Chapter 5-Physician's Guide to Mangement of HD
While depression is the most common psychiatric problem in HD,
a smaller number of patients will become manic, displaying elevated or irritable mood, overactivity, decreased need for
sleep, impulsiveness, and grandiosity.
Some may alternate between spells of depression and spells of
mania with times of normal mood in between, a condition known as bipolar disorder. Patients with these conditions are usually
treated with a mood stabilizer. Lithium is probably still the most popular mood stabilizer for people with idiopathic
bipolar disorder, but we have not found it to be as helpful in patients with HD.
It is not known why this is the case. Lithium has a narrow therapeutic
range, particularly in patients whose food and fluid intake may be spotty, but there may be some other aspect to the mood
disorders found in HD patients which make them poor lithium responders.
We recommend beginning with the anticonvulsant divalproex sodium (Depakote)
or valproic acid (Depekene) at a low dose such as 125 to 250mg po bid and gradually increasing to
efficacy, or to reach a blood level of 50-150mcg/ml. A dose of 500mg po bid is fairly typical, but some patients will require
as much as several grams per day.
Another anticonvulsant, carbamazepine (Tegretol),
is also an effective mood stabilizer. This can be started at 100-ZOOmg per day, and gradually increased by lOOmg/day to reach
an effect or a therapeutic level of 5-IZmcg/ml, which may require a dose of 800-1 ZOOmg/day.
Therapeutic ranges for these drugs were established on the basis of
their anticonvulsant properties, so it is important to remember that a patient may show a good psychiatric response below
the minimum "therapeutic" level (but generally should not exceed the maximum level in any case).
Both drugs carry a small risk of liver function abnormalities
(particularly divalproex sodium) and blood dyscrasias (particularly carbamazepine), and so LFT's, and CBC should be
routinely monitored every few months and clinicians should be alert for suggestive symptoms.
Valproic acid may cause thrombocytopenia, and both drugs are associated
with neural tube defects when used during pregnancy.
Manic patients with HD who have delusions and hallucinations
may require a neuroleptic, and patients who are very agitated may need a neuroleptic or a benzodiazepine for immediate
control of these symptoms.
As discussed for depression, the doctor may wish to prescribe one
of the newer antipsychotics which have fewer parkinsonian side effects, such as risperidone, olanzepine, or quetiapine.
In cases of extreme agitation, a rapidly acting injectable
agent, such as droperidol (Inapsine) or lorazepam may be necessary.
Finally, ECT is known to be a very effective treatment for idiopathic
mania and should be considered when other treatments fail, or when the individual is extremely dangerous.
TABLE 15: MEDICATIONS USED FOR MANIA IN HD|
|Neuroleptics (see table 14)
||G.I. upset, sedation, tremor, |
liver toxicity, throbocytopenia
||sedation, dizziness, ataxia, rash, |
bone marrow suppression
Obsessions are recurrent, intrusive thoughts or impulses
which are experienced as being senseless, at least initially.
A compulsion is a repetitive performance of the same
activity, a stereotyped routine which must be followed, often in response to an obsession, such as handwashing because of
an obsessive concern with germs.
Obsessions are usually a source of anxiety and the patient may
struggle to put them aside, whereas the acting out of compulsions generally relieves anxiety and may not be as strongly resisted.
True Obsessive-Compulsive Disorder (OCD) is rare in HD, but HD patients
often display an obsessive preoccupation with particular ideas. Patients may worry about germs or contamination,
or engage in excessive checking of switches or locks. Sometimes patients will become fixated on an episode of being wronged
in the past (e.g. fired from a job, divorced, driver's license revoked), and then bring it up constantly, or become preoccupied
with some perceived need, such as a desire to go shopping, or to eat a certain food.
Serotonergic antidepressants are used to treat OCD
and may ameliorate obsessions and compulsions in HD patients that do not meet the criteria for the full syndrome. The use
of the tricyclic antidepressant clomipramine (Anafranil) has largely been superceded by the SSRIs
fluoxetine, sertraline, paroxetine and fluvoxamine (Luvox) which have milder side effects and lower lethality
Patients may require higher doses than those needed for depression,
e.g. 40-60mg of fluoxetine. For relentless perseverative behavior unresponsive to these agents, one might consider neuroleptics,
keeping in mind that the newer, atypical drugs may be better tolerated.
Schizophrenia and schizophrenia-like conditions are much less
common than affective disorder in HD.
The new onset of delusions and hallucinations should prompt
a search for specific causes or precipitating factors, including mood disorders, delirium related to metabolic or neurologic
derangements and intoxication with or withdrawal from illicit or prescription drugs.
Once these possibilities of mood disorder, drug intoxication, and
delirium have been considered, neuroleptics may be employed for HD patients with schizophrenia-like syndromes.
The doses used for treatment of psychosis may be somewhat higher than those used for treatment of chorea.
As mentioned before, if neuroleptics are not needed for the control
of involuntary movements, patients may do better on newer agents such as risperidone, olanzepine or quetiapine which
do not cause as many extrapyramidal side effects.
Some patients will respond completely and others only partly, reporting
that "voices" have been reduced to a mumble, or becoming less preoccupied with delusional concerns.
Patients with delusions will rarely respond to being argued
with, but a clinician may certainly express skepticism regarding a delusional belief and explain to the patient that it may
be the product of a mental illness.
Caregivers should be encouraged to respond diplomatically,
to appreciate that the delusions are symptoms of a disease, and to avoid direct confrontation if the issue is not crucial.