Bilateral Deep-Brain Stimulation of the Globus Pallidus in Primary Generalized Dystonia
Marie Vidailhet, M.D., Ph.D., Laurent Vercueil, M.D., Jean-Luc Houeto, M.D., Ph.D., Pierre Krystkowiak, M.D., Alim-Louis Benabid, M.D., Ph.D., Philippe Cornu, M.D., Christelle Lagrange, Ph.D., Sophie Tézenas du Montcel, M.D., Ph.D., Didier Dormont, M.D., Ph.D., Sylvie Grand, M.D., Ph.D., Serge Blond, M.D., Olivier Detante, M.D., Bernard Pillon, Ph.D., Claire Ardouin, Ph.D., Yves Agid, M.D., Ph.D., Alain Destée, M.D., Pierre Pollak, M.D., Ph.D., for the French Stimulation du Pallidum Interne dans la Dystonie (SPIDY) Study Group
Background Severe forms of dystonia respond poorly to medicaltreatment. Deep-brain stimulation is a reversible neurosurgicalprocedure that has been used for the treatment of dystonia,but assessment of its efficacy has been limited to open studies.
Methods We performed a prospective, controlled, multicenterstudy assessing the efficacy and safety of bilateral pallidalstimulation in 22 patients with primary generalized dystonia.The severity of dystonia was evaluated before surgery and 3,6, and 12 months postoperatively during neurostimulation, withthe use of the movement and disability subscores of the Burke–Fahn–MarsdenDystonia Scale (range, 0 to 120 and 0 to 30, respectively, withhigher scores indicating greater impairment). Movement scoreswere assessed by a review of videotaped sessions performed byan observer who was unaware of treatment status. At three months,patients underwent a double-blind evaluation in the presenceand absence of neurostimulation. We also assessed the patients'quality of life, cognition, and mood at baseline and 12 months.
Results The dystonia movement score improved from a mean (±SD)of 46.3±21.3 before surgery to 21.0±14.1 at 12months (P<0.001). The disability score improved from 11.6±5.5before surgery to 6.5±4.9 at 12 months (P<0.001).General health and physical functioning were significantly improvedat month 12; there were no significant changes in measures ofmood and cognition. At the three-month evaluation, dystoniamovement scores were significantly better with neurostimulationthan without neurostimulation (24.6±17.7 vs. 34.6±12.3,P<0.001). There were five adverse events (in three patients);all resolved without permanent sequelae.
Conclusions These findings support the efficacy and safety ofthe use of bilateral stimulation of the internal globus pallidusin selected patients with primary generalized dystonia.
Source Information
From the Department of Neurology, Saint Antoine Hospital, Paris (M.V.); INSERM Unité 289 (M.V.), the Department of Neurosurgery (P.C.), the Department of Neuroradiology and UPR 640, Centre National de la Recherche Scientifique Laboratoire de Neurosciences Cognitives et Imagerie Cerebrale (D.D.), INSERM E007 and the Department of Neurology (B.P.), and the Department of Neurology, Centre d'Investigation Clinique, and INSERM Unité 289 (Y.A.), Pitié–Salpêtrière Hospital, Paris; the Department of Biological and Clinical Neurosciences (L.V., A.-L.B., C.L., O.D., C.A., P.P.) and the Magnetic Resonance Imaging Unit, Department of Neuroradiology (S.G.), Grenoble University Hospital, and INSERM Unité 318, Joseph Fourier University (L.V., A.-L.B., P.P.), Grenoble; the Department of Neurology, University Hospital, Poitiers (J.-L.H.); the Neurology and Movement Disorders Unit (P.K., A.D.) and the Department of Neurosurgery (S.B.), Lille University Hospital, Lille; Equipe Associée 2683, University of Lille, Lille (P.K., A.D.); and the Department of Biostatistics, University Hospital Pitié–Salpêtrière and INSERM Unité 535, Paul Brousse Hospital, Villejuif (S.T.M.) — all in France.
Address reprint requests to Dr. Vidailhet at the Department of Neurology, Saint Antoine Hospital, 184 Faubourg Saint Antoine, 75571, Paris CEDEX 12, France, or at marie.vidailhet{at}sat.ap-hop-paris.fr.
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