Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: case report and review of the literature
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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.
KeywordsHuntington disease Deep brain stimulation Globus pallidus Subthalamic nucleus Chorea
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.
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)
- Armstrong MJ, Miyasaki JM (2012) Evidence-based guideline: pharmacologic treatment of chorea in Huntington disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology 79(6):597–603. doi: 10.1212/WNL.0b013e318263c443 PubMedCrossRefPubMedCentralGoogle Scholar
- Follett KA, Weaver FM, Stern M, Hur K, Harris CL, Luo P, Marks WJ Jr, Rothlind J, Sagher O, Moy C, Pahwa R, Burchiel K, Hogarth P, Lai EC, Duda JE, Holloway K, Samii A, Horn S, Bronstein JM, Stoner G, Starr PA, Simpson R, Baltuch G, De Salles A, Huang GD, Reda DJ (2010) Pallidal versus subthalamic deep-brain stimulation for Parkinson’s disease. N Engl J Med 362(22):2077–2091. doi: 10.1056/NEJMoa0907083 PubMedCrossRefGoogle Scholar
- Huys D, Bartsch C, Poppe P, Lenartz D, Huff W, Prutting J, Timmermann L, Klosterkotter J, Maarouf M, Rommel T, Hartmann A, Sturm V, Kuhn J (2013) Management and outcome of pallidal deep brain stimulation in severe Huntington’s disease. Fortschr Neurol Psychiatr 81(4):202–205. doi: 10.1055/s-0033-1335097 PubMedCrossRefGoogle Scholar
- Kupsch A, Benecke R, Muller J, Trottenberg T, Schneider GH, Poewe W, Eisner W, Wolters A, Muller JU, Deuschl G, Pinsker MO, Skogseid IM, Roeste GK, Vollmer-Haase J, Brentrup A, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Naumann M, Volkmann J (2006) Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355(19):1978–1990PubMedCrossRefGoogle Scholar
- Ligot N, Krystkowiak P, Simonin C, Goldman S, Peigneux P, Van Naemen J, Monclus M, Lacroix SF, Devos D, Dujardin K, Delmaire C, Bardinet E, Delval A, Delliaux M, Defebvre L, Yelnik J, Blond S, Destee A, De Tiege X (2011) External globus pallidus stimulation modulates brain connectivity in Huntington’s disease. J Cereb Blood Flow Metab 31(1):41–46. doi: 10.1038/jcbfm.2010.186 PubMedCrossRefPubMedCentralGoogle Scholar
- Odekerken VJ, van Laar T, Staal MJ, Mosch A, Hoffmann CF, Nijssen PC, Beute GN, van Vugt JP, Lenders MW, Contarino MF, Mink MS, Bour LJ, van den Munckhof P, Schmand BA, de Haan RJ, Schuurman PR, de Bie RM (2013) Subthalamic nucleus versus globus pallidus bilateral deep brain stimulation for advanced Parkinson’s disease (NSTAPS study): a randomised controlled trial. Lancet Neurol 12(1):37–44. doi: 10.1016/S1474-4422(12)70264-8 PubMedCrossRefGoogle Scholar
- Schjerling L, Hjermind LE, Jespersen B, Madsen FF, Brennum J, Jensen SR, Lokkegaard A, Karlsborg M (2013) A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia. J Neurosurg 119(6):1537–1545. doi: 10.3171/2013.8.JNS13844 PubMedCrossRefGoogle Scholar
- Schuepbach WM, Rau J, Knudsen K, Volkmann J, Krack P, Timmermann L, Halbig TD, Hesekamp H, Navarro SM, Meier N, Falk D, Mehdorn M, Paschen S, Maarouf M, Barbe MT, Fink GR, Kupsch A, Gruber D, Schneider GH, Seigneuret E, Kistner A, Chaynes P, Ory-Magne F, Brefel Courbon C, Vesper J, Schnitzler A, Wojtecki L, Houeto JL, Bataille B, Maltete D, Damier P, Raoul S, Sixel-Doering F, Hellwig D, Gharabaghi A, Kruger R, Pinsker MO, Amtage F, Regis JM, Witjas T, Thobois S, Mertens P, Kloss M, Hartmann A, Oertel WH, Post B, Speelman H, Agid Y, Schade-Brittinger C, Deuschl G (2013) Neurostimulation for Parkinson’s disease with early motor complications. N Engl J Med 368(7):610–622. doi: 10.1056/NEJMoa1205158 PubMedCrossRefGoogle Scholar
- Volkmann J, Wolters A, Kupsch A, Muller J, Kuhn AA, Schneider GH, Poewe W, Hering S, Eisner W, Muller JU, Deuschl G, Pinsker MO, Skogseid IM, Roeste GK, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Classen J, Naumann M, Benecke R (2012) Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 11(12):1029–1038. doi: 10.1016/S1474-4422(12)70257-0 PubMedCrossRefGoogle Scholar