April 2009
M	T	W	T	F	S	S
« Mar		May »
	1	2	3	4	5
6	7	8	9	10	11	12
13	14	15	16	17	18	19
20	21	22	23	24	25	26
27	28	29	30

Subscribe to RSS Feed

UNTREATABLE CAUSES OF DEMENTIA: HUNTINGTON’S CHOREA

on April 2nd, 2009 by admin

Huntington’s chorea is a very complicated disorder of the brain. In most families it is inherited in what is known as a dominant autosomal pattern. This means that approximately half the children of an affected parent will suffer with the condition, which usually strikes in middle age or early old age. It therefore poses quite an ethical problem, as a potential sufferer cannot know that he or she will definitely develop the condition until the time for having children is past. However, if we become able to determine accurately whether or not a person from an affected family is going to develop the disease, anybody who is so diagnosed will immediately know what the future holds for them and can plan accordingly.

The disease tends to start in middle age with abnormal movements of the limbs which usually worsen over the years; at the same time dementia sets in. People suffering with Huntington’s chorea may also be afflicted by other psychiatric conditions, the most common of which is probably depression.

Most of the damage from Huntington’s chorea falls on some of the deep-lying areas of grey matter, especially a structure called the caudate nucleus which lies in the outside wall of the main ventricle. Cells are also lost from the cerebral cortex and there are changes in the brain’s biochemistry.

A brain scan will show that the ventricles have increased in size, especially the part that lies adjacent to the caudate nucleus. It is the loss of the caudate nucleus that is partly responsible for the increase in the ventricular size.

Some of the abnormal movements technically described as chorea, hence the name, can be reduced with special drugs, but none of these are of any help in treating the dementia itself.

The most important aspect of this condition at the moment is the need for genetic counselling for members of affected families.

*18\138\2*