Neuropsychiatric Symptoms of Huntington's Disease Clinical Practice Guidelines (2019)

Journal of Huntington's Disease

This is a quick summary of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

March 04, 2019

Guidelines for treating neuropsychiatric symptoms of Huntington’s disease were published in November 2018 in the Journal of Huntington’s Disease.[1,2]

Guidelines for Agitation in Huntington’s Disease (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.

If 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.


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