School of Medicine

Wayne State University School of Medicine

Huntington's Disease

Huntington's disease (HD) is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and psychiatric problems. It typically becomes noticeable in mid-adult life. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea.

It is much more common in people of Western European descent than in those of Asian or African ancestry. The disease can affect both men and women. The disease is caused by an autosomal dominant mutation in either of an individual's two copies of a gene called Huntington, which means any child of an affected person typically has a 50% chance of inheriting the disease. Physical symptoms of Huntington's disease can begin at any age from infancy to old age, but usually begin between 35 and 44 years of age.

Everyone has the HD gene but it is those individuals that inherit the expansion of the gene who will develop HD and perhaps pass it onto each of their children. Presently, there is no effective treatment or cure. Although medications can relieve some symptoms, research has yet to find a means of slowing the deadly progression of HD. Current estimates are that 1 in every 10,000 Americans has HD and more than 250,000 others are at-risk of having inherited it from a parent. Once thought a rare disease, HD is now considered one of the more common hereditary disorders.

Our Laboratory Investigates:

Quantification of tissue injury by using a variety of imaging techniques including MRS, DTI, and MTR.

Coronal section of a patient with HD showing atrophy of the heads of the caudate nuclei and enlargement of the frontal horns of the lateral ventricles
Basal ganglia cells (ROI) (in red) and reference cells (in green). Hypo-intense pixels are displayed in yellow/orange.