K Amande M Pauls1, Joachim K Krauss2, Constanze E Kämpfer3, Andrea A Kühn4, Christoph Schrader5, Martin Südmeyer6, Niels Allert7, Rainer Benecke8, Christian Blahak9, Jana K Boller3, Gereon R Fink10, Wolfgang Fogel11, Thomas Liebig12, Faycal El Majdoub13, Philipp Mahlknecht14, Josef Kessler3, Joerg Mueller15, Juergen Voges16, Matthias Wittstock8, Alexander Wolters8, Mohammad Maarouf13, Elena Moro17, Jens Volkmann18, Kailash P Bhatia19, Lars Timmermann3. 1. Department of Neurology, Uniklinik Köln, Cologne, Germany. Electronic address: amande.pauls@uk-koeln.de. 2. Department of Neurosurgery, Medizinische Hochschule Hannover, Hannover, Germany. 3. Department of Neurology, Uniklinik Köln, Cologne, Germany. 4. Department of Neurology, Charite Universitätsmedizin Berlin, Berlin, Germany. 5. Department of Neurology, Medizinische Hochschule Hannover, Hannover, Germany. 6. Department of Neurology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany. 7. Neurological Rehabilitation Center Godeshöhe, Bonn, Germany. 8. Department of Neurology, Universitätsmedizin Rostock, Rostock, Germany. 9. Department of Neurology, Universitätsmedizin Mannheim, Mannheim, Germany. 10. Department of Neurology, Uniklinik Köln, Cologne, Germany; Institute of Neuroscience and Medicine (INM-3), Forschungszentrum Jülich, Jülich, Germany. 11. Department of Neurology, Deutsche Klinik für Diagnostik, Wiesbaden, Germany. 12. Department of Neuroradiology, Uniklinik Köln, Cologne, Germany. 13. Department of Stereotaxic and Functional Neurosurgery, Uniklinik Köln, Cologne, Germany. 14. Department of Neurology, Medical University Innsbruck, Innsbruck, Austria. 15. Department of Neurology, Medical University Innsbruck, Innsbruck, Austria; Department of Neurology, Vivantes Klinikum Spandau, Berlin, Germany. 16. Department for Stereotactic Neurosurgery, Universitätsklinikum Magdeburg, Magdeburg, Germany; Leibniz Institute of Neurobiology, Magdeburg, Germany. 17. Division of Neurology, University Hospital Center of Grenoble, Grenoble, France. 18. Department of Neurology, Universitätsklinikum Würzburg, Würzburg, Germany. 19. Institute of Neurology, University College London, London, UK.
Abstract
INTRODUCTION: Pallidal deep brain stimulation (GPi-DBS) is an effective therapy for isolated dystonia, but 10-20% of patients show improvement below 25-30%. We here investigated causes of insufficient response to GPi-DBS in isolated dystonia in a cross-sectional study. METHODS: Patients with isolated dystonia at time of surgery, and <30% improvement on the Burke-Fahn-Marsden dystonia-rating-scale (BFMDRS) after ≥6 months of continuous GPi-DBS were videotaped ON and OFF stimulation, and history, preoperative videos, brain MRI, medical records, stimulation settings, stimulation system integrity, lead location, and genetic information were obtained and reviewed by an expert panel. RESULTS: 22 patients from 11 centres were included (8 men, 14 women; 9 generalized, 9 segmental, 3 focal, 1 bibrachial dystonia; mean (range): age 48.7 (25-72) years, disease duration 22.0 (2-40) years, DBS duration 45.5 (6-131) months). Mean BFMDRS-score was 31.7 (4-93) preoperatively and 32.3 (5-101) postoperatively. Half of the patients (n = 11) had poor lead positioning alone or in combination with other problems (combined with: other disease n = 6, functional dystonia n = 1, other problems n = 2). Other problems were disease other than isolated inherited or idiopathic dystonia (n = 5), fixed deformities (n = 2), functional dystonia (n = 3), and other causes (n = 1). Excluding patients with poor lead location from further analysis, non-isolated dystonia accounted for 45.5%, functional dystonia for 27.3%, and fixed deformities for 18.2%. In patients with true isolated dystonia, lead location was the most frequent problem. CONCLUSION: After exclusion of lead placement and stimulation programming issues, non-isolated dystonia, functional dystonia and fixed deformities account for the majority of GPi-DBS failures in dystonia.
INTRODUCTION: Pallidal deep brain stimulation (GPi-DBS) is an effective therapy for isolated dystonia, but 10-20% of patients show improvement below 25-30%. We here investigated causes of insufficient response to GPi-DBS in isolated dystonia in a cross-sectional study. METHODS:Patients with isolated dystonia at time of surgery, and <30% improvement on the Burke-Fahn-Marsden dystonia-rating-scale (BFMDRS) after ≥6 months of continuous GPi-DBS were videotaped ON and OFF stimulation, and history, preoperative videos, brain MRI, medical records, stimulation settings, stimulation system integrity, lead location, and genetic information were obtained and reviewed by an expert panel. RESULTS: 22 patients from 11 centres were included (8 men, 14 women; 9 generalized, 9 segmental, 3 focal, 1 bibrachial dystonia; mean (range): age 48.7 (25-72) years, disease duration 22.0 (2-40) years, DBS duration 45.5 (6-131) months). Mean BFMDRS-score was 31.7 (4-93) preoperatively and 32.3 (5-101) postoperatively. Half of the patients (n = 11) had poor lead positioning alone or in combination with other problems (combined with: other disease n = 6, functional dystonia n = 1, other problems n = 2). Other problems were disease other than isolated inherited or idiopathic dystonia (n = 5), fixed deformities (n = 2), functional dystonia (n = 3), and other causes (n = 1). Excluding patients with poor lead location from further analysis, non-isolated dystonia accounted for 45.5%, functional dystonia for 27.3%, and fixed deformities for 18.2%. In patients with true isolated dystonia, lead location was the most frequent problem. CONCLUSION: After exclusion of lead placement and stimulation programming issues, non-isolated dystonia, functional dystonia and fixed deformities account for the majority of GPi-DBS failures in dystonia.
Authors: Takashi Tsuboi; Joshua K Wong; Leonardo Almeida; Christopher W Hess; Aparna Wagle Shukla; Kelly D Foote; Michael S Okun; Adolfo Ramirez-Zamora Journal: J Neurol Date: 2020-01-14 Impact factor: 4.849
Authors: L Okromelidze; T Tsuboi; R S Eisinger; M R Burns; M Charbel; M Rana; S S Grewal; C-Q Lu; L Almeida; K D Foote; M S Okun; E H Middlebrooks Journal: AJNR Am J Neuroradiol Date: 2020-02-13 Impact factor: 3.825
Authors: Laura Cif; Diane Demailly; Jean-Pierre Lin; Katy E Barwick; Mario Sa; Lucia Abela; Sony Malhotra; Wui K Chong; Dora Steel; Alba Sanchis-Juan; Adeline Ngoh; Natalie Trump; Esther Meyer; Xavier Vasques; Julia Rankin; Meredith W Allain; Carolyn D Applegate; Sanaz Attaripour Isfahani; Julien Baleine; Bettina Balint; Jennifer A Bassetti; Emma L Baple; Kailash P Bhatia; Catherine Blanchet; Lydie Burglen; Gilles Cambonie; Emilie Chan Seng; Sandra Chantot Bastaraud; Fabienne Cyprien; Christine Coubes; Vincent d'Hardemare; Asif Doja; Nathalie Dorison; Diane Doummar; Marisela E Dy-Hollins; Ellyn Farrelly; David R Fitzpatrick; Conor Fearon; Elizabeth L Fieg; Brent L Fogel; Eva B Forman; Rachel G Fox; William A Gahl; Serena Galosi; Victoria Gonzalez; Tracey D Graves; Allison Gregory; Mark Hallett; Harutomo Hasegawa; Susan J Hayflick; Ada Hamosh; Marie Hully; Sandra Jansen; Suh Young Jeong; Joel B Krier; Sidney Krystal; Kishore R Kumar; Chloé Laurencin; Hane Lee; Gaetan Lesca; Laurence Lion François; Timothy Lynch; Neil Mahant; Julian A Martinez-Agosto; Christophe Milesi; Kelly A Mills; Michel Mondain; Hugo Morales-Briceno; John R Ostergaard; Swasti Pal; Juan C Pallais; Frédérique Pavillard; Pierre-Francois Perrigault; Andrea K Petersen; Gustavo Polo; Gaetan Poulen; Tuula Rinne; Thomas Roujeau; Caleb Rogers; Agathe Roubertie; Michelle Sahagian; Elise Schaefer; Laila Selim; Richard Selway; Nutan Sharma; Rebecca Signer; Ariane G Soldatos; David A Stevenson; Fiona Stewart; Michel Tchan; Ishwar C Verma; Bert B A de Vries; Jenny L Wilson; Derek A Wong; Raghda Zaitoun; Dolly Zhen; Anna Znaczko; Russell C Dale; Claudio M de Gusmão; Jennifer Friedman; Victor S C Fung; Mary D King; Shekeeb S Mohammad; Luis Rohena; Jeff L Waugh; Camilo Toro; F Lucy Raymond; Maya Topf; Philippe Coubes; Kathleen M Gorman; Manju A Kurian Journal: Brain Date: 2020-12-05 Impact factor: 13.501
Authors: Simone Zittel; Ute Hidding; Maria Trumpfheller; Vanessa Lupici Baltzer; Alessandro Gulberti; Miriam Schaper; Maxine Biermann; Carsten Buhmann; Andreas K Engel; Christian Gerloff; Manfred Westphal; Jana Stadler; Johannes A Köppen; Monika Pötter-Nerger; Christian K E Moll; Wolfgang Hamel Journal: J Neurol Date: 2020-02-17 Impact factor: 4.849