- Introduction to Huntington's disease
- What causes Huntington's disease?
- How is Huntington's disease inherited?
- What are the symptoms and major effects of Huntington's disease?
- At what age does Huntington's disease appear?
- How is Huntington's disease diagnosed?
- What is presymptomatic testing?
- How is the presymptomatic test conducted?
- How does a person decide whether to be tested?
- Is there a treatment for Huntington's disease?
- What kind of care does an individual with Huntington's disease need?
- What community resources are available for Huntington's disease?
- What research is being done on Huntington's disease?
- How can I help?
- What is the role of voluntary organizations?
- Where can I get more information about Huntington's disease?
- Patient Comments: Huntington Disease - Experience
- Patient Comments: Huntington's Disease - Share Your Experience
- Patient Comments: Huntington's Disease - Symptoms
- Patient Comments: Huntington's Disease - Family History
Introduction to Huntington's disease
In 1872, the American physician George Huntington wrote about an illness that he called "an heirloom from generations away back in the dim past." He was not the first to describe the disorder, which has been traced back to the Middle Ages at least. One of its earliest names was chorea,* which, as in "choreography," is the Greek word for dance. The term chorea describes how people affected with the disorder writhe, twist, and turn in a constant, uncontrollable dance--like motion. Later, other descriptive names evolved. "Hereditary chorea" emphasizes how the disease is passed from parent to child. "Chronic progressive chorea" stresses how symptoms of the disease worsen over time. Today, physicians commonly use the simple term Huntington's disease (HD) to describe this highly complex disorder that causes untold suffering for thousands of families.
More than 15,000 Americans have HD. At least 150,000 others have a 50 percent risk of developing the disease and thousands more of their relatives live with the possibility that they, too, might develop HD.
Until recently, scientists understood very little about HD and could only watch as the disease continued to pass from generation to generation. Families saw the disease destroy their loved ones' ability to feel, think, and move. In the last several years, scientists working with support from the National Institute of Neurological Disorders and Stroke (NINDS) have made several breakthroughs in the area of HD research. With these advances, our understanding of the disease continues to improve.
This brochure presents information about HD, and about current research progress, to health professionals, scientists, caregivers, and, most important, to those already too familiar with the disorder: the many families who are affected by HD.
What causes Huntington's disease?
HD results from genetically programmed degeneration of nerve cells, called neurons,* in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. Specifically affected are cells of the basal ganglia, structures deep within the brain that have many important functions, including coordinating movement. Within the basal ganglia, HD especially targets neurons of the striatum, particularly those in the caudate nuclei and the pallidum. Also affected is the brain's outer surface, or cortex, which controls thought, perception, and memory.
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