Chapter 4-Physicians Guide To The Management of HD
The Cognitive Disorder
The cognitive disorder in HD is considered a "subcortical" syndrome
and usually lacks features such as aphasia, amnesia, or agnosia that are associated with dementia of the Aizheimer's type.
The most prominent cognitive impairments in HD involve the so-called
"executive functions" abilities such as organization, regulation and perception. These fundamental abilities
can affect performance in many cognitive areas, including speed, reasoning, planning, judgment, decision making, emotional
engagement, perseveration, impulse control, temper control, perception, awareness, attention, language, learning, memory and
Several studies have suggested that cognitive and behavioral impairments
are greater sources of impaired functioning than the movement disorder in persons with HD, both in the work place and at home.
In addition, family members most often report that placement outside the home is initiated because of cognitive and behavioral
deterioration rather than motor symptoms.
This chapter provides an overview of cognitive impairments and the
related behavior problems that typically accompany HD. In addition, compensation and adaptation strategies are provided, which
physicians may recommend to patients, families and other professionals.
The Progression of Cognitive Impairments
Although performance in IQ tests often remains within the normal range
in the early stages of the disease, cognitive deficits are evident in speed of processing, cognitive flexibility (or the ability
to shift topics readily) and the organization of complex information.
The most sensitive indicator of early HD on the Mini-Mental State
Examination is serial sevens (the ability to subtract 7 from 100 serially) and the most sensitive subscale on the Mattis Dementia
Rating Scale is initiation (the ability to begin and maintain verbal and motor behaviors).
There exist few longitudinal studies of the cognitive decline in HD.
Based upon the information available, speed, organization, and initiation of behavior are impaired in early HD, constructional
impairments worsen in mid-stage HD, and some abilities remain relatively spared (memory, language comprehension) even in the
later stages of the disease.
Clinically, as the disease progresses, the severity of cognitive impairments
increases and patients are often unable to speak or communicate their views in late stages.
Difficulties in planning, organization, sequencing and prioritizing
can affect responsibilities at home and at work. Daily tasks, such as attempts to follow a recipe, to maintain a daily planner,
to complete a list of household errands, to develop a meeting agenda, or to apply for social security benefits, become daunting.
Many early-stage HD patients complain of problems with organization
and report that they just "can't get things done." There are several ways to compensate for poor organization, which can be
instituted early in the disease.
Routines should be established at work or in the
home so that the environment can provide structure and organization. Activities should be organized so that each day is
TABLE 6: COPING STRATEGIES FOR PLANNING|
Rely on routines, which can be easier to initiate or
continue without guidance.
Make lists which help organize tasks
needed to do
Prompt each step of an activity with
external cues (routine, lists, familiar verbal cues).
limited choices and avoid open ended questions.
Use short sentences with 1-2 pieces of information.
basically the same. For example, 7:00 shower, 7:30 breakfast, 8:00
take bus to work, 8:30 check mail, 9:30 dictate letters, 10:00 coffee, 10:30 staff meeting, 12:00 lunch, 1:00 return phone
calls, 2:30 review accounting, 4:00 open meeting to schedule with customers, 5:00 take bus home, 6:00 dinner, 7:00 family
time with kids, 8:30 time with spouse, 9:30 read, 10:00 lights out.
A central location could be established for posting a daily schedule.
Persons who never before used daily planners or computer calendars may need to start. A centralized message center can be
used to make lists and organize tasks to be accomplished each day. Additional strategies for dealing with poor organization
are offered in Table 6.
Lack of Initiation
Some family members complain that the person with HD "just sits
around all day and won't do anything." Regulation of behavior involves getting started, maintaining the desired behavior
and stopping unwanted behaviors.
The initiation, or starting of an activity, conversation or behavior
is often compromised in HD. A lack of initiation is often misinterpreted as laziness, apathy or lack of interest,
and may be a reason for poor performance at work.
Once started, persons with HD may be able to execute the behaviors
adequately (i.e., compute taxes, calculate sales, administrate employees, teach school), but may be unable to organize and
initiate the behaviors at the appropriate time.
External initiation often helps the person with HD remain active and
participate in both social and work activities. Keeping a daily routine can minimize the need for internal initiation. Maintaining
the desired behavior is usually less of a problem for persons with HD. If this aspect of regulation is impaired, however,
the HD patient may be unable to regulate ongoing behaviors in an appropriate manner.
Perseveration, or being fixed on a specific thought or action,
can occur when behaviors are inadequately regulated by the brain. Spouses often report that patients become behaviorally rigid,
and tend to get stuck on an idea or task. Established routines
and gentle reminders of changing tasks can help avoid problems.
An activity that is atypical for the established routine will be particularly
stressful and challenging for the person with HD. For instance, travel out of town, or a visit to the doctor or dentist, may
disrupt a safe routine. When shifting to a new task, help prepare the person with HD and allow plenty of time for him to adapt
to the new idea. There is a delicate balance of how much preparation is needed. Telling of a change in plans too early can
cause increased anxiety. Typically, inform the HD patient only one day prior to an event or a few hours before. Allow plenty
of time and frequent gentle cues to allow the shift to take place.
Some persons with HD experience difficulties with impulse control
and may develop problem behaviors such as irritability, temper outbursts, sexual promiscuity and acting without thinking.
Some degree of impulsivity and dysregulation of behaviors is quite common in HD.
Some strategies to help family members and caregivers cope with impulsivity
are addressed below.
TABLE 7: COPING STRATEGIES FOR IMPULSIVITY
- Since the person with HD cannot control his responses, a predictable
daily schedule can reduce confusion, fear and, as a result, outbursts.
- It is possible that a behavior is a response to something that needs
your attention. Don't be too quick to discount it as an outburst.
- Stay calm. This will help you remain able to think and not react
emotionally and impulsively yourself. In addition, staying calm may help the person calm down.
- Let the person know that yelling is not the best way to get your
attention and offer alternative methods for getting your attention.
- Remember, although the things being said are hurtful or embarrassing,
generally the person is not doing this intentionally. This is the HD talking, not your loved one.
- The person may be remorseful afterward. Be sensitive to his efforts
- Do not badger the person after the fact. It won't help. Remember,
this lack of control, likely, is not by choice.
- Medications may be helpful for outbursts and sexually inappropriate
behavior. Talk to your physician.