Pallidal Deep Brain Stimulation in the Treatment of Huntington’s Chorea

Abstract

Background: Stereotactic lesions have been occasionally performed in Huntington's chorea since the dawn of functional stereotactic surgery, however, with modest results. Despite the success of deep brain stimulation (DBS) in surgical treatment of Parkinson's disease and dystonia, the interest of testing DBS in Huntington's Disease (HD) has been limited. So far, promising results of pallidal DBS in 7 patients with HD have been reported in the literature. Objectives: To present the results of pallidal DBS in a patient with HD. Methods: A 59 year old woman with HD since 12 years and severe motor symptoms was implanted bilaterally in the Globus pallidus internus. The patient was evaluated at 12 months after surgery. Results: The effect of DBS was deemed satisfactory concerning the patient's choreo/dystonic symptoms. The improvement according to the unified Huntington's disease rating scale was modest, with a score reduction from 92 before surgery to 81 at one year. Conclusions: The results of pallidal DBS were deemed satisfactory in the patient presented here, confirming previous reports of the role of DBS in HD. However, further randomized studies are needed to ascertain the role of DBS in HD, especially considering the progressive nature of the disease.