Comprehensive Huntington's Disease Clinic Opens at Northwestern Medicine
Northwestern Memorial Hospital May 14, 2015
“Huntington’s disease is a hereditary disorder characterized by abnormal movements and cognitive difficulties caused by a mutation in the gene huntingtin,” said Dimitri Krainc, MD, PhD, Aaron Montgomery Ward Professor and chair of The Ken & Ruth Davee Department of Neurology at the Northwestern University Feinberg School of Medicine. “The very complex nature of this disease requires a collaboration of top experts in the fields of neurology, psychiatry, genetics, social work and more. Our Huntington’s clinic will provide multidisciplinary care with researchers at the forefront of research and physicians providing the most comprehensive treatment available. It’s a group dedicated to improving the quality of life for our patients, finding better treatments and one day, hopefully, a cure.
In the United States, about 30,000 people have Huntington’s disease and many more are at risk of developing it. For these patients, brain cells, or neurons, in certain areas of the brain start to break down. As the neurons degenerate, the disease can lead to emotional disturbances, loss of intellectual abilities and uncontrolled movement. A parent with Huntington disease has a 50 percent chance of passing the disease on to their child. A genetic test can determine if someone has the gene and will develop the disease. There is no cure for Huntington’s disease and while medicine can help manage some of the symptoms, it cannot slow down or stop the disease.
Throughout the past decade, Krainc has been recognized as one of the leading Huntington’s disease researchers, having discovered how abnormal huntingtin leads to the dysfunction of brain cells early in the disease or before the symptoms appear. This research has identified the underpinnings of the disease that serves as the foundation for development of new treatments.
“Our physicians and researchers all have the same goal - to improve the quality of care for patients at every stage of this disease,” said Danny Bega, MD, a neurologist and clinical director of the Huntington’s Disease Clinic at Northwestern Medicine Memorial Hospital and a faculty member at Northwestern University Feinberg School of Medicine. “While there is no cure for Huntington’s, we will work with patients to help them cope with emotional challenges while also finding the best medications to treat symptoms. In addition, we provide support for their caregivers, families and loved ones.”
The other key researchers who study Huntington’s disease at Northwestern University are:
- D. James Surmeier, PhD, Nathan Smith Davis Professor of Physiology and chair of Feinberg’s Department of Physiology. Surmeier researches how the brain’s basal ganglia network stops working normally in Huntington’s patients, which affect their core motor symptoms. His lab utilizes an animal model of Huntington’s disease with the same genetic mutation associated with the human disease. Insights gained from this work are currently being applied to clinical trials testing new therapies.
- Richard B. Silverman, PhD, John Evans Professor of Chemistry and professor in Molecular Biosciences at Northwestern University’s Weinberg College of Arts and Sciences. Silverman studies the mechanisms and design of drug agents to treat several neurodegenerative and neurological diseases. In a previous Huntington’s disease project, his group synthesized compounds active in cells that had drug-like properties and investigated their potential to reverse the effects of neurodegeneration.
- Richard I. Morimoto, PhD, Bill and Gayle Cook Professor in Molecular Biosciences at Weinberg. Morimoto investigates cell stress response pathways and protein homeostasis networks that determine the folding, transport and clearance of the proteins expressed in the body. His lab employs biochemical, genetic, molecular, proteomic and genomic methods to identify changes that occur during Huntington’s disease and other age-associated neurodegenerative diseases.
- Ravi Allada, MD, chair of Weinberg’s department of Neurobiology and professor in Feinberg’s department of Pathology. Allada’s research focuses on the circadian regulation of sleep behavior. Allada’s laboratory tests the hypothesis that the circadian clock function is linked to the neurodegenerative decline seen in Huntington’s disease and other conditions, and to discover the genetic bases of these links in hopes of providing novel pharmaceutical targets and protective strategies.