Huntington's disease

Huntington’s disease is a progressive hereditary condition that causes both movement dysfunction and mental deterioration. The gene for Huntington’s disease is dominant, meaning that the child of a person with the disease has a fifty percent chance of developing Huntington’s disease themselves. Huntington’s disease begins very subtly, often with an occasional muscle spasm or jerk. For this reason, the exact age of onset can be difficult to discern. The more obvious symptoms of Huntington’s disease develop in a patient’s 30s and 40s.

During the initial stages of Huntington’s disease, a person may experience abnormal and unintended muscle spasms which are barely noticeable. As the condition progresses a person may begin to experience these unintentional movements more frequently. Movement and coordination may become more difficult. People with Huntington’s disease frequently grimace, blink excessively, and flick their limbs. In time, a person with Huntington’s disease may have extreme difficulty walking, eating, speaking, dressing, and sitting.

Huntington’s disease also has significant cognitive effects, for it causes a progressive loss of brain cells. At first, mental changes are often subtle. Emotional control may become more difficult to manage. A patient with Huntington’s disease may lose the ability to control their temper, impulses, and other emotions. Memory and rational thinking may also be lost in time. Depression and severe dementia are common in advanced stages of this genetic disease.

Early diagnosis of Huntington’s disease is difficult because the symptoms are often subtle for years after onset. A patient’s symptoms, medical history, family background, and current physical condition will be evaluated in order to make a diagnosis. Patients should inform their doctor about any family members who may have had Huntington’s disease, a similar condition, or any psychological condition. A CT scan and/or an MRI can indicate deterioration of the basal ganglia, which is present in most patients with Huntington’s disease.

There is, unfortunately, no known cure for Huntington’s disease. Certain drugs may be prescribed to manage a patient’s condition by relieving symptoms and regulating behavior. Drugs such as chlorpromazine (a sedative) and antipsychotic drugs may be prescribed to people with Huntington’s disease. It is important to discuss use of these drugs with a knowledgeable physician, as each is associated with certain risks and side effects.

Death from Huntington’s disease usually occurs between 13 to 15 years after symptoms begin. The cause of death is commonly pneumonia or a fall. Towards the final years of life a patient will often require full time care in a nursing home or other long term care facility. A patient who develops Huntington’s disease may wish to consider advance directives which tell loved ones how to care for you as the condition progresses.