equally.

Once the disease declares itself, the sufferer shows involuntary muscular movements, incoordination and mental degeneration.

Although it occurs in most countries of the world, it has its greatest incidence in closed communities where there is a degree of inbreeding.

Tasmania has one of the highest prevalences of Huntington’s disease in the world and this has been traced to the arrival in the last century of an affected Somerset woman who had nine affected children.

Unfortunately, there is no treatment.

Considerable research is under way to improve the outlook for sufferers. Proper genetic counselling for those who have a family history of this disorder can help a couple to decide about having a family.

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