Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease

S. Zittel; V. Tadic; C. K.E. Moll; T. Bäumer; A. Fellbrich; A. Gulberti; D. Rasche; N. Brüggemann; V. Tronnier; A. Münchau

doi:10.1016/j.parkreldis.2018.02.030

Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease

S. Zittel^*, V. Tadic, C. K.E. Moll, T. Bäumer, A. Fellbrich, A. Gulberti, D. Rasche, N. Brüggemann, V. Tronnier, A. Münchau

^*Corresponding author for this work

University Hospital Hamburg-Eppendorf

21 Citations (Scopus)

Abstract

Introduction: Pharmacological treatment of chorea in Huntington's disease (HD) is often limited by poor efficacy or side effects. Pallidal deep brain stimulation (DBS) has been considered in these patients but experience is so far limited. Methods: We prospectively evaluated the effects of bilateral DBS of the Globus pallidus internus (GPi) over one year in six severely affected HD patients with treatment refractory chorea in an advanced stage of the disease. Primary endpoint of the study was improvement in chorea. Additionally, we evaluated the effects of GPi DBS on the motor part of the Unified Huntington's Disease Rating Scale (UHDRS), bradykinesia, dystonia, functional impairment, psychiatric and cognitive symptoms. Side effects were systematically assessed. Results: The chorea subscore was significantly reduced postoperatively (−47% six months, −40% twelve months postoperatively). The UHDRS total motor score was significantly reduced at six months postoperatively (- 17%) but the effect was not sustained twelve months after the operation (- 5%). Pallidal DBS did not improve other motor symptoms or functional impairment. There was no effect on psychiatric symptoms or cognition. A number of side effects were noted, especially spasticity in three of the patients. Conclusions: Pallidal DBS is a treatment option for HD patients with severe pharmacologically refractory chorea. Further studies are needed to define optimal candidates for this procedure.

Original language	English
Journal	Parkinsonism and Related Disorders
Volume	51
Pages (from-to)	96-100
Number of pages	5
ISSN	1353-8020
DOIs	https://doi.org/10.1016/j.parkreldis.2018.02.030
Publication status	Published - 01.06.2018

Funding

Simone Zittel has no financial disclosures. Vera Tadic has no financial disclosures. Christian Moll served as medico-scientific consultant to Abbott/St. Jude, BrainLab and AlphaOmega Inc. Tobias Bäumer receives support from the Possehl-Stiftung, he received honoraria from Merz Pharmaceuticals, Allergan and Ipsen Pharma. Anja Fellbrich has no financial disclosures. Alessandro Gulberti has no financial disclosures. Dirk Rasche has no financial disclosures. Norbert Brüggemann was funded by the Collaborative Center for X-linked Dystonia-Parkinsonism, received speaker's honoraria from the German Neurological Society, and received travel support from Merz and UCB. Volker Tronnier has no financial disclosures. Alexander Münchau received commercial research support from Pharm Allergan, Ipsen, Merz Pharmaceuticals, Actelion. He received honoraria for lectures from Pharm Allergan, Ipsen, Merz Pharmaceuticals, Actelion, GlaxoSmithKline, Desitin and Teva. He is supported by the Possehl-Stiftung (Lübeck, Germany), Margot und Jürgen Wessel Stiftung (Lübeck, Germany), Tourette Syndrome Association (Germany), Interessenverband Tourette Syndrom (Germany) and CHDI. He receives academic research support as follows: Multicentre Tics in Children Studies (EMTICS) as part of the FP 7 program ( HEALTH.2011.2.2.1-3 ); Deutsche Forschungsgemeinschaft : projects MU 1692/3-1, 4-1 and SFB 936 (project C5) ; Bundesministerium für Bildung und Forschung : DysTract consortium ; Other: Innovationsausschuss of the Gemeinsamer Bundesausschuss: Translate NAMSE (structural support for the Lübeck Center for Rare Diseases). Alexander Münchau received an unrestricted grant of St. Jude Medical. We are grateful to the patients and their families for their contribution. Support by the Deutsche Forschungsgemeinschaft (DFG, SFB, project C5) is gratefully acknowledged. Appendix A

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Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)

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10.1016/j.parkreldis.2018.02.030

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title = "Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease",

abstract = "Introduction: Pharmacological treatment of chorea in Huntington's disease (HD) is often limited by poor efficacy or side effects. Pallidal deep brain stimulation (DBS) has been considered in these patients but experience is so far limited. Methods: We prospectively evaluated the effects of bilateral DBS of the Globus pallidus internus (GPi) over one year in six severely affected HD patients with treatment refractory chorea in an advanced stage of the disease. Primary endpoint of the study was improvement in chorea. Additionally, we evaluated the effects of GPi DBS on the motor part of the Unified Huntington's Disease Rating Scale (UHDRS), bradykinesia, dystonia, functional impairment, psychiatric and cognitive symptoms. Side effects were systematically assessed. Results: The chorea subscore was significantly reduced postoperatively (−47\% six months, −40\% twelve months postoperatively). The UHDRS total motor score was significantly reduced at six months postoperatively (- 17\%) but the effect was not sustained twelve months after the operation (- 5\%). Pallidal DBS did not improve other motor symptoms or functional impairment. There was no effect on psychiatric symptoms or cognition. A number of side effects were noted, especially spasticity in three of the patients. Conclusions: Pallidal DBS is a treatment option for HD patients with severe pharmacologically refractory chorea. Further studies are needed to define optimal candidates for this procedure.",

author = "S. Zittel and V. Tadic and Moll, \{C. K.E.\} and T. B{\"a}umer and A. Fellbrich and A. Gulberti and D. Rasche and N. Br{\"u}ggemann and V. Tronnier and A. M{\"u}nchau",

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T1 - Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease

AU - Zittel, S.

AU - Tadic, V.

AU - Moll, C. K.E.

AU - Bäumer, T.

AU - Fellbrich, A.

AU - Gulberti, A.

AU - Rasche, D.

AU - Brüggemann, N.

AU - Tronnier, V.

AU - Münchau, A.

PY - 2018/6/1

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N2 - Introduction: Pharmacological treatment of chorea in Huntington's disease (HD) is often limited by poor efficacy or side effects. Pallidal deep brain stimulation (DBS) has been considered in these patients but experience is so far limited. Methods: We prospectively evaluated the effects of bilateral DBS of the Globus pallidus internus (GPi) over one year in six severely affected HD patients with treatment refractory chorea in an advanced stage of the disease. Primary endpoint of the study was improvement in chorea. Additionally, we evaluated the effects of GPi DBS on the motor part of the Unified Huntington's Disease Rating Scale (UHDRS), bradykinesia, dystonia, functional impairment, psychiatric and cognitive symptoms. Side effects were systematically assessed. Results: The chorea subscore was significantly reduced postoperatively (−47% six months, −40% twelve months postoperatively). The UHDRS total motor score was significantly reduced at six months postoperatively (- 17%) but the effect was not sustained twelve months after the operation (- 5%). Pallidal DBS did not improve other motor symptoms or functional impairment. There was no effect on psychiatric symptoms or cognition. A number of side effects were noted, especially spasticity in three of the patients. Conclusions: Pallidal DBS is a treatment option for HD patients with severe pharmacologically refractory chorea. Further studies are needed to define optimal candidates for this procedure.

AB - Introduction: Pharmacological treatment of chorea in Huntington's disease (HD) is often limited by poor efficacy or side effects. Pallidal deep brain stimulation (DBS) has been considered in these patients but experience is so far limited. Methods: We prospectively evaluated the effects of bilateral DBS of the Globus pallidus internus (GPi) over one year in six severely affected HD patients with treatment refractory chorea in an advanced stage of the disease. Primary endpoint of the study was improvement in chorea. Additionally, we evaluated the effects of GPi DBS on the motor part of the Unified Huntington's Disease Rating Scale (UHDRS), bradykinesia, dystonia, functional impairment, psychiatric and cognitive symptoms. Side effects were systematically assessed. Results: The chorea subscore was significantly reduced postoperatively (−47% six months, −40% twelve months postoperatively). The UHDRS total motor score was significantly reduced at six months postoperatively (- 17%) but the effect was not sustained twelve months after the operation (- 5%). Pallidal DBS did not improve other motor symptoms or functional impairment. There was no effect on psychiatric symptoms or cognition. A number of side effects were noted, especially spasticity in three of the patients. Conclusions: Pallidal DBS is a treatment option for HD patients with severe pharmacologically refractory chorea. Further studies are needed to define optimal candidates for this procedure.

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Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease

Abstract

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