Huntington Disease Treatment & Management
- Author: Fredy J Revilla, MD; Chief Editor: Selim R Benbadis, MD more...
Ablative surgical procedures and fetal cell transplantation have been attempted in patients with HD. Currently, enough data to support this type of treatment are not available. It is still experimental.
Consider general safety measures and nonpharmacologic interventions first in the management of Huntington disease (HD).
If chorea is severe enough to interfere with function, consider treatment with benzodiazepines, such as clonazepam or diazepam; valproic acid; dopamine-depleting agents, such as reserpine or tetrabenazine (approved by the US Food and Drug Administration [FDA] in August 2008); and finally, neuroleptics.
The drug tetrabenazine has shown some positive effects in the treatment of chorea, for patients with HD. It selectively depletes central monoamines by reversibly binding to the type-2 vesicular monoamine transporter.
Results from a phase III clinical study showed that this investigational drug is an effective treatment for chorea associated with HD. The dosing range that proved effective was 12.5-100 mg/d. Its manufacturer has been granted fast track and orphan drug status by the FDA. It is the first treatment for chorea in patients with HD in the United States. Always weigh potential adverse effects against the benefits of each drug.
Patients who have HD and predominant features of bradykinesia and rigidity may benefit from treatment with levodopa or dopamine agonists.
Depression in patients with HD is treatable and should be recognized promptly. Selective serotonin reuptake inhibitors (SSRIs) should be considered as first-line therapy. Other antidepressants, including bupropion, venlafaxine, nefazodone, and tricyclic antidepressants, also can be used. Electroconvulsive therapy (ECT) can be used in patients with refractory depression.
Antipsychotic medications may be necessary in patients with hallucinations, delusions, or schizophrenia-like syndromes. Newer agents, such as quetiapine, clozapine, olanzapine, and risperidone, are preferred to older agents because of the lower incidence of extrapyramidal side effects and the decreased risk for tardive syndromes.
Irritability may be treated with antidepressants, particularly the SSRIs; mood stabilizers, such as valproic acid or carbamazepine; and, if needed, atypical neuroleptics.
Other less frequent aspects of HD that may require pharmacologic treatment are mania, obsessive-compulsive disorder, anxiety, sexual disorders, myoclonus, tics, dystonia, and epilepsy.
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