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Tell me a little bit about yourself.
"My subspecialty training, clinical work and research are in movement disorders. I serve as the Director of the Movement Disorders Program at the University of California, Irvine. I am also the Director of the Phillip amp; Carol Traub Center for Movement Disorders of the Eisenhower Medical Center in Rancho Mirage, California."

What is Huntington's disease?
"Huntington's disease is a degenerative disease of the brain that is typically inherited. The gene for Huntington's disease is passed from parent to child in a manner called autosomal dominant, meaning that each child of an affected parent has a 50-50 chance of acquiring the abnormal gene. How this abnormal gene causes the disease is not yet understood and is an area of active research. Although the average age at which symptoms appear is between 35 and 40 there have been unusual cases where the diagnosis was established in very young children and elderly adults. The disease is progressive and ultimately leads to death after a course usually of several years. Presently there is no known cure for the underlying disease, although some of the symptoms can be improved by treatments including medications and rehabilitative therapies, such as physical and speech therapy."

What are the signs and symptoms of Huntington's disease?
"People with Huntington's disease experience symptoms of both motor and cognitive impairment and decline. Patients find coordination declines, movement becomes slower and more awkward, and abnormal, involuntary movements called chorea often emerge. Chorea consists of jerking or twitching type movements that can involve almost any part of the body and may initially have the appearance of restlessness and later be larger, squirming or flinging type movements. Balance is often unsteady and falls occur. Speech and swallowing are also affected leading to reduced clarity of speech and choking or coughing when eating or drinking. Less commonly, and particularly in very young patients, rigidity of the limbs causing stiffness and slowness of movement is the predominant motor abnormality rather than chorea."

"The cognitive symptoms may include depression, memory difficulties and poor judgment and insight. Personality is sometimes affected with abnormal behavior emerging, including aggression that may be verbal or physical."

"The first symptoms may be either motor or cognitive, although all patients generally experience decline in both areas over time."

What type of impact does Huntington's disease have on a person's life?
"The disease has a devastating impact not only on the person affected but also on their family members. People with Huntington's disease develop symptoms often when they still have young children and are employed and dependent upon their income for maintaining their home and health insurance. The mood and behavioral changes in particular can be very difficult and lead to separation of patients from their families. The motor changes eventually lead to increasing need for assistance in basic daily activities such as dressing, hygiene and eating."

What type of help is available for someone who has Huntington's disease?
"Genetic counseling is available for patients and their families to better understand how the disease is transmitted and the risks to other members who do not have symptoms. This can include a blood test for the Huntington's disease gene even in people who have no symptoms. This is a very serious decision that requires careful and prolonged thought and counseling."

"There are medications available that can successfully treat the depression and the behavior problems that occur with Huntington's disease."

"The chorea can be reduced my medications, including tetrabenazine (also known as Xenazine), the first medication approved by the FDA specifically for this purpose."

"Physical therapy can be helpful to reduce the risk of falls and to optimize mobility. A speech pathologist can provide instruction to improve speech clarity and can also perform a swallowing evaluation and develop a strategy to avoid choking or aspiration of food and liquid into the lungs."

What advice would you like to leave for friends or family members who know of someone who has Huntington's disease?
" Fortunately Huntington's disease is uncommon, affecting less people in the U.S., compared to a disease such as Multiple Sclerosis or Parkinson's disease. However, the rarity of Huntington's disease also means that most doctors and health care providers have little or no experience with it."

"I recommend that people seek help and treatment at an established center or clinic for Huntington's disease. These are often within the Neurology Department of medical schools, although there are some excellent programs that are not in academic centers."

"Referrals for experienced doctors as well as further information on Huntington's disease can be obtained from the Huntington's Disease Society of America (www.hdsa.org)."

"I also encourage patients and their families to inquire about current clinical trials of medications that are being tested for Huntington's disease. There are several underway currently, and it is only through patient participation in these studies that we will be able to develop new treatments while also learning more about Huntington's disease itself."

Thank you Dr. Neal Hermanowicz for the interview. If you would like more information about Dr. Neal Hermanowicz check out his website at .