Huntington Disease

The symptoms in Huntington disease begin with noticeable behavioral changes including aggression, paranoia, and irritability. Affected individuals may seem restless, with tapping feet or odd twitches. Patients begin to suffer from impaired judgment and an inability to be organized. They become forgetful and their I.Q. declines, coinciding with the deterioration of the brain. Emotionally, they may suffer from psychiatric disorders and even suicidal thoughts or actions. They may drop things and become less efficient in their usual activities. Depression, anxiety, and apathy are also common experiences in the beginning stages of the disease. As the illness progresses, the chorea worsens. The entire body moves in uncoordinated, jerky movements.

Although wide variations in clinical manifestations exist, the illness typically lasts 13–16 years. In later stages of the illness, patients cannot walk or care for themselves. They may barely speak and may fail to recognize friends and family. They eventually require full time nursing care. Eating is quite difficult and death is very frequently caused by choking or by the pneumonia that results after accidentally inhaling bits of food. Although some symptoms can be treated with medication, currently no cure exists to delay the onset of Huntington disease or to slow its course.

Huntington disease is a hereditary disease caused by a dominant gene and, therefore, follows an autosomal dominant pattern of inheritance. This means that only one copy of the gene is necessary to cause the disease. It is transmitted from one generation to the next. The child of a mother or father suffering from Huntington disease has a 50% chance of inheriting the disease gene and, thus, of contracting the disease later in life. About 30,000 Americans suffer from Huntington disease and another 150,000 are at risk for developing it. One of the best-known disease victims was an American folk singer Woody Guthrie (1912–1967), who died of the disease.