How to manage agitation in Huntington's disease (HD)?

Medical Advisory BoardAll articles are reviewed for accuracy by our Medical Advisory Board
Article Review Status
Submitted
Under Review
Approved

Last updated: May 12, 2025 • View editorial policy

From the Guidelines

To manage Huntington's agitation, SSRIs are considered first-line treatments due to their efficacy in reducing overall neuropsychiatric symptoms, agitation, and depression, as evidenced by recent guidelines 1. When managing agitation in Huntington's disease, a comprehensive approach is crucial. This approach should combine non-pharmacological and pharmacological interventions. Non-pharmacological strategies include:

  • Creating a calm, predictable environment with reduced stimulation
  • Maintaining consistent routines
  • Using clear communication techniques
  • Considering therapies like simulated presence therapy (SPT), massage therapy, animal-assisted interventions, or personally tailored interventions, which have been shown to help agitation in similar contexts 1

For pharmacological management,

  • SSRIs, such as citalopram (10-20mg daily), are recommended as first-line treatments for their ability to improve overall neuropsychiatric symptoms, agitation, and depression in individuals with vascular cognitive impairment (VCI) and potentially in Huntington's disease, given the shared neuropsychiatric symptomatology 1.
  • Atypical antipsychotics, though effective, should be used with caution due to the increased risk of death, likely from cardiac toxicities, as noted in the management of Alzheimer’s disease or vascular dementia with neuropsychiatric symptoms 1.
  • The use of valproic acid or benzodiazepines like lorazepam may be considered for short-term management of severe agitation but should be approached with caution due to potential side effects and the risk of dependence.

It's essential to start with low doses and titrate slowly due to the increased sensitivity to side effects in patients with neurodegenerative diseases. Regular monitoring for adverse effects is critical, particularly for extrapyramidal symptoms with antipsychotics. Treatment should be individualized based on symptom severity, comorbidities, and response, with the goal of improving quality of life while minimizing side effects.

From the Research

Management of Huntington's Agitation

  • The management of agitation in Huntington's disease (HD) can be complex due to the co-existence of multiple neuropsychiatric symptoms 2.
  • A selective serotonin reuptake inhibitor (SSRI), such as sertraline, or the mood stabilizer valproate may be recommended as a first-line treatment for irritability in HD 3.
  • If the initial treatment is insufficient, a switch between these two medications or to a low dose of an atypical antipsychotic may be advised 3.
  • Buspirone may be another alternative treatment option for agitation in HD 3.
  • It is essential to identify possible comorbid psychiatric disorders, as irritability may be secondary to a psychiatric condition, and the choice of medication partly depends on the co-occurrence of a specific psychiatric disorder 3.
  • Behavioral therapy or other psychotherapeutic interventions may be helpful in reducing levels of stress and should be considered in addition to pharmacotherapy 3.

Treatment Options

  • Sertraline has been used to treat severe aggressiveness in HD, with reports of complete cessation of aggressive behavior in some cases 4.
  • Atypical antipsychotics may be used as an alternative treatment option for agitation in HD, preferably at a low dose and twice daily 3.
  • Valproate may be used as a mood stabilizer to treat irritability in HD 3.
  • Buspirone may be used as an add-on treatment for agitation in HD 3.

Considerations

  • The choice of medication for agitation in HD depends on the general medical condition, side effect profile, and drug-drug interactions with other medications in concomitant use 3.
  • Patients with advanced disease are particularly likely to be using various other types of medications, and treatment decisions should be adapted to cover all symptoms while limiting polypharmacy 2.
  • There is currently no cure for HD, and treatment is primarily focused on managing symptoms and supportive care 5, 6.

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.