Deep Brain Stimulation in Huntington’s Disease: The Current Status

Abstract

Background: Huntington's disease (HD), a progressive neurodegenerative disorder, is characterized by choreic movements and psychiatric changes. Within 10 to 20 years after the onset of the disease most patients die of disease - related complications. Until now, no effective therapies have been found for this devastating disease. Deep brain stimula- tion (DBS) is a promising new symptomatic treatment for HD. In the last decade, theories on the potential benefit of DBS in HD have been made and different groups have explored this in case studies. Objectives: In this paper we provide an overview of the result obtained with DBS in patients with HD. Results: The studies performed thus far are case studies. Their results suggest that DBS of the globus pallidus internus is safe and well-tolerated, and reduces choreic movements, without substantial effects on psychiatric functions. Conclusions: DBS in HD should still be seen as experimental. The data published until now, demonstrate a therapeutic ef- fect on refractory motor symptoms and justify further investigation.