EP 34. Effects of deep brain stimulation of the globus pallidus interna on Huntington’s disease at 1 year

Abstract

Introduction Huntington’s disease (HD) is an autosomal dominant and progressive neurodegenerative disorder. The disease is caused by a strongly increased number of trinucleotide CAG repeats in the Huntingtin gene and characterized by a progressive loss of striatal neurons. Phenotypically, patients present with severe motor symptoms, such as chorea, dystonia, and bradykinesia. Deep brain stimulation (DBS) of the globus pallidus interna (GPi) has been proposed to treat abnormal movements in HD. Here, we report the clinical outcomes of DBS of the GPi in three HD patients followed for 1 year respectively. Objectives Our main objective was to assess the efficacy and safety of DBS of the GPi in alleviating motor symptoms, particularly chorea. Materials and methods Patients with major functional disability due to pharmacoresistant chorea, preserved or mildly altered cognitive function and no severe psychiatric disorder were selected. Due to the severity of chorea, DBS of the GPi were performed under general anesthesia. Microelectrode recording were also used in the preoperative period in all patients. They were followed for 1 year respectively. Outcome measure were scored by the change of the chorea and total motor scores of the Unified Huntington’s Disease Rating Scale (UHDRS) between pre- and last postoperative assessments. Results Chorea improved in all patients (13%, 86% and 29%) and total motor score decreased in 1 patient (%24) postoperatively. 1 patient had a transient dysarthria because of frontal cerebral contusion after operation. Conclusion Based on these results and previous published reports, DBS of the GPi may be considered as an alternative treatment in non-demented HD patients with severe chorea.