Michael D Fox1, Ron L Alterman2. 1. Berenson-Allen Center for Noninvasive Brain Stimulation, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts2Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston. 2. Division of Neurosurgery, Department of Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
Abstract
IMPORTANCE: Dystonia is a heterogeneous neurologic disorder characterized by abnormal muscle contractions for which standard medical therapy is often inadequate. For such patients, therapeutic brain stimulation is becoming increasingly used. OBJECTIVES: To review the evidence and effect sizes for treating different types of dystonia with different types of brain stimulation and to discuss recent advances relevant to patient selection, surgical approach, programming, and mechanism of action. EVIDENCE REVIEW: PubMed was searched for publications on the clinical effect of brain stimulation in dystonia up through December 31, 2014. Recent meta-analyses, consensus statements, and evidence-based guidelines were incorporated. Emphasis was placed on deep brain stimulation (DBS) and randomized clinical trials; however, other stimulation modalities and trial designs were included. For each intervention the mean change in dystonia severity, number of patients studied, and evidence of efficacy based on American Academy of Neurology criteria were determined. FINDINGS: Strong (level B) evidence supports the use of DBS for the treatment of primary generalized or segmental dystonia, especially when due to mutation in the DYT1 gene, as well as for patients with cervical dystonia. Large effect sizes have also been reported for DBS treatment of tardive dystonia, writer's cramp, cranial dystonia, myoclonus dystonia, and off-state dystonia associated with Parkinson disease. Lesser benefit is generally seen in dystonia secondary to structural brain damage. Other brain stimulation techniques, including epidural cortical stimulation and noninvasive brain stimulation, have been investigated, but generally report smaller effect sizes in fewer patients. CONCLUSIONS AND RELEVANCE: Patients with dystonia that is not adequately controlled with standard medical therapy should be referred for consideration of DBS, especially patients with generalized, segmental, or cervical dystonia. Other less-invasive stimulation modalities require further research before being considered a therapeutic alternative.
IMPORTANCE: Dystonia is a heterogeneous neurologic disorder characterized by abnormal muscle contractions for which standard medical therapy is often inadequate. For such patients, therapeutic brain stimulation is becoming increasingly used. OBJECTIVES: To review the evidence and effect sizes for treating different types of dystonia with different types of brain stimulation and to discuss recent advances relevant to patient selection, surgical approach, programming, and mechanism of action. EVIDENCE REVIEW: PubMed was searched for publications on the clinical effect of brain stimulation in dystonia up through December 31, 2014. Recent meta-analyses, consensus statements, and evidence-based guidelines were incorporated. Emphasis was placed on deep brain stimulation (DBS) and randomized clinical trials; however, other stimulation modalities and trial designs were included. For each intervention the mean change in dystonia severity, number of patients studied, and evidence of efficacy based on American Academy of Neurology criteria were determined. FINDINGS: Strong (level B) evidence supports the use of DBS for the treatment of primary generalized or segmental dystonia, especially when due to mutation in the DYT1 gene, as well as for patients with cervical dystonia. Large effect sizes have also been reported for DBS treatment of tardive dystonia, writer's cramp, cranial dystonia, myoclonus dystonia, and off-state dystonia associated with Parkinson disease. Lesser benefit is generally seen in dystonia secondary to structural brain damage. Other brain stimulation techniques, including epidural cortical stimulation and noninvasive brain stimulation, have been investigated, but generally report smaller effect sizes in fewer patients. CONCLUSIONS AND RELEVANCE: Patients with dystonia that is not adequately controlled with standard medical therapy should be referred for consideration of DBS, especially patients with generalized, segmental, or cervical dystonia. Other less-invasive stimulation modalities require further research before being considered a therapeutic alternative.
Authors: Michael D Fox; Randy L Buckner; Hesheng Liu; M Mallar Chakravarty; Andres M Lozano; Alvaro Pascual-Leone Journal: Proc Natl Acad Sci U S A Date: 2014-09-29 Impact factor: 11.205
Authors: Michele Tagliati; Paul Krack; Jens Volkmann; Tipu Aziz; Joachim K Krauss; Andreas Kupsch; And Marie Vidailhet Journal: Mov Disord Date: 2011-06 Impact factor: 10.338
Authors: Andreas Kupsch; Reiner Benecke; Jörg Müller; Thomas Trottenberg; Gerd-Helge Schneider; Werner Poewe; Wilhelm Eisner; Alexander Wolters; Jan-Uwe Müller; Günther Deuschl; Marcus O Pinsker; Inger Marie Skogseid; Geir Ketil Roeste; Juliane Vollmer-Haase; Angela Brentrup; Martin Krause; Volker Tronnier; Alfons Schnitzler; Jürgen Voges; Guido Nikkhah; Jan Vesper; Markus Naumann; Jens Volkmann Journal: N Engl J Med Date: 2006-11-09 Impact factor: 91.245
Authors: Ewgenia Barow; Wolf-Julian Neumann; Christof Brücke; Julius Huebl; Andreas Horn; Peter Brown; Joachim K Krauss; Gerd-Helge Schneider; Andrea A Kühn Journal: Brain Date: 2014-09-10 Impact factor: 13.501
Authors: Paolo Calabresi; Antonio Pisani; John Rothwell; Veronica Ghiglieri; Josè A Obeso; Barbara Picconi Journal: Nat Neurosci Date: 2016-06-28 Impact factor: 24.884
Authors: H A Jinnah; Ron Alterman; Christine Klein; Joachim K Krauss; Elena Moro; Marie Vidailhet; Robert Raike Journal: J Neural Transm (Vienna) Date: 2017-02-03 Impact factor: 3.575
Authors: Corina N A M van den Heuvel; Marina A J Tijssen; Bart P C van de Warrenburg; Cathérine C S Delnooz Journal: Mov Disord Clin Pract Date: 2016-08-03
Authors: Meike E van der Heijden; Dominic J Kizek; Ross Perez; Elena K Ruff; Michelle E Ehrlich; Roy V Sillitoe Journal: J Physiol Date: 2021-01-09 Impact factor: 5.182