Some good news about Huntington disease: most of the behavioral symptoms can be managed with treatments developed for other conditions.
Most patients with Huntington disease have multiple psychiatric symptoms, which are related to:
• Progressive neurodegeneration
• Psychological reaction to the disease
• Adverse effects of medication
Insufficient data exist to develop evidence-based guidelines on neuropsychiatric symptoms in Huntington disease. Thus, expert opinion–based consensus guidelines were created to help guide treatment.1
Development of the guidelines: Eleven multidisciplinary experts from the Unites States and Europe developed the guidelines based on a modified Institute of Medicine process. A group of 84 international experts on Huntington disease were surveyed for agreement on the guidelines, and at least 85% strongly agreed or agreed with almost all the statements.
Clinical experience suggests that most of the neuropsychiatric symptoms in the guidelines can be managed using treatments studied in other conditions.
The consensus guidelines provide the following recommendations for general management:
• Identify and address coexisting psychiatric symptoms, comorbid medical conditions, medications (dose, side effects, scheduling), and environmental factors (noise, pain) that may contribute to the neuropsychiatric symptom
• First consider nonpharmacologic options
• When medication is needed, first use the agent with the least unwanted adverse effects; drug choice is influenced by the stage of Huntington disease
• Limit polypharmacy
• Regularly reassess medications and the potential to reduce dosage
• Consult a psychiatrist as needed
Recommended behavioral interventions for agitation in Huntington disease:
• Education of caregivers on behavioral strategies
• The preferred initial response (when not a threat to self or others): safe, quiet space; time to calm down; gentle verbal support
Recommended pharmacologic therapy for agitation in Huntington disease:
• Acute agitation: preferred drugs are benzodiazepines or antipsychotics
• Chronic agitation: antipsychotic or mood-stabilizing antiepileptic drug
• Trial of pain medication in nonverbal patients in whom have other medications have failed
Behavioral interventions are first-line therapy in patients with earlier-stage Huntington disease who have anxiety.
Recommended medications for anxiety in Huntington disease:
• Preferred drug: SSRI
• Consider short-term benzodiazepine if anxiety temporarily increases after starting an SSRI
• If the SSRI is ineffective or not tolerated, consider an alternative SSRI, an SNRI, or clomipramine
• For a coexisting sleep disorder, consider mirtazapine
• For coexisting chorea, consider an antipsychotic
• For coexisting obsessive perseverative symptoms, consider clomipramine
• Avoid long-term benzodiazepines in ambulatory individuals, unless all other options have failed
Behavioral management of apathy in patients with Huntington disease:
• Encourage individualized social/physical activities
Recommendations for pharmacologic management of apathy:
• First-line therapy: Antidepressant (when difficult to differentiate apathy from depression)
• Consider a trial of an activating antidepressant or a stimulant in non-depressed patients
• Warn patients about potential worsening of irritability and sleep disturbance with an activating antidepressant or stimulant
Recommended medications for psychosis in patients with Huntington disease:
• Preferred therapy: Second-generation antipsychotic
• Consider a first-generation antipsychotic when chorea predominates
• Avoid exceeding maximum recommended antipsychotic dose
• Avoid combining antipsychotics (reserve only for patients with severe symptoms)
If the patient has an inadequate response, consider an alternative antipsychotic or clozapine if interval blood testing is available.
Regularly reassess the continued need for an antipsychotic. Adverse drug effects may be difficult to distinguish from disease progression.
Behavioral management of sleep disorders in patients with Huntington disease:
• Initial step: educate about sleep hygiene
Recommendations for pharmacologic management of sleep disorders:
• Consider melatonin in circadian rhythm disorders
• Consider sedating antidepressants (mirtazapine, trazodone) or sedating neuroleptics (olanzapine, quetiapine)
• Consider clomipramine with coexisting obsessive perseverative symptoms
• Avoid benzodiazepines in ambulatory individuals, unless all other options have failed
• A group of international experts has released expert consensus opinion guidelines on the treatment of neuropsychiatric symptoms in Huntington disease
• The guidelines cover general issues in management, as well as agitation, anxiety, apathy, psychosis, and sleep disorders
1. Anderson KE, van Duijn E, Craufurd D, et al. Clinical management of neuropsychiatric symptoms of Huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis. 2018;7:239-250. doi: 10.3233/JHD-180293.