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Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: case report and review of the literature

  • Neurology and Preclinical Neurological Studies - Original Article
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Abstract

Deep brain stimulation (DBS) represents an established treatment option in a growing number of movement disorders. Recent case reports suggest beneficial effect of globus pallidus internus (GPi)-DBS in selected patients suffering from Huntington’s disease with marked disabling chorea. We present a 41-year-old man with genetically confirmed HD following quadruple GPi- and subthalamic nucleus (STN)-DBS. Motor function was assessed by Abnormal Involuntary Movement Scale (AIMS) and by Unified Huntington Disease Rating Scale (UHDRS) presurgery and postsurgery for up to 4 years. Furthermore, cognitive, neuropsychiatric state and quality of life (QoL) including life satisfaction (QLS) were annually evaluated. Chorea assessed by AIMS and UHDRS subscores improved by 52 and 55 %, 45 and 60 %, 35 and 45 % and 55–66 % at 1–4 years, respectively, compared to presurgical state following GPi–STN-DBS. During these time periods bradykinesia did not increase following separate STN- and combined GPi–STN-DBS compared to presurgical state. Mood, QoL and QLS were ameliorated. However, dysexecutive symptoms increased at 4 years postsurgery. The present case report suggests that bilateral GPi- and STN-DBS may represent a new treatment avenue in selected HD patients. Clinically, GPi-DBS attenuated chorea and was associated with a larger effect–adverse effect window compared to STN-DBS. However, GPi-DBS-induced bradykinesia may emerge as one main limitation of GPi-DBS in HD. Thus, quadruple GPi–STN-DBS may be indicated, if separate GPi-DBS does not result in sufficient control of motor symptoms. Future controlled studies need to confirm if the present anecdotal observation of additive beneficial effects of GPi- and STN-DBS in a HD patient with severe generalized chorea and relatively intact cognitive and affective functions indeed represents a new therapeutic option.

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Acknowledgments

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors except for partial support of AKu’s research by DFG KFO 247 DBS Berlin, KU 810-1. We thank Dr. Klaus Kopitzki, University of Magdeburg, for help calculating the MRI-based stereotactic coordinates.

Conflict of interest

DG, AKi, GHS, BM received speaker’s honoraria from Medtronic. AAK received honoraries from Medtronic, St. Jude Medical, Boston Scientific, Novartis, Bayer AG and support for travel to conferences by Ipsen Pharma. JH received speaker’s honoraria from Merz Pharmaceuticals. AKu received honoraria for speaking from Allergan, Boehringer Ingelheim, Ipsen Pharma, Lundbeck, Medtronic, Merck, Merz Pharmaceuticals, Orion, St. Jude, UCB and grant support from German Research Council & German Ministry of Education and Research. TS, EL, UAK reported no conflict of interest.

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Correspondence to D. Gruber.

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Part I: Presurgical clip with the patient (HD) in resting and sitting position; gait and postural stability are separately assessed. Part II-IV: One–3 years postsurgery following GPi–STN-DBS in resting and sitting position; gait and postural stability are separately assessed. Note that bradykinesia increased in comparison to preoperative state. Part V: 4 years postsurgery a) GPi–STN-DBS OFF in resting, sitting and lying position, HD patient is unable to stand; b) GPi–STN-DBS ON in resting and sitting position; gait and postural stability are separately assessed. Note that bradykinesia increased in comparison to preoperative state. (M4V 16915 kb)

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Gruber, D., Kuhn, A.A., Schoenecker, T. et al. Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: case report and review of the literature. J Neural Transm 121, 1303–1312 (2014). https://doi.org/10.1007/s00702-014-1201-7

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  • DOI: https://doi.org/10.1007/s00702-014-1201-7

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