Chapter 5-Physician's Guide to Mangement of HD

Mania

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
MEDICATION	STARTING DOSE	MAXIMAL DOSE	SIDE EFFECTS
Neuroleptics (see table 14)	see table	see table	see table
Divalproex sodium	250mg	500-2000mg	G.I. upset, sedation, tremor, liver toxicity, throbocytopenia
Carbamazepine	100-200mg	1200-1600mg	sedation, dizziness, ataxia, rash, bone marrow suppression

Obsessive-Compulsive Disorders

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 in overdose.

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-Like Disorders

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.