Huntington's disease (HD) is an inherited disorder characterized by abnormalities in motor function, personality and thinking and memory. While its age of onset averages 40-45, onset may be much earlier. HD is a progressive disorder which leads to an early death on average 17 years from onset.
HD is dominantly inherited. The child of a person with HD has a 50 percent risk of inheriting the gene, thus developing the disorder. The abnormal gene causing HD was discovered in 1993. The gene controls manufacture of a protein which appears to be essential to normal brain function. In HD, the protein manufactured appears to be larger and "stickier" than normal. This large, sticky protein appears to interfere with normal brain cell function, leading to premature death of these brain cells. The discovery of the HD gene was a landmark occurrence that has thrown open the doors to development of meaningful therapeutic strategies.
Protective therapies: The goal of Huntington's disease treatment is to prevent the cell death which leads to its devastating symptoms. There is no proven way to do this at this point, but we are on the threshold of clinical studies of agents purported to be useful in slowing the degenerative process.
Symptomatic therapies: Symptomatic therapies are aimed at improving the symptoms of brain degeneration. Medications are available to improve some of the abnormal movements in Huntington's disease and to assist with control of behavioral and psychiatric changes. However, currently available therapies are woefully inadequate and in need of further study. A very active area of interest is in the surgical therapy of Huntingon's disease. Basic science and early clinical research suggest transplantation of human or other fetal tissue may be useful in Huntingon's disease therapy. Additional research must be done before these treatments are proven effective.
Our neurologists work side-by-side with neuropsychologists, specially trained nurses and other staff to provide patients with the best possible treatment. Rush is part of the Huntington Study Group, a consortium of clinical investigators in HD. This group has an international database which aims to increase awareness of the clinical features of Huntingon's disease, including the rate of disease progression. Our researchers are also involved in other investigations. For more information on clinical trials at Rush, click on the link to your left.