Deep Brain Stimulation in Adult Huntington Disease for Treatment of Axial Dystonia

Abstract

Huntington disease (HD) is a progressive neurodegenerative disorder characterized by chorea and cognitive and psychiatric disturbances. Dystonia is also a common symptom, and a recent case study points toward 50% of HD patients having truncal dystonia. Although tetrabenazine is approved for treatment of Huntington chorea, no medications have proved efficacious for the dystonia and bradykinesia that become more prominent as the disease progresses. Although several reports have outlined the effect of globus pallidus interna (GPi) deep brain stimulation (DBS) in HD patients with a choreic phenotype, less is known about the effect of DBS in HD patients with a predominately dystonic phenotype. When specific symptoms known to respond well to DBS, such as dystonia, interfere significantly with a patient’s quality of life, and optimal medical management has failed, DBS could be considered a viable option. This case reports demonstrates the utility of bilateral GPi DBS for dystonia in a patient with HD, so long as there is no neuropsychiatric impairment that would interfere with the patient’s comprehension of the potential risks of surgery. The patient and caregivers should have realistic expectations of outcomes and be willing to follow through with regular programming.