Deep brain stimulation for dystonia

Abstract

The few reported controlled studies show that bilateral stimulation of the globus pallidus interna (GPi) is a safe and effective long-term treatment for hyperkinetic disorders. However, the recently published data on deep brain stimulation (DBS) applied to different targets or patients (especially those with secondary dystonia) are mainly uncontrolled case reports, precluding a clear determination of its efficacy, and providing little guidance as to the choice of a "good" target in a "good" patient. This chapter reviews the literature on DBS in primary dystonia, paying particular attention to the risk:benefit ratio in focal and segmental dystonias (cervical dystonia, cranial dystonia) and to the predictive factors for a good outcome. The chapter also highlights recent data on the marked benefits of the technique in myoclonus dystonia (in which pallidal, as opposed to thalamic, stimulation is more effective) and in tardive dystonia-dyskinesia. Although, the decision to treat appears relatively straightforward in patients with primary dystonia, myoclonus-dystonia, and tardive dystonia who have a normal findings on magnetic resonance imaging and normal cognitive function, there are still no reliable tools to help predict the timescale of postoperative benefit. This chapter provides a comprehensive analysis of the use of the treatment in various types of secondary dystonia, with little to moderate benefit in most cases, based on single cases or small series. Beyond the reduction in the severity of dystonia, the global motor and functional outcome is difficult to determine owing to the paucity of adequate evaluation tools. Because of the large interpatient variability, different targets may be effective depending on the symptoms in each individual.

Keywords: GPi; cerebral palsy; deep brain stimulation; dystonia; dystonia–choreoathetosis; myoclonus dystonia; pallidum; secondary dystonia; subthalamic nucleus (STN); tardive dyskinesia; thalamus.