Huntington Disease Center
The Movement Disorders Center at Baylor College of Medicine, Department of Neurology, has been selected by the Huntington's Disease Society of America as the HDSA Center of Excellence in 2001. Directed by Dr. Joseph Jankovic, professor of neurology, the Huntington Disease Center is committed to providing the most expert and compassionate care for patients with HD. In addition to social services, physical, occupational and speech therapy, the patient's condition is managed by a team of skilled clinicians with medications and other therapies targeted to the specific neurologic and psychiatric symptoms. Patients are also invited to participate in a broad range of clinical trials. In addition to experimental therapeutics, the HDC focuses on research into mechanisms of neudodegeneration. Finally the HDC provides a setting for training of young physician-scientists and for educational programs directed to scientists, physicians, alied health professionals and patients and their families. The HDC, located on the 18th floor of Smith Tower, is a joint Baylor-Methodist project.
What is Huntington Disease?
Huntington disease (HD) is a hereditary neurodegenerative disorder caused by a mutation in the gene called huntingtin on chromosome 4. HD is inherited in an autosomal dominant fashion, so that each child of an affected parent has a 50% chance of inheriting the mutated gene and developing the disease. Most people with HD develop the symptoms in their 40's and 50's. About 10% of patients have the symptom onset before age 20, which is known as juvenile HD, and about 10% have onset after age 60.
HD typically manifests as a triad of motor, cognitive, and psychiatric symptoms which begin gradually and progress over many years. The movement disorder is characterized by involuntary movements known as chorea, and by the impairment of voluntary movements. The latter often presents the greatest disability resulting in reduced manual dexterity, slurred speech, swallowing difficulties, balance problems and falls. Both chorea and impairment of voluntary movements progress in the middle stages of the disease, but later, chorea may gradually subside as rigidity and stiffness progress. Cognitive changes are initially characterized by forgetfulness and impaired judgment. These mental changes may gradually progress to confusion and true dementia. The most frequent psychiatric symptoms include irritability, depression, paranoia, and erratic behavior. Patients may also exhibit manic and obsessive-compulsive states. Other symptoms may include anxiety, agitation, impulsivity, apathy, and social withdrawal.
How is Huntington Disease Treated?
Currently, there is no known cure for HD, but many effective and safe treatments are available to alleviate the symptoms. Neuroprotective therapies designed to slow down the disease process are currently being studied. Caring for patients with HD is both challenging and rewarding. The lack of definitive treatments can be frustrating, but careful attention to the changing symptoms and open lines of communication between professionals, family members, and affected individuals all contribute to the successful management of the disease process.