Huntington's Disease (HD) is an inherited neurological disease characterized by progressive loss of control over movements, thinking, and emotions. The behavioural changes of HD are largely a result of degeneration of select parts of the brain, specifically the basal ganglia and within that the caudate nucleus. The person with HD is undergoing specific progressive changes in the brain that directly affect body movements, feelings, thinking perception of the world and behaviour in general. Only when this is understood and accepted successful and sensitive management of behaviour take place.

HD is commonly characterized by choreatic movements, although every person affected by HD is unique. In addition to chorea, changes in behaviour occur frequently as well. In fact, for some individuals and families these behaviour changes are the primary disabling condition, not the chorea. Neuropsychological research has been important in characterizing these behavioural changes as a result of the disease and not the personality or character of the individual with HD.

This booklet will address the behavioural changes commonly associated with HD. Chorea will not be discussed except in its effects on other behaviours. In addition, the booklet will provide strategies for caregivers to use when interacting with a loved one with HD.

Behavioural Changes in HD

Speech and Communication

One of the most striking aspects of HD is the limitation it causes on speech. Individuals with HD typically have significant difficulties with starting conversation (initiation) and speaking clearly (articulation). In fact, many people become totally unable to communicate or express themselves in the late stages of HD. This problem is due to damage in several parts of the brain. These areas are involved in controlling the muscles needed for speech (such as the tongue), the speed of thinking, and finding the words one wishes to say. The loss of the ability to express ones' thoughts, ideas and needs can be isolating, dehumanizing and frightening. Although several strategies are offered below, it may be important to ask the person if they would like some help before giving it. It is important to note here that although speaking is difficult for persons with HD, understanding what others say is often relatively normal, even in the later stages of HD.

Strategies for Communication

1. Allow the person enough time to answer questions to express him/herself. Remember, it is not necessary to speak more slowly yourself, since the person with HD generally understands well. He or she just needs additional time to respond.

Offer cues and prompts to get him or her started. Fill in words that may be on the tip of the tongue, but be sensitive to "putting words in someone's mouth."

3. Rather than asking, "What do you want for dinner?", give specific choices like, "Do you want hamburgers or meat loaf?" or "Would you like Mexican or Japanese food?".

4. Alphabet boards, Yes-No cards, or technical communication devices should be made

available to every person with HD to allow for at least a simple method of communication.

5. Once verbal communication is impossible for someone with HD, do not stop talking to him or her. This can only worsen the feelings of isolation that come with losing speech.

Memory and Forgetfulness.

The most common cognitive complaint of persons affected by HD is memory difficulties. Briefly, memory involves three steps:

1) understanding an experience,

2) storing the experience in the brain and

3) retrieving the memory or "remembering" the experience.

Persons with HD experience difficulties primarily with stage 3. They cannot effectively retrieve the memories from storage. It can be assumed that the knowledge the person once had still exists, although the ability of the brain to bring the facts up from storage is defective.

The memory deficit also affects step 2-learning new information. For example, learning a new address and phone number, or how to use the new phone answering machine, or where to locate items at home if their usual place is changed may be difficult for persons with HD.

Finally, there is no memory gradient in HD. That is, individuals have equal difficulty with memories from all decades of their life. For example, it may be just as frustrating to remember an event that happened last week as it is to recall an event to took place 30 years ago. That is, memory from all time frames are affected equally.

Strategies for Learning

1. When trying to teach something new, break complex information into simple steps.

2. Write the steps down and encourage practising (repetition) of the steps.

3. Have the person repeat back the steps.

4. Allow ample time for the learning of new information.

5. Avoid giving complex tasks (i.e., with more than three simple steps) without using the strategies above. In advanced stages of HD it may be necessary to avoid complex tasks

entirely.

Strategies for Memory

1. Offer a list of choices rather than ask an open-ended question with an unlimited number of possible answers.

2. Provide aids such as "cues" or "hints" which offer strategies for retrieval of information.

3. Use "to do" lists, daily morning routine lists, calendars, notes, white erasable boards, an alarm wrist watch, medication schedules and boxes, and signs around the house to serve as retrieval aids and reminders.

Slowed Thinking

Thought processes are slowed in nearly all cases of HD. This general slowing presents itself as long pauses during conversations and increased time required to do previously routine tasks.

Strategies for Slowed Thinking

1. Patience! The person with HD needs time to think about and understand that has been told to them and then to formulate an answer. Give the person time to answer questions or express him/herself.

Executive Functions

Executive functions are critical to our ability to take care of ourselves and to manage job, home and family. These functions include planning, thought organization, abstract thinking, creativity, judgement, as well as control over our feelings and experiences in life. These functions will be discussed in three main areas: Organization, regulation and awareness.

A. Organization

Difficulties in planning, organization, prioritizing and sequencing can affect attempts to follow a recipe, make a decision, or prioritize lists of tasks for the day. These same organization impairments affect problem-solving, judgement and logical thinking. When the brain can no longer sequence bits of information, many aspects of intellectual, social, and personality functions are impaired. Errands which used to be completed in two hours now take all day, and do not get finished. Problems might include the following; the errands are not written down and some get forgotten, they are not put in a logical order from closest to farthest from home, necessary papers are left at home so that errand is skipped or a trip home is needed. Suppose, for example, you have to make a withdrawal at the bank machine, pick up the dry cleaning, and stop at the market for milk. If you cannot plan for these activities or sequence your errands, you may not have the dry-cleaning ticket for the clothes at the dry cleaner or you may get to the store without any money to pay for the milk.

Strategies for Planning

1. Structure specific tasks to do for the HD person or have them make a list beforehand.

2. Make all steps of tasks visible and clear in written format.

3. Organize tasks spatially so that sequence or structure is more visible.

Strategies for Decision Making

1. Restructure your expectations. Ideally, loss of ability will take place slowly and family members need to discuss these limitations in a diplomatic way to minimize effects on the patient's self-esteem.

2. Avoid right-wrong, yes-no, power struggles and confrontations with the person.

3. Offer choices to the HD patient which are all available options.

4. Avoid open-ended questions (e. g., What would you like for lunch?" Instead offer choices (e.g., would you like " x " or " y?").

5. Keep verbal requests simple. Use short sentences. Ask the person to repeat back to you something that you tell him . Although language comprehension is usually relatively intact in HD patients, they often have problems paying attention and organizing what was said.

Long, grammatically complex sentences may be difficult for them to organize or to attend to.

Strategies for Attention/Concentration

1. Locate the HD-affected person in a quiet environment.

2. Tell the HD-affected person to focus on a person's eyes when given instructions.

3. Make expectations clear cut.

4. Break down activities into simple steps.

5. Do one step of a task at a time.

6. Underline key points in directions.

7. Avoid excess materials.

B. Resolution

Damage to the caudate nucleus and other areas make it difficult to initiate new behaviours (e.g., strike up a conversation) to maintain behaviours in progress (e.g., continuing to fold clothes until completely finished) to inhibit opposing behaviours (scratch self where it itches), and to end or stop behaviours (e.g., to stop self from rehashing and recounting an unpleasant interaction with another person).

Strategies for Temper Outbursts

1. First, redirect the HD person away from the source of the anger.

2. Try to identify "triggers" and then avoid them. A determination should be made as to the possible underlying causes or triggers to the behaviour. The person may be hungry or in pain. He or she may be attempting to communicate, but be unable to do so. He or she may want a coffee refill.

3. Assess your own expectations regarding the HD affected individual. The irritability may stem from inflexible interpersonal interactions. A family member may assume that the patient is capable of carrying out the same responsibility as before the onset of illness, and may insist that the person do so. Or the person may be unwilling or unable to accept new limitations. Therefore, there must be a restructuring of interactions, expectations and responsibilities.

4. Although initially very challenging for family members, they should learn to respond diplomatically, appreciating the irritability as a symptom of HD.

5. Confrontations and ultimatums should be avoided.

6. The environment should be as calm and structured as possible.

7. Establish daily routines and break them as infrequently as possible. People with HD tend to become mentally inflexible and are typically comforted by stability.

8. When all else fails, there are medications which can help with irritability. It is important to see a physician (a neurologist or psychiatrist is best) who has current knowledge of Huntington's Disease.

9. Finally, family members are responsible to themselves and to each other to provide a safe environment so that no person is ever in danger. Remove potential weapons from the house (e.g., guns, ammunition, large knives and so on) and have emergency numbers taped to the phone receiver. HD in itself does not cause an individual to become dangerous, but a loss of normal regulation of impulses can contribute to unsafe situations.

Initiation

Many family members describe how the person with HD "just sits in front of the TV all day long." Though this can be a sign of depression, it also is a good example of decreased initiation, or how HD impairs an individual's "get up and go." HD patients often become apathetic and have trouble initiating behaviour. Apathy can be an important and disabling symptom at any time during the course of the illness, and once present tends to persist or worsen. People with HD become unmotivated and uninterested. They lose enthusiasm and spontaneity and may appear indifferent. Performance at work and school becomes sluggish. The affected person may be content to sit in front of the television all day. Apathy can be particularly troubling to the family who no longer see the usually active and energetic person they once knew. It can be a source of conflict when caregivers know the person is physically capable of activities but won't do them.

Strategies for Initiation

1. Don't interpret non-activity as " laziness."

2. Provide psychological support and education to family members and friends.

3. Sometimes all that is needed is a "jump start" (i.e., an idea or suggestion, with an offer to help them get started).

4. It may be necessary to avoid asking things in a question form and to state things directly, politely and respectfully (e.g., "Come on, Mom, it's time to go to the... "). Gently guide the individual to initiate or begin behaviours, such as a walk or a card game. Although this strategy may seem "bossy" most people appreciate the direction and support.

5. Adding structure to the day can help. Set up a daily schedule in writing with routine activities developed with the person. Once initiated, the person can continue to enjoy many

activities.

Repetition of Behaviour - Another behaviour often reported by family members is that the person with HD becomes "stuck" or perseverates on one idea or activity. Individuals may become rigid in their behaviour and become unable to change easily from one activity to another or to change their routines. Rigidity ("stubbornness") and difficulty stopping behaviours can also create verbal and physical outbursts.

Strategies for Repetition of Behaviour

l. Maintain a list of activities or food that are of interest to the individual. These items can be used to break a cognitive "set" (a topic that the person is stuck on) when needed. Use items to gently shift patients off of a perseverative topic. Many caregivers effectively use humour to shift a perseveration.

2. Some family members have found it helpful to establish a time limitation for some activities or behaviours that are repeated continuously.

C. Unawareness

Many people with HD suffer from anosagnosia (lack of self-awareness) and don't recognize their disabilities. That is, they are unable to evaluate their own

behaviour. It is like looking down at yourself and not being able to "see" what you are doing, saying or feeling. There is rarely any benefit in someone with HD accepting his or her illness and all of its consequences. Therefore, we encourage family and staff members to accept the "denial" as a common symptom of HD.

Strategies for Unawareness

1. There is no single way to cope with this difficulty. At times it may require the caregiver

to use creative thinking to get the person with HD to cooperate with a request.

2. Accept "unawareness" of illness as a component of HD that is not a treatment obstacle.

3. Avoid interpreting noncompliance with therapy or nursing care as intentional. It may be

helpful to develop a contract that includes incentives for compliance. It is important that the

rewards be things the person likes (e.g., foods, activities), not just things the caregiver chooses.

4. A formal written agreement that explains expectations will increase the chances of success.

Visual Spatial Ability

The ability to perceive body position in the environment is impaired even in the early stages of HD. For instance, the ability to perceive where your house is (to the right or to the left) when taking a walk, or the ability to sense how close your tie is to your soup or how close your arm is to the burner on the stove are examples of how impaired "body sense" can get in the way of day-to-day activities. Some family members have reported a difficulty with directions (right, left, north, south) and maps, early in the HD disease. In addition some observed deficits are due to poor organizational ability and slowed motor ability in general.

Strategies for Visual Spatial Disability

1. Avoid dangerous or complex tasks which require body awareness.

2. Create situations where the HD affected individual can prepare their own meal without having to turn on a burner or use a sharp knife. It is important to maintain independence as long as possible.

3. Avoid independent travel in new areas. Provide structure and assistance in new neighbourhoods.

4. Write directions down in concrete steps without reliance on spatial cues. For example, don't say go down the road 1-1/2 miles, go east and head towards the lake. Instead, say, go straight on Maple until you reach Oak. Go right on Oak, past the grocery store until you see the lake up ahead.

Reading

Difficulty reading may be due to visuospatial disorientation, distractibility, or inattention.

Strategies for Reading Disability

1. Large print newspapers and books are available in most community libraries and book stores.

2. Books on tape may be appropriate for those who still appreciate literature but are unable to track text, turn pages, or hold the book still.

3. For general notes around the house and "refrigerator lists," use coloured markers to outline specific information to focus upon.