Medications and Non-Drug Approaches Used to Treat Huntington's Disease

Man with Huntington's disease watching his daughter on swing along with wife
Life with HD. Huntstock / Getty Images

HD has a wide range of symptoms that may develop, some of which can be successfully managed with medications and non-drug approaches. Before delving into treatments, understanding symptoms as part of the disease can also help cope with them and take a more active role in your own or a family member's care.

Types of Symptoms

As a neurological condition, Huntington's causes symptoms that typically fall into one of three categories: physical/movement changes, cognitive changes, and emotional/behavioral changes.

HD is also one of several conditions that cause dementia to develop.

Symptoms most often develop between 30 and 50 years old. However, some people who are younger than age 20 develop a type of Huntington's disease called juvenile Huntington's disease. 

Physical Changes

One of the hallmark symptoms of HD is chorea. Chorea refers to the loss of ability to control physical movements. It produces movements that are involuntary, jerky, and sudden. These movements often occur in the upper body and include the upper arms, trunk, head, neck, and face. They can also occur in the legs. Chorea is present in about 90 percent of people with HD and is one of the symptoms often targeted with medication.

Other physical signs of HD include a decline in walking and talking, a lack of coordination, an impairment in swallowing food and liquids and, consequently, significant weight loss. Due to these symptoms, the risk for falling is high in people with HD, but working with specialists can help reduce risk.

Cognitive Changes

HD is a result of changes in the brain, so it's understandable that cognitive abilities are affected. While memory can be affected, other areas of cognition are impacted as well. These include impaired executive functioning (such as the ability to plan and make decisions), poor concentration, a lack of focus, poor judgment and a lack of insight into your own behaviors.

A lack of inhibitions may also develop. For example, if you have HD, you might do something that you normally wouldn't feel is appropriate because your impulse control is decreased.

Emotional and Behavioral Changes

HD symptoms include several mood and behavior changes. You may feel extreme irritability and anger, along with unpredictable mood swings. Verbal and physical aggression may also develop. In fact, some research concludes that 22 percent to 66 percent of people with HD will display aggression, often in the early stages of the disease.

As with other kinds of dementia, apathy is common in HD. Depression (which is similar to apathy but usually involves feelings of sadness and hopelessness) can be especially significant and is a strong predictor of thinking about or considering suicide. Research indicates that there is a high risk for suicidal thoughts in those living with HD, with some studies indicating the presence of suicidal ideation in about 19 percent of the research participants.

Feelings of anxiety often accompany depression. Research studies show that anywhere from 34 to 61 percent of those with HD experience feelings of anxiety.

Perseveration, where you get "stuck" on a word, thought or action, is not uncommon.

This can combine with obsessions and compulsions and make it difficult to move on to a new task. It also may cause socially inappropriate behaviors that make it difficult for others to know how to spend time with the person with HD.

Treatment Overview

While there is no cure available for HD at this time, there are a few medications and complimentary approaches that may help relieve some symptoms for a time. Keep in mind that you should not use complimentary or supplementary substances without checking with your physician, as some can have significant negative side effects or drug interactions with your other medications.

Since there is no cure, the goal of treatment in HD is to improve quality of life and maintain functioning for as long as possible.

Medications

Xenazine (tetrabenazine)

Xenazine was approved in 2008 by the U.S. Food and Drug Administration (FDA) to treat chorea in HD. It has been shown to help reduce involuntary movements and is generally regarded as one of the most common and effective ways to treat HD.

Xenazine does, however, carry a warning about use in people with depression since it appears to increase depression and suicidal thoughts. Side effects may also include both drowsiness and insomnia.

Austedo (deutetrabenazine)

Austedo was approved by the FDA in 2017. It is also prescribed to treat the involuntary movements (chorea) in Huntington's disease.

Austedo is chemically similar to Xenazine but its effectiveness lasts longer. Consequently, Austedo is usually prescribed to be taken once or twice a day, while Xenazine is usually prescribed to be taken three times each day.

Austedo has demonstrated effectiveness in reducing chorea symptoms, but like Xenazine, this medication comes with a strongly-worded warning indicating it should not be used for people with HD who are experiencing feelings of depression or suicidal thoughts, as it could increase those feelings.

Antipsychotic Medications

Antipsychotic medications, also referred to as neuroleptics, are sometimes prescribed to treat chorea. This is an off-label use of the medication, meaning that the FDA hasn't approved these medications specifically for this purpose; however, some of them have demonstrated some benefit in this area.

Research results vary, but atypical antipsychotics that are often used to treat HD include Zyprexa (olanzapine), Risperdal (risperidone), and Seroquel (quetiapine). Older antipsychotics such as Haldol (haloperidol) and Clozaril (clozapine) are also prescribed but have potential side effects of tremors and tardive dyskinesia, both of which cause other involuntary movements and thus may be counterproductive.

Additionally, some antipsychotic medications such as Abilify (aripiprazole) have shown some effectiveness in reducing symptoms of depression in HD, as well as improving cognition.

Antipsychotics are also used at times with the goal of reducing challenging behaviors (such as aggression) that can develop in HD; however, there are several potential side effects with the use of antipsychotics, so caution is warranted.

Symmetrel

Research on the effectiveness of Symmetrel (amantadine) have demonstrated conflicting results. Symmetrel is an antiviral drug that is used to treat the involuntary tremors that sometimes occur in Parkinson's disease, so its target in HD is the symptom of chorea. It appears beneficial for some people with HD.

SSRIs

Selective serotonin reuptake inhibitor (SSRI) antidepressant medications are prescribed at times to treat depression as well as the obsessions and compulsions sometimes experienced by those living with HD. As with other medications, effectiveness varies.

Mood Stabilizers

Mood stabilizers such as Depakote (divalproex) may be utilized to address the great variation in emotions that can accompany HD, as well as the aggression, impulsivity, and obsessive-compulsive symptoms.

Other Medications

In response to the various symptoms that a patient reports to their doctor, other medications may also be ordered to address those specific issues. For example, if insomnia and anxiety are primary concerns in HD, the physician will often prescribe a medication to target them. Thus, it is important to be open with your healthcare team and let them know about everything that is bothering you. They may have an option that'll improve your situation.

Non-Drug Approaches

Since medications are currently limited to attempting to manage specific symptoms in HD, other non-drug complementary approaches are recommended.

Speech and Language Therapy

A speech and language therapist can work with you to assist in communicating your needs and preferences. Certain exercises can help you strengthen your tongue and mouth muscles to maintain your functioning as long as possible.

Speech therapists can also evaluate your swallowing ability and determine what equipment or interventions will be the most helpful for you. This can be important since, as the disease progresses, it may become more difficult to swallow food or water without choking.

Physical and Occupational Therapy

Physical therapy and occupational therapy can help you in several ways. In the earlier stages of HD, physical therapy can help improve and maintain overall strength and functioning. As HD progresses, equipment can be ordered and tailored to your specific needs, and a home exercise program can be designed to maximize physical health.

An occupational therapist can work with you on determining the best way to perform activities of daily living, such as showering and getting dressed. Occupational therapists can also identify mental exercises, with the goal of maintaining your cognitive functioning.

Therapists can also work with your caregivers as the disease progresses to help them know how best to care for you.

Physical Exercise

Physical exercise has been highly correlated with stable—or even improved—cognitive abilities in many diseases that cause dementia, and this holds true in HD as well. Research has demonstrated that higher levels of physical exercise are predictive of better scores on cognitive tests and improved daily functioning.

Psychotherapy/Supportive Counseling

Talking with a clinical social worker or psychologist can be very important as you adjust to the changes that HD brings and figure out your own coping strategies. Talk therapy can also be very beneficial for spouses or partners, as well as children and families.

HD brings significant changes to your functioning level, and it can raise concerns in family members about their own risk of developing HD since it is genetically passed on to children. A therapist can help you and your family work through the changes that are occurring, the genetic risk of HD, and connect you with resources in your community and for your home.

Creative Therapies

Other approaches are likely to also benefit those living with HD. For example, music, art, and drama therapy have all been utilized. While they won't change the physical symptoms of HD, they may be able to impact the overall well-being and improve quality of life.

Dietitian Services

Eating a healthy diet is important for all of us, and even more so when you're coping with HD. Ensuring that your body is getting the right nutrients it needs can help maintain your strength. This can be difficult, especially as HD progresses, so you may benefit from assistance from a registered dietitian.

A Word From Verywell

Most importantly, people with HD and their families have a risk for isolation. Know that there are resources and support available to encourage you and help you determine your next steps. If you're not sure what to do or need help, the Huntington's Disease Society of America has local chapters as well as online support groups that can encourage you, answer your questions or walk with you or just be a listening ear as you live with HD.

Sources:

Arora, G. (2015). Managing Aggression in Huntington's Disease. http://hdsa.org/wp-content/uploads/2015/07/Managing-Aggression-in-HD_Garima-Arora_ver005.pdf

Coppen, E. and Roos, R. (2016). Current Pharmacological Approaches to Reduce Chorea in Huntington’s Disease. Drugs, 77(1), pp.29-46. 10.1007/s40265-016-0670-4

Dale, M. and van Duijn, E. (2015). Anxiety in Huntington’s Disease. The Journal of Neuropsychiatry and Clinical Neurosciences, 27(4), pp.262-271.

Wallace M, Downing N, Lourens S, et al. Is There an Association of Physical Activity with Brain Volume, Behavior, and Day-to-day Functioning? A Cross Sectional Design in Prodromal and Early Huntington Disease. PLoS Currents. 2016;8.

Wetzel HH, Gehl CR, Dellefave L, et al. Suicidal ideation in Huntington disease: The role of comorbidity. Psychiatry research. 2011;188(3):372-376. doi:10.1016/j.psychres.2011.05.006.

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