Treatment of huntington disease

Many pharmacological agents have been utilized in the treatment of Huntington disease (HD). Several excellent reviews about the treatment of HD are available. Formal treatment guidelines are however lacking. This is mainly the result of limited evidence available in the literature. Further, available treatment studies are frequently hard to compare due to variable outcomes/instruments used, differences in the study population, and confounding effects of complex medication regimens. Generally speaking, the treatment paradigm for an HD patient will depend on the constellation of 3 main clinical domains affected in HD: motor, behavioral/psychiatric, and cognitive. Symptoms within each of these domains remain dynamic throughout the course of HD. It is therefore necessary to monitor patients clinically and adjust drugs accordingly as the disease progresses. The most commonly used chorea drugs are antipsychotics and tetrabenazine (TBZ). Antipsychotic drugs are preferred in patients with coexistent psychiatric/behavioral comorbidities as well as in the presence of depression. Amantadine may be considered in the treatment of chorea, but data supporting its effectiveness remain conflicting. Selective serotonin reuptake inhibitors (SSRIs) are the treatment of choice for irritability and obsessive-compulsive behaviors associated with HD. Antipsychotic agents and antiepileptic mood stabilizers may be used as add-on therapies. There is very limited evidence for the treatment of cognitive impairment associated with HD. Each drug used in treatment of HD has a potential for causing significant side effects. It is, therefore, critical to assess the risk-benefit ratio on an individual basis, and carefully monitor patients throughout the course of treatment. Non-pharmacological and surgical treatment strategies for HD have not been systematically explored. Despite the lack of evidence, behavioral interventions, as well as physical, occupational, and speech therapies may provide additional benefits to a wide spectrum of disabilities associated with HD.