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Standard of Care Treatment and Symptom Management

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While there is currently no cure for Huntington disease, symptoms can be effectively managed to improve quality of life. Treatment focuses on VMAT2 inhibitors to calm involuntary movements (chorea), psychiatric support for mood changes, and a multi-specialty care team to assist with daily living.

Key Takeaways

  • • VMAT2 inhibitors are the standard medications used to reduce the involuntary movements known as chorea.
  • • Certain chorea medications carry a high risk of worsening depression or suicidal thoughts, requiring careful psychiatric monitoring.
  • • Managing psychiatric symptoms like depression, irritability, and apathy is crucial for maintaining daily function and social well-being.
  • • Patients require specialized nutritional support and a high-calorie diet to combat the severe weight loss associated with the disease.
  • • Optimal management requires a multi-specialty care team, including neurologists, psychiatrists, and various specialized therapists.

Managing Huntington Disease (HD) focuses on improving quality of life by treating symptoms as they appear. While there is no disease-modifying therapy (a cure that stops the disease) yet, there are highly effective ways to manage movement and mood [1][2].

Managing Movement: VMAT2 Inhibitors

For many, the most visible symptom is chorea—the involuntary, jerky movements of the body. The primary medications used to calm these movements are called VMAT2 inhibitors [3][4].

How They Work

Think of dopamine as a chemical messenger that tells your muscles to move. In HD, there is often an “overflow” of dopamine signals. VMAT2 inhibitors act like a dam, reducing the amount of dopamine released into the brain, which helps “turn down the volume” on involuntary movements [3][5].

There are three FDA-approved medications in this class:

  • Tetrabenazine (Xenazine): The first approved medication for chorea. It typically requires dosing 2-3 times per day [6].
  • Deutetrabenazine (Austedo): A newer version that stays in the body longer, often allowing for twice-daily dosing with fewer “peaks and valleys” in side effects [7][8].
  • Valbenazine (Ingrezza): The most recent approval, which is taken just once a day [9].

Critical Safety Warning

It is crucial to be aware of the psychiatric side effects of these medications. Tetrabenazine and deutetrabenazine carry a strict FDA Black Box Warning for an increased risk of depression and suicidal thoughts or behavior in HD patients [10][11]. Because people with HD are already at a higher risk for depression, you and your family must monitor for severe mood changes when starting these drugs. Valbenazine (Ingrezza), the newest drug in this class, does not currently carry this specific boxed warning for HD, which is an important clinical distinction to discuss with your doctor [9][12].

Managing Mood and Mind

Psychiatric symptoms—like apathy (lack of motivation), irritability, and depression—often impact a person’s ability to work or stay social more than the movement symptoms do [13][14].

  • Suicidal Ideation Risk: It is important to know that the baseline risk for suicidal ideation is elevated in the HD population compared to the general public [15][16]. Recognizing this helps destigmatize the symptom and highlights exactly why psychiatric support is a non-negotiable part of your care.
  • Depression & Anxiety: These are often treated with SSRIs (antidepressants like fluoxetine or sertraline), which help balance serotonin in the brain [17][18].
  • Irritability: Doctors may use medications like olanzapine or risperidone to help even out mood swings and aggression [19][20].
  • Apathy: This can be one of the hardest symptoms to treat. While non-drug strategies—like using a structured daily schedule and external “prompts” from family—are essential, addressing it is critical for improving functional outcomes [21][13].

Your Multi-Specialty Care Team

Because HD affects many areas of life, a “standard of care” approach involves a team of experts working together [1][22].

  1. Neurologist: Manages your movement medications and overall neurological health.
  2. Psychiatrist: Specializes in the complex mood and behavioral changes of HD.
  3. Dietitian/Nutritionist: People with HD often experience severe, unexplained weight loss due to metabolic changes and the massive amount of calories burned by chorea. A dietitian is crucial for managing the high-calorie diet needed to maintain strength [23][24].
  4. Physical Therapist (PT): Focuses on balance, gait, and preventing falls [25].
  5. Occupational Therapist (OT): Helps you find ways to stay independent in daily tasks like cooking, bathing, or using a computer [1].
  6. Speech-Language Pathologist (SLP): Essential for managing dysphagia (difficulty swallowing) and speech clarity [26][27].
  7. Genetic Counselor: Helps you and your family navigate the emotional and practical side of an inherited disease [28].

Frequently Asked Questions

What medications are used to treat chorea in Huntington disease?
The primary medications for treating chorea are called VMAT2 inhibitors, which include tetrabenazine, deutetrabenazine, and valbenazine. These drugs work by reducing the amount of dopamine released in the brain to help calm down involuntary, jerky movements.
Are there serious side effects to taking VMAT2 inhibitors?
Yes, some VMAT2 inhibitors like tetrabenazine and deutetrabenazine carry a strict FDA boxed warning for an increased risk of depression and suicidal thoughts. It is crucial to monitor for severe mood changes when starting these medications, especially since Huntington disease already elevates depression risks.
Why do people with Huntington disease need a dietitian?
People with Huntington disease often experience severe, unexplained weight loss due to metabolic changes and the massive amount of calories burned by involuntary movements. A dietitian helps design and manage the high-calorie diet needed to maintain strength and energy levels.
How is apathy managed in Huntington disease?
Apathy, or a severe lack of motivation, can be one of the hardest symptoms to treat with medication alone. Non-drug strategies, such as using a structured daily schedule and receiving external prompts from family members, are highly effective and essential for improving daily functioning.
What kind of doctors should be on my Huntington disease care team?
A comprehensive standard of care involves a multi-specialty team working together. This typically includes a neurologist, psychiatrist, dietitian, physical therapist, occupational therapist, speech-language pathologist, and genetic counselor.

Questions for Your Doctor

  • • Which VMAT2 inhibitor do you recommend for my chorea, and how does the safety profile relate to my current mood?
  • • How will we closely monitor for depression or suicidal thoughts if I begin taking tetrabenazine or deutetrabenazine?
  • • Should my current psychiatric medications be adjusted before we try treating my chorea?
  • • Can you refer me to a dietitian to help manage my high caloric needs and prevent weight loss?
  • • How can we distinguish between my 'apathy' and 'depression,' and are there different treatments for each?

Questions for You

  • • Which symptoms—movement, mood, or thinking—are currently having the biggest impact on my daily life?
  • • How has my motivation or interest in activities changed recently? Do I find it hard to start tasks?
  • • Am I experiencing any severe side effects from my current medications, such as sudden shifts in mood or dark thoughts?
  • • Who is the primary person coordinating my various appointments and therapies?

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This page provides educational information about Huntington disease treatments and symptom management. Always consult your neurologist and multidisciplinary care team before starting or changing any medications.

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