Chapter 5-Physicians Guide To Management of HD
Symptoms not Belonging to a Specific Diagnostic Category
Patients with Huntington's disease may suffer from a variety
of emotional symptoms which do not fit any specific psychiatric diagnosis, but may nevertheless be a source of distress and
a focus of treatment including irritability, anxiety and apathy.
Some of these symptoms are related to the disease itself, and
others can be seen as a response to changing circumstances, such as a patient who becomes anxious about going to
the market because her involuntary movements attract attention.
Patients with HD may undergo personality changes, becoming irritable,
disinhibited, or obsessional. In some cases these changes represent an accentuation, or coarsening of personality characteristics
the person already had.
Other times they will be a radical departure from the patient's
usual state, which can be very distressing to families. Families should be reassured, as patients can usually be helped
by better communication, environmental interventions, and judicious use of medications.
Irritability is a common complaint from persons with HD and their families. It is often associated with a depressed
mood, but may also result from a loss of the ability of the brain to regulate the experience and expression of emotion.
Irritability in persons with HD may take the form of an increase
in the patients' baseline level of irritability, or there may be episodes of explosiveness as irritable responses to life
events become exaggerated in intensity and duration.
Other patients may not be irritable under most circumstances,
but will develop a kind of rigidity of thinking which will cause them to perseverate relentlessly on a particular desire or
idea, becoming progressively more irritable if their demands are not met. One woman, for example, insists on having ten or
twelve varieties of juice in the refrigerator at all times and was markedly irritable during a recent visit to the clinic.
Her husband had started the car to drive to the clinic and had refused to go back into the house to get her another glass
of juice. Hours later she was still dwelling on it and kept interrupting the interview to say that she wanted to go home to
have a drink.
Irritability in HD may have a variety of triggers and exacerbating
causes. It is important to understand it in context and avoid premature use of medications.
One must first understand exactly what the informant means by saying
the patient is irritable or agitated. Does the patient appear restless? Is the patient yelling or verbally abusive? Is there
potential for violence?
Many factors can precipitate an irritable episode, such as hunger,
pain, inability to communicate, frustration with failing capabilities, boredom, and changes in expected routine.
Family members and caregivers should learn to respond diplomatically,
appreciating the patient's irritability as a symptom. Confrontations and ultimatums should be avoided if the issu^ is not
The environment should be made as calm and structured as possible.
Some families achieve this more easily than others. Family settings in which there are children and adolescents, unpredictable
working hours, noise, or general chaos may lead to irritability and aggressiveness in persons with HD. Caretaker and family
support groups can provide emotional support and are a forum for sharing strategies that members have found useful in their
When irritability is severe, or enduring, or is expressed physically,
patients are often described as agitated. A great deal of overtreatment, particularly with
neuroleptics, stems from continuous use of a drug for an episodic problem. It is always necessary to revisit
the situation and see whether the drug has actually reduced the frequency of outbursts. For episodic outbursts, success often
results from combining drug therapy with a careful analysis of the context and precipitants of the outburst.
Nevertheless, we have found a number of medications helpful in treating
enduring irritability. Patients may respond to antidepressants, particularly the SSRIs (sertraline, fluoxetine, and
paroxetine) even if they do not meet all the criteria for major depression.
The optimal doses for treating irritability are not known but one
should start at a low dose and increase gradually as in the treatment of depression (see table 13). These agents may be particularly
useful when the irritability seems tied to obsessions and perseveration on a particular topic. As in the treatment of depression, improvement may not occur for several weeks.
TABLE 16: COPING STRATEGIES FOR IRRITABILITY
- Restructure the person's expectations and responsibilities to manage
frustration. The environment should be as calm and structured as possible.
- Respond diplomatically, acknowledging the irritability as a symptom.
Confrontations and ultimatums should be avoided unless the issue is crucial.
- Try to identify circumstances which trigger temper outbursts, and
redirect the person away from the source of anger.
- Family and caretaker support groups can provide valuable emotional
support and are good places to learn and share effective strategies.
Mood stabilizers such as divalproex sodium and carbamazepine
have also been helpful and could be administered as outlined for bipolar disorder (see table 15).
Low dose neuroleptics may be helpful, particularly the newer, "atypical"
ones which have fewer side effects.
Long-acting benzodiazepines, such as clonazepam (Klonopin),
starting at low doses, e.g. 0.5mg/day, have also been helpful.
The clinician must carefully monitor patients treated with these agents,
as overdosing can lead to falls or aspiration.
Apathy is common in HD and is probably related to frontal lobe
dysfunction. Apathetic patients become unmotivated and uninterested in their surroundings. They lose enthusiasm and
spontaneity. Performance at work or school becomes sluggish.
The symptom of apathy can be very troubling to families, if
they see the active person they knew slipping away. It can be a source of conflict for caregivers, who know the person is
physically capable of activities but "won't" do them.
Families need much education and support in this regard and should
learn to practice a combination of exhortation and accommodation. While apathetic patients have trouble initiating
actions, they will often participate if someone else suggests an activity and works along with them to sustain energy and
attention. For example, a man with HD had always loved fishing, but when his brother came to take him fishing for his birthday
he wanted to stay home in front of the television. The brother insisted, and when they left the house, he had a good time
fishing all day. When he returned, he immediately turned the television back on.
Apathy can be hard to distinguish from depression. Apathetic
patients, like those with depression, may be sluggish, quiet, and disengaged. They may
talk slowly, or not at all.
By and large apathetic patients will deny being sad, but in distinguishing
the two it is important to ask not only about the patient's mood, but about other depressive symptoms as well, such as a change
in sleeping or eating patterns, feelings of guilt, or suicidal thoughts.
Neuroleptics and benzodiazepines can cause or worsen apathy.
The need for these medications should be reexamined if the patient is apathetic.
TABLE 17: COPING STRATEGIES FOR APATHY
- Use calendars, schedules and routines to keep the person busy.
- Do not interpret lack of activity as "laziness."
- Patients may not be able to initiate activities, but may participate
if encouraged by others.
- Gently guide behaviors, but accept "no."
Depressed patients with apathy should be treated aggressively for
their depression, which may cause the other symptoms to remit.
It can be very difficult to distinguish depression from primary
apathy, but patients with primary apathy sometimes respond to psychostimulants such as methylphenidate (Ritalin),
pemoline (Cylert) or dextroamphetamine (Dexedrine).
These medicines are highly abusable and may exacerbate irritability.
They should be used with caution. It may be more prudent to make a trial of a non-sedating antidepressant, such as
an SSRI, first even if the patient does not seem to meet the criteria for depression, as these agents have also occasionally
Patients with HD are vulnerable to anxiety because of life circumstances,
but also because of physical changes in the brain. Patients may develop a social phobia related to embarrassment about visible
symptoms. As thought processes become less flexible, patients may be made anxious by trivial departures from the usual routine.
Patients may worry for days in advance about what to wear when going to the hairdresser or whether to attend a family function.
In addressing anxiety, attempts should be made to decrease the
complexity of the patient's environment. Stopping a job that has become too difficult may result in a remarkable decline
Assisting the caregiver in establishing a predictable routine for
the patient is helpful. Some caregivers find it useful to refrain from- discussing any special events until the day before
they are to occur. Patients who are very fearful of going to the doctor may need to be told only that they are going on an
errand until they reach the clinic.
Some patients will not improve with counseling and environmental interventions
and will require pharmacotherapy. The clinician should first assess whether the anxiety is a symptom of some other psychiatric
condition, such as a major depression. Patients with obsessive-compulsive disorder may be made anxious by obsessions about
danger or "germs," or if their rituals are interrupted.
Panic disorder, although uncommon in HD, is a highly
treatable condition. It is characterized by the acute onset of overwhelming anxiety and dread, accompanied by physiological
symptoms of rapid heartbeat, sweating, hyperventilation, lightheadedness, or paraesthesias.
Panic attacks usually last only fifteen or twenty minutes, may begin
during sleep, and may result in syncope. Suspected panic attacks require a good medical work-up, because most of the other
possible explanations for the symptoms represent highly dangerous conditions.
Once these other causes have been ruled out, the usual treatment consists
of SSRIs, sometimes temporarily supplemented with benzodiazepines. SSRIs are usually mildly stimulating and may need to start
at a lower dose than that used for depression.
Benzodiazepines should be used judiciously in anxious
persons with HD because of the vulnerability of these patients to delirium and falls and because of their potential
for abuse, especially in patients whose judgement may already be impaired. PRN medications may have to be controlled by a
family member. Some patients will respond to the non-benzodiazepine anxiolytic buspirone, which can be started
at 5mg two to three times per day and advanced to 20-30mg per day in divided doses.