Huntington’s disease (HD) is a neuropsychiatric illness, which means that people with HD suffer from both physical symptoms, like chorea, and mental symptoms, such as depression or problems with thinking and memory.  This article provides an overview of behavioral symptoms common in HD, and suggests strategies for how to best manage them.

Psychiatric and behavioral symptoms in HD are a direct result of changes in the brain caused by the illness. This happens because HD damages important structures and pathways in the brain—and this damage causes the problems with movement, thinking, and behavior. Though the psychiatric and behavioral symptoms can be challenging for patients and families, it’s important to remember that these problems are caused by the illness, and not by the person suffering from HD. Additionally, it’s crucial to keep in mind that people with HD face many losses, including loss of independence, health, and eventually, their lives. These losses burden HD patients with frustration, anger, and grief in addition to any psychiatric and behavioral symptoms they may have from the illness. Understanding that the issues in HD stem from a combination of brain changes and the effects of profound loss helps to guide coping strategies for patients and families.

As we discuss difficult behaviors that your HD loved one may be experiencing and explore strategies to deal with these behaviors, it is important that you remember to:

• Take breaks
• Tag team
• Allow change and flexibility
• Breathe
• Forgive yourself and others
• Each day is a new day

Overview of Common Behaviors in HD

Anxiety

It is common for people with HD to experience anxiety. Anxiety can be described as an inner feeling of discomfort, worry, panic, restlessness, dread or apprehension, often with no clear explanation for the feeling. Often imminent events, change and situations with uncertain outcomes will increase, or provoke anxiety. Anxiety differs from fear, which is a response to a clear and actual danger.

The presence of these symptoms can worsen challenging behaviors, therefore recognizing and treating symptoms is critical. Anxiety can manifest in the form of outbursts of irritability, agitation, insomnia and in severe situations, panic attacks. Fatigue is often associated with anxiety. A person with HD and anxiety may constantly ask for reassurance and not want to be left alone. Or they may closely follow a cargiver or family member around. They may also be restless and pace or fidget.

There are medications that can be used to treat anxiety in persons with HD.

Apathy

Symptoms of apathy include a lack of motivation to do, complete, plan, or accomplish anything. This can also be accompanied by a person’s unwillingness to act upon tasks or activities that need attention. This lack of motivation may also include low energy levels. Activities or events that your loved one with HD used to be interested in may now create little to no response.

Apathy can also cause diminished emotional responses to news or personal events and seem to be uninterested or detached and indifferent. Some symptoms of apathy are also common in people who have depression. The main difference is that a person with depression will often feel sad, tearful, hopeless or have low self-esteem whereas a person with apathy is often not bothered by its symptoms.

People with HD tend to become more likely to develop apathy as their condition progresses. However, apathy can start during the very early stages of HD. Apathy is caused by damage to the frontal lobes of the brain.

Declining Executive Function

The loss of executive function can lead to problems with the speed of thinking, planning, prioritizing, concentration, decision-making, flexibility, or creativity. It can also impair the ability to manage time, pay attention, switch focus, and remember details and multitask. When executive function isn’t working as it should, behavior is less controlled. There are no medication options to improve the loss of executive function.

Depression and Suicide

It is common for people with HD to experience depression. Depression can lead to feelings of hopelessness and persistent sadness, inactivity, difficulty in thinking and concentration and significant changes in appetite and time spent sleeping. Persons with depression may also have difficulty with concentration, irritability or cry often and have little or no interest in once pleasurable activities.  

A HD loved one that has depression may have frequent thoughts about death or suicide. The presence of these symptoms can worsen challenging behaviors, therefore recognizing and treating symptoms is critical.  There are medications that can be used to successfully treat depression.


Suicidal thoughts and statements in patients with HD should always be taken seriously and addressed immediately. If suicidal thoughts are present, even in the absence of an eminent plan, it is important to remove any weapons from the home (guns, bullets, knifes, etc). If someone with HD expresses intent to harm themselves or has a plan to commit suicide, it is an emergency, and action must be taken (Call 911).

Irritability and Disproportionate Anger

Sometimes, individuals with HD may experience inappropriate anger that seems out of proportion to the situation at hand. Individuals may lose patience more quickly than they used to, or may not be able to “shrug off” minor irritations. The person’s anger may escalate quickly, and they may yell, slam doors, throw objects, or even hurt others. This can be especially challenging if the person with HD also suffers from unawareness and can’t process the fact that their thoughts and moods don’t reflect reality. Irritability and anger often stem from frustration about losses (e.g. abilities, independence) combined with brain changes that decrease the ability to regulate emotions.  There are medications that can be used to successfully treat irritability and disproportionate anger.

Perseveration and Obsessive Compulsive Behaviors

Perseveration and obsessive compulsive behavior is best described as a fixation or being stuck on a thought, behavior, word, activity, or emotion in the absence of an ongoing reason for its recurrence. An individual with HD may ask the same question, make the same statement, or repeat the same behavior or activity over and over. Often there is great difficulty moving onto a new topic or activity and they quickly return to a more favored topic/activity. They seem driven to continue in a way you could compare to an itch that cannot be scratched.

Stress and anxiety can trigger these behaviors and make them intensify when present already. These recurrent, intrusive thoughts paired with repetitive behaviors can be a way of reducing inner discomfort. It is important to understand that when a person is perseverating they feel unable to stop. Perseveration and obsessive compulsive behavior may or may not interfere with daily life or activities or they may be more extreme and cause concern for safety. In either case these behaviors are often difficult for loved ones to cope with and manage each day. There are medications that can be used to successfully treat perseveration and obsessive compulsive behavior.

Psychosis – Delusions and Hallucinations

Delusions are false beliefs, often held with strong conviction. As an example, an individual may be paranoid that someone did something or someone/something intends to harm them. Hallucinations may be auditory (sounds or voices), visual (forms, animals, people) or even smells or tastes. Insight to delusions or hallucinations may or may not be preserved (the ability to recognize the perception is not real). Psychosis in individuals with HD may be caused by changes to the brain manifested in HD, be medical in origin (certain medications, illnesses, infections or traumas) or can be caused by recreational drugs/alcohol. A person experiencing psychosis may be a danger to themselves or others and should be medically evaluated.  New onset of delusions and/or  hallucinations is cause for prompt medical evaluation and treatment.  There are medications that can be used to treat psychosis in individuals with HD.

Substance Abuse or Dependence

Substance abuse and dependence can includes alcohol, recreational drugs and/or tobacco. Abuse or dependence on substances can mask and/or intensify behavior symptoms.  It may also be used to “self-medicate” from the symptoms of HD.  Substance abuse interferes and/or disrupts daily life, social relationships, work performance, etc. Substance abuse has been shown to accelerate onset of the motor (physical) symptoms of HD.

Unawareness / Lack of Insight

Anosognosia (unawareness or lack of insight) is a word of Greek origin that roughly translates to “without knowledge of disease”. It is common for individuals with HD to experience an unawareness of the severity of their symptoms/behaviors.  Someone with anosognosia isn’t simply in denial or being stubborn. Their brain can’t process the fact that their thoughts and moods don’t reflect reality. Anosognosia can lead to refusal to take medications and/or seek treatment. Lack of awareness in HD is caused by damage and interruptions in the circuits within the brain. 

Unawareness and denial are often used synonymously, but they are different. Denial is thought to be under the control of the individual to “protect” them from reality at that period in time, where by unawareness is not something the individual with HD can control as they just don’t have any recognition that something is different or changed. This is often more problematic for the family and caregivers and can pose a risk to safety.

Unawareness isn’t always all-or-nothing. Some people partially lose the ability to see themselves clearly, or it can come and go. That can confuse friends and loved ones. It’s hard to understand why someone seems to fully understand their diagnosis one moment, then claim they’re perfectly healthy the next, even though objective evidence shows they aren’t. There are no medications to treat unawareness or lack of insight. 

Adapted from an article by: Vicki Wheelock, MD, Lorin Scher, MD, Barbara J. Kocsis, MD, Terry Tempkin, NP, and Lisa Kjer-Mooney, LCSW