News Release

Expert-based clinical guidelines focus on behavioral symptoms in Huntington's disease

Clinicians treating patients with HD are often not trained to recognize or treat neuropsychiatric symptoms of the disease, according to new guidelines published in the Journal of Huntington's Disease

Peer-Reviewed Publication

IOS Press

Amsterdam, NL, January 30, 2019 - Although Huntington's disease (HD) is traditionally thought of as a neurological disorder, behavioral symptoms are a common feature and frequently cause distress and difficulty to patients, family members, and other caregivers. Since an estimated 70% of US patients with HD do not seek specialist care, they are often treated by general practitioners, general neurologists, and psychiatrists, many of whom may not be trained to recognize or treat HD-related behavioral symptoms. A group of experts has now developed consensus guidelines published in the Journal of Huntington's Disease written in simple terms to guide both specialists and non-specialists in the effective management of neuropsychiatric disorders in HD patients.

"These guidelines convey the important message that we have treatments available now for many neuropsychiatric symptoms of HD. This should encourage patients to seek care. They also help non-specialist clinicians understand that HD is a not a hopeless condition," explained Karen E. Anderson, MD, of the Departments of Psychiatry and Neurology of Georgetown University and Director of the Huntington's Disease Care, Education, and Research Center (HDCERC), Washington, DC.

For any disease, the most highly regarded clinical guidelines are based on robust clinical evidence. However, conditions with low prevalence, such as HD, often lack the necessary randomized clinical trial evidence required to assess treatment efficacy and safety. In such cases, expert-led guidelines help overcome these limitations.

An international, multidisciplinary expert panel was tasked to develop statements about target symptoms about five common behavioral symptoms exhibited by HD patients: agitation, anxiety, apathy, psychosis, and sleep disorders. These were then submitted to HD experts around the world for consensus agreement on the merits of each statement. Significantly, in the absence of randomized controlled trials, experts do largely agree on the treatment of the behavioral symptoms.

Clinical Practice Guidelines (CPG) for neuropsychiatric symptoms that include general, behavioral, and pharmacologic recommendations are presented and include topics such as the preferential use of medications with the fewest unwanted adverse effects, regular reevaluation of drug therapy as a patient's condition changes, and consultation with a psychiatrist with particular expertise in HD-related behavioral symptoms, especially if symptoms become resistant to treatment.

"Clinical experience indicates that most of the neuropsychiatric symptoms discussed are treatable using non-pharmacologic and pharmacologic strategies developed for use in other patient groups (e.g. Alzheimer's disease). Treatment of the symptoms as experienced in HD generally follows the same recommendations as in other patient groups. However, the management of neuropsychiatric symptoms in HD is frequently more complex because symptoms often coexist, and treatment decisions should be adapted to cover all symptoms while limiting the simultaneous use of multiple drugs," noted Dr. Anderson.

The guidelines emphasize that educating patients, their family members, and caregivers about behavioral symptoms in HD is an important part of treatment, and that behavioral disorders in HD can be treated. "We encourage patients and families to use these guidelines to partner with their clinicians when seeking care since these symptoms often have a huge impact on patients' wellbeing and their relationships with individuals close to them," advised Dr. Anderson.



"Clinical Management of Neuropsychiatric Symptoms of Huntington Disease: Expert-Based Consensus Guidelines on Agitation, Anxiety, Apathy, Psychosis and Sleep Disorders," by Karen E. Anderson, Erik van Duijn, David Craufurd, Carolyn Drazinic, Mary Edmondson, Nathan Goodman, Daniel van Kammen, Clement Loy, Josef Priller, and LaVonne Veatch Goodman (DOI: 10.3233/JHD-180293), published in the Journal of Huntington's Disease, Volume 7, Issue 4, published by IOS Press. This article is openly available at

Contact Diana Murray, IOS Press, +1 718-640-5678 or for additional information. Journalists who wish to interview the authors should contact Karen E. Anderson at

This study was supported by the Griffin Foundation and Auspex Pharmaceuticals.


The Journal of Huntington's Disease is an international multidisciplinary journal to facilitate progress in understanding the genetics, molecular correlates, pathogenesis, pharmacology, diagnosis and treatment of Huntington's disease and related disorders. The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational research, and clinical medicine that will expedite our fundamental understanding and improve treatment of Huntington's disease and related disorders.


IOS Press is headquartered in Amsterdam with satellite offices in the USA, Germany, India and China and serves the information needs of scientific and medical communities worldwide. IOS Press now publishes over 100 international journals and about 75 book titles each year on subjects ranging from computer sciences and mathematics to medicine and the natural sciences.

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