Huntington=s disease, also known as Huntington=s chorea is an inherited disease which becomes evident usually in mid-life, causing uncontrollable jerks or spasms and gradual loss of brain cells, progressing to chorea, athetosis, and mental deterioration. The Huntington=s gene is dominant, and therefore children of people who have the disease have a 50 percent chance of developing it too. This paper will look at Huntington=s disease, recent findings from research, and how electrophoretic techniques can be used to look at the proteins involved in the pathogenesis of disease.
It is often difficult to determine the exact time of onset of Huntington=s disease as it comes on subtly, usually between the ages of 35 and 40 (1). During the early stages of Huntington=s disease, spontaneous abnormal movements can often be blended with normal movements so they are less noticeable, but with time, the abnormal movements become more obvious. With time, they come to involve the entire body, and make the simplest tasks such as dressing and eating almost impossible without aid. Distinct changes can be seen in the brain with computed tomography (a CAT scan). Mental changes also occur - mild at first, but increasing in severity over time. Patients become irritable and excitable, and gradually lose interest in normal activities.
As the disease progresses, Huntington=s patients may behave irresponsibly, wander aimlessly, and even lose control over their impulses and become promiscuous. Loss of memory and rational thinking can occur, and depression and suicidal tendencies may follow. In the later stages of the disease, all functions become impaired, and full-time assistance or nursing home care becomes necessary. Death usually occurs 13 to 15 years after symptoms first become evident, often due to pneumonia or injuries from a fall (1).
The treatment for Huntington=s disease usually consists of drugs to control the symptoms and control irr...