• Lars Wojtecki, Stefan J Groiss, Stefano Ferrea, Saskia Elben, Christian J Hartmann, Stephen B Dunnett, Anne Rosser, Carsten Saft, Martin Südmeyer, Christian Ohmann, Alfons Schnitzler, Jan Vesper, and Surgical Approaches Working Group of the European Huntington’s Disease Network (EHDN).
• Department of Neurology, Centre for Movement Disorders and Neuromodulation, Medical Faculty, Heinrich-Heine-University Düsseldorf , Düsseldorf , Germany ; Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich- Heine-University Düsseldorf , Düsseldorf , Germany.
• Front Neurol. 2015 Jan 1;6:177.

BackgroundMovement disorders in Huntington's disease are often medically refractive. The aim of the trial was assessment of procedure safety of deep brain stimulation, equality of internal- and external-pallidal stimulation and efficacy followed-up for 6 months in a prospective pilot trial.MethodsIn a controlled double-blind phase six patients (four chorea-dominant, two Westphal-variant) with predominant movement disorder were randomly assigned to either the sequence of 6-week internal- or 6-week external-pallidal stimulation, or vice versa, followed by further 3 months chronic pallidal stimulation at the target with best effect-side-effect ratio. Primary endpoints were changes in the Unified Huntington's Disease Rating Scale motor-score, chorea subscore, and total motor-score 4 (blinded-video ratings), comparing internal- versus external-pallidal stimulation, and 6 months versus baseline. Secondary endpoints assessed scores on dystonia, hypokinesia, cognition, mood, functionality/disability, and quality-of-life.ResultsIntention-to-treat analysis of all patients (n = 3 in each treatment sequence): Both targets were equal in terms of efficacy. Chorea subscores decreased significantly over 6 months (-5.3 (60.2%), p = 0.037). Effects on dystonia were not significant over the group due to it consisting of three responders (>50% improvement) and three non-responders. Westphal patients did not improve. Cognition was stable. Mood and some functionality/disability and quality-of-life scores improved significantly. Eight adverse events and two additional serious adverse events - mostly internal-pallidal stimulation-related - resolved without sequalae. No procedure-related complications occurred.ConclusionPallidal deep brain stimulation was demonstrated to be a safe treatment option for the reduction of chorea in Huntington's disease. Their effects on chorea and dystonia and on quality-of-life should be examined in larger controlled trials.

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