Huntington Disease Guidelines

Updated: Feb 27, 2019
  • Author: Fredy J Revilla, MD, FAAN, FANA; Chief Editor: Selim R Benbadis, MD  more...
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Guidelines

Guidelines Summary

Guidelines for treating neuropsychiatric symptoms of Huntington’s disease (HD) were published in November 2018 in the Journal of Huntington’s Disease. [19, 20]

Guidelines for Agitation in HD

  • Identify and treat comorbid medical conditions that can precipitate acute agitation.
  • Either a benzodiazepine or an antipsychotic drug is the preferred pharmacologic option for treating acute agitation that is not responsive to behavioral strategies.
  • Antipsychotics or mood-stabilizing antiepileptic drugs can be used for chronic agitation characterized by recurrent and ongoing distress, or continuing threat of harm to self or others.
  • Pain medication may prove helpful for agitation when other therapies have failed.

Guidelines for Anxiety in HD

  • Treat coexisting psychiatric symptoms or comorbid medical conditions that can contribute to anxiety.
  • SSRIs are the preferred pharmacologic option for treating anxiety when it occurs either as an isolated symptom or when coexisting depression or obsessive perseverative behaviors are present.
  • Warn patient that SSRIs may lead to short-term exacerbation of anxiety. If exacerbation occurs it may be appropriate to add a short-term course (one or two weeks) of a benzodiazepine.
  • Is initial SSRI is ineffective or not tolerated, alternative serotonergic drugs (SSRI, NSRI, clomipramine) may be used.
  • Mirtazapine may be used if coexisting sleep disorder is present.
  • An antipsychotic may be used if needed for treatment of coexisting chorea.
  • Clomipramine may be used if needed for coexisting obsessive perseverative behaviors.
  • Long-term use of a benzodiazepine drug is discouraged in ambulatory individuals with HD unless all other options have failed.

Guidelines for Apathy in HD

  • Use an antidepressant when there is difficulty differentiating apathy of depression from apathy of HD.
  • Consider a trial of an activating antidepressant or stimulant drug for the non-depressed individual.
  • Warn patient that the activating antidepressant or stimulant drug may worsen irritability and sleep disturbances.
  • Consider reducing dose of medications that may have been prescribed for other symptoms and that may be contributing to apathy.

Guidelines for Psychosis in HD

  • Identify and treat comorbid medical conditions that can precipitate acute onset of psychotic symptoms
  • Use an antipsychotic drug as the first line of pharmacologic treatment for psychosis in HD.
  • If psychotic symptoms are not controlled by the initial antipsychotic, choose an alternative drug and do not exceed the maximum recommended dose. Do not combine antipsychotics.
  • Consider clozapine when psychotic symptoms have not adequately responded to other antipsychotics in those situations where interval blood testing is possible.

Guidelines for Sleep Disorders in HD

  • Treat co-morbid medical conditions, coexisting psychiatric symptoms, pain, or substance use that can contribute to sleep disturbance in HD.
  • Assess and adjust dosing schedule of drugs that may contribute either to daytime sleepiness or nocturnal insomnia.
  • Melatonin may be used when there is pattern of circadian rhythm disordered sleep.
  • Sedating antidepressants (mirtazapine or trazodone) or sedating neuroleptics (olanzapine and quetiapine) may be used for treating sleep disorders in HD.
  • Clomipramine may be used for managing coexisting obsessive perseverative symptoms.
  • Use of a benzodiazepine is discouraged in ambulatory individuals unless all other options have failed.