[Home]Huntingtons disease

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Huntington's disease or Huntington's chorea is an inherited disorder characterized by abnormal body movements called chorea and loss of memory. The incidence is about 1/200,000. The causative gene (which is one of the first identified to cause an inherited disease) is located on chromosome 4. The product of this gene is referred to as huntingtin and the continous accumulation of this protein in neuronal cells gives rise to cell death, especially in the frontal lobes and the [basal ganglia]? (mainly in the caudate nucleus) by some unknown mechanism. The symptoms usually start in the 4th decade of life and include loss of cognitive functions, personality changes, quick jerking movements of face and body and unsteady gate. The diagnosis is established by neurological examination findings and the demonstration of cell loss, especially in the caudate nucleus, supported by a cranial CT? or MRI scan findings. Although dopamine? receptor blockers may have restricted benefits, there is no definite treatment for disease.

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Last edited July 6, 2001 5:06 pm by Gareth Owen (diff)