Volume 33, Issue 7 p. 1168-1173
Brief Report

Deep brain stimulation treated dystonia-trajectory via status dystonicus

Elodie Nerrant MD

Elodie Nerrant MD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Victoria Gonzalez MD, PhD

Victoria Gonzalez MD, PhD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Christophe Milesi MD, PhD

Christophe Milesi MD, PhD

Université Montpellier, 34000 Montpellier, France

Département Pédiatrie néonatale et réanimations; Centre Hospitalier Régional Montpellier, Montpellier, France

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Xavier Vasques PhD

Xavier Vasques PhD

Laboratoire de Recherche en Neurosciences Cliniques (LRENC), Montpellier, France

IBM Systems, IBM, Montpellier, France

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Diane Ruge MD, PhD

Diane Ruge MD, PhD

Department of Psychology and Neurosciences. Leibniz Research Centre for Working Environment and Human Factors, Technical University Dortmund, Dortmund, Germany

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Thomas Roujeau MD

Thomas Roujeau MD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Isabel De Antonio Rubio

Isabel De Antonio Rubio

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Fabienne Cyprien MD, PhD

Fabienne Cyprien MD, PhD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Inserm U1061, Hôpital La Colombière, Montpellier, France

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Emilie Chan Seng MD

Emilie Chan Seng MD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Université Montpellier, 34000 Montpellier, France

INSERM U 1051, Institut des Neurosciences Montpellier, Montpellier, France

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Diane Demailly

Diane Demailly

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Agathe Roubertie MD, PhD

Agathe Roubertie MD, PhD

Université Montpellier, 34000 Montpellier, France

Département de Neuropédiatrie, Centre Hospitalier Régional Montpellier, Montpellier, France

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Alain Boularan MD

Alain Boularan MD

Université Montpellier, 34000 Montpellier, France

Anesthésie-Réanimation Gui de Chauliac, Centre Hospitalier Régional Montpellier, Montpellier, France

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Fréderic Greco MD

Fréderic Greco MD

Université Montpellier, 34000 Montpellier, France

Anesthésie-Réanimation Gui de Chauliac, Centre Hospitalier Régional Montpellier, Montpellier, France

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Pierre-François Perrigault MD

Pierre-François Perrigault MD

Université Montpellier, 34000 Montpellier, France

Anesthésie-Réanimation Gui de Chauliac, Centre Hospitalier Régional Montpellier, Montpellier, France

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Gilles Cambonie MD, PhD

Gilles Cambonie MD, PhD

Université Montpellier, 34000 Montpellier, France

Département Pédiatrie néonatale et réanimations; Centre Hospitalier Régional Montpellier, Montpellier, France

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Philippe Coubes MD, PhD

Philippe Coubes MD, PhD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Université Montpellier, 34000 Montpellier, France

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Laura Cif MD, PhD

Corresponding Author

Laura Cif MD, PhD

Département de Neurochirurgie, Centre Hospitalier Régional Montpellier, France

Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), Montpellier, France

Université Montpellier, 34000 Montpellier, France

Correspondence to: Dr. Laura Cif, Département de Neurochirurgie, Centre Hospitalier Universitaire Montpellier, 80, Avenue Augustin Fliche, 34295 Montpellier Cedex 5, France; E-mail: [email protected]Search for more papers by this author
First published: 22 May 2018
Citations: 29

Philippe Coubes and Laura Cif are shared last authors.

Relevant conflicts of interest/financial disclosures: Nothing to report.

Full financial disclosures and author roles may be found in the online version of this article.

ABSTRACT

Background: Status dystonicus (SD) is a life-threatening condition.

Objective and Methods: In a dystonia cohort who developed status dystonicus, we analyzed demographics, background dystonia phenomenology and complexity, trajectory previous to-, via status dystonicus episodes, and evolution following them.

Results: Over 20 years, 40 of 328 dystonia patients who were receiving DBS developed 58 status dystonicus episodes. Dystonia was of pediatric onset (95%), frequently complex, and had additional cognitive and pyramidal impairment (62%) and MRI alterations (82.5%); 40% of episodes occured in adults. Mean disease duration preceding status dystonicus was 10.3 ± 8 years. Evolution time to status dystonicus varied from days to weeks; however, 37.5% of patients exhibited progressive worsening over years. Overall, DBS was efficient in resolving 90% of episodes.

Conclusion: Status dystonicus is potentially reversible and a result of heterogeneous conditions with nonuniform underlying physiology. Recognition of the complex phenomenology, morphological alterations, and distinct patterns of evolution, before and after status dystonicus, will help our understanding of these conditions. © 2018 International Parkinson and Movement Disorder Society