Huntington's disease

n. A progressive neurodegenerative disease typified by the slow and steady development of involuntary muscle movements in the hands, feet, face, and trunk. Involuntary motor responses may include rapid, jerky movements, called chorea. Huntington's disease is also characterized by dementia, or a progressive deterioration of cognitive and memory functioning. Dementia in Huntington's disease is typically associated with disorientation, confusion, personality and behavior changes, memory impairment, and agitation.

Huntington's disease is an inherited disease that is caused by a mutated gene. It is often diagnosed by blood test. Symptoms generally appear in midlife. Among younger people, symptoms tend to be more severe and progress more quickly. It is rare for this disease to be seen in children.

The disease has a gradual progression, with increasingly prominent symptoms in later stages associated with more widespread neuron damage. Most cell loss occurs in the caudate nucleus and putamen, with some damage in the globus pallidus. The earliest signs of Huntington's disease may include changes in personality and cognitive abilities. Early physical symptoms such as mild balance problems, clumsiness, and involuntary facial movements may also be seen. Later in the disease, prominent involuntary movements throughout the body, acute balance and coordination problems, broken or slurred speech, and a wide gait may be present. Finally, ability to perform basic functions such as swallowing, eating, speaking, and walking tends to decline with time. Because of the slow decline of functioning, individuals with Huntington's disease may become depressed and can be at risk for suicide.

Currently, although medications may assist with some involuntary movements and behaviors, there is no cure for Huntington's disease. After the onset of Huntington's disease, symptoms persist until death. Death typically occurs as the result of complications such as infections or injuries between 10 and 30 years after symptoms first appear.

- TJM