Agitation is an extreme irritability behavior. It is not unique to HD, and often occurs in other diseases causing cognitive impairment and dementia. It can also be the result of any severe medical illness, trauma, or be related to drug use or withdrawal. Agitation in HD shows up in many ways. In milder forms it looks like general mental or physical restlessness but can progress to more severe forms with verbal or physically agressive behaviors. It can come on quickly without warning, but more commonly is preceded by other neuropsychiatric symptoms of HD.

Because agitation can be thought of as extreme irritability, identifying and and treating milder forms of irritability is vital. When agitation occurs in HD, whether quickly or gradually, the treatment broadly follows recommendtions for agitation when it occurs with other medical conditions.

Agitation can look like general restlessness, or be an exaggerated response to a minor aggravation. It can progress to more severe forms of behaviors including pacing, anger, screaming, and verbal or physically agressive behaviors. It is vitally important to treat other neuropsychiatric symptoms of HD such as irritability, obsessive compulsive behaviors(perseveration), poor sleep, depression and anxiety, all of which are behaviors that can can escalate to agitation.

And it's important to remember that agitation in an individual with HD may be due to things other than HD. Particularly if agitation comes on quickly, the cause may be other medical problems like infection or trauma. Frequently agitation may be the result of building frustration caused by the increased difficulty of doing routine physical and mental tasks or being unable to communicate needs such as hunger or pain.

Clinical Practice Guidelines for Agitation in HD

Note that this author has changed some of the wording that appears in the journal article for purposes of making it more user friendly. If you wish to share with your medical provider, it would be best to copy the journal link [Anderson KE 2018] for the doctor's review.

General recommendations

  1. Make sure the agitation is not being caused by factors not related directly to HD. (Fever, infection, trauma, new drug, etc)
  2. Identify and promptly treat irritability, sleep problems and other neuropsychiatric symptoms of HD (an office visit for treatment of these symptoms may prevent the emergency room agitation crisis)
  3. Identify and modify environmental factors like excessive noise or other over-stimulation, or unmet needs such as hunger or pain that may have precipitated the agitation

Behavior recommendations

  1. Provide educational information about strategies that may lessen or prevent agitation (keep to routines, don't over-stimulate, maintain personal space. (The medical provider should help identify a cause for agitation and recommend changes)
  2. When agitation behavior isn't threatening to the person or others, the first step is to provide a quiet and safe place to calm down

Drug recommendations (note that treatment of acute agitation is different than for chronic or ongoing agitation)

  1. For agitation that is acute, or comes on quickly, and is not responding to behavioral calming down, a benzodiazepine like lorazepam (Ativan®) or an antipsychotic like olanzapine (Zyprexa®) or haloperidal (Haldol®) can be used short-term
  2. For agitation that is ongoing and causing significant distress for the individual with HD and/or carers, an antipsychotic or a mood-stabilizing drug like divalproex (Depakote®) or carbamazepine (Tegretol®) may be used. (Note that long term use of a benzodiazepine is not recommended in most chronic situations due to increased risk of falls)
  3. For agitation that has not responded to recommended medication in an individual with advanced disease who can not communicate cause of distress, a trial of pain medication should be considered

It is important to remember (as in the general recommendations) that drugs used for the treatment of agitation are associated with side effects like apathy, slowed thinking, slowed movement, trouble swallowing and loss of balance leading to falls. A trial of tapering of antipsychotic or mood stabilizing drug is indicated in most situations once agitation has been calmed.

Author's Take Home Points

Prevention of agitation is the treatment goal. Though it cannot be prevented totally, reducing the frequency and severity of this extreme behavior by treating irritability and other associated symptoms of HD is vital. Care should be taken in not treating an agitation behavior too long with drugs that mimic worsening of disease. In most situations where safety is not compromised, drug taper should be attempted. This is consistent with recommendations for agitation in other diseases of cognitive impairment.

References

Anderson KE, van Duijn E, Craufurd D, Drazinic C, Edmondson M, Goodman N, van Kammen D, Loy C, Priller J, Goodman LV. 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(3):239-250. doi: 10.3233/JHD-180293. PubMed abstract