Deep Brain Stimulation for Craniocervical Dystonia (Meige Syndrome): A Report of Four Patients and a Literature-Based Analysis of Its Treatment Effects.

Originally published in the Neuromodulation journal 2016; 19: 818-23. Translation: Dr. Juan Carlos Andreani.

Authors

  • Xiu Wang Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Chao Zhang Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Yao Wang Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Chang Liu Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Baotian Zhao Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Jian-guo Zhang Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.
  • Wenhan Hu Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Instituto Neuroquirúrgico de Beijing, Beijing, China
  • Xiaoqiu Shao Beijing Key Laboratorio de Neuroestimulación, Beijing, China. Departamento de Neurología del Hospital Beijing Tiantan, Capital Medical University, Beijing, China.
  • Kai Zhang Departamento de Neurocirugía del Hospital Beijing Tiantan, Capital Medical University, Beijing, China. Beijing Key Laboratorio de Neuroestimulación, Beijing, China.

DOI:

https://doi.org/10.47924/neurotarget2017141

Keywords:

Craniocervical dystonia, deep brain stimulation, Meige Syndrome, Treatment outcome

Abstract

Objectives: The aim of this study was to report on four patients with craniocervical dystonia (CCD) treated with deep brain stimulation (DBS). In addition, we investigated the treatment eficacy and surgical outcome predictors by the review and analysis of previously published studies.
Methods: Four patients with CCD underwent DBS of the globus pallidus internus (Gpi) or subthalamic nucleus (STN). PubMed and MEDLINE searches were performed to obtain detailed information on patients who underwent DBS for CCD. The primary eficacy endpoint was the change in the Burke–Fahn–Marsden Dystonia Rating Scale (movement and disability scores, BFMDRS- M/D) after surgery.
Results: Seventyfive patients were included in the pooled analysis, including 69 patients with Gpi-DBS and 6 patients with STNDBS. The mean follow-up of time was 28.0 months after surgery. The mean BFMDRS-M score was 24.5 ± 11.2 preoperatively and 8.1 ± 5.7 postoperatively at the final follow-up evaluation, with a mean improvement of 66.9% (p < 0.001). The mean BFMDRS-D score was 8.1 ± 4.6 preoperatively and 3.6 ± 2.5 postoperatively, with a mean percentage improvement of 56.0% (p < 0.01). Positive correlations were found between each of the preoperative movement and disability scores and percentage of postoperative improvement (r = 0.247, p = 0.034; r = 0.331, p = 0.034, respectively).
Conclusion: GPi/STN-DBS is an efective treatment for patients with medically refractory CCD, including those with severe preoperative symptoms. The age at CCD onset and the disease duration do not predict improvement in movement scores.

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References

Tolosa E, Kulisevsky J, Fahn S. Meige syndrome: primary and secondary forms. Adv Neurol 1988;50:509-15.

Tolosa ES, Klawans HL. Meiges disease: a clinical form of facial convulsion, bilateral and medial. Arch Neurol 1979;36:635-37.

Ababneh OH, Cetinkaya A, Kulwin DR. Long-term efficacy and safety of botulinum toxin A injections to treat blepharospasm and hemifacial spasm. Clin Experiment Ophthalmol 2014;42:254-61.

Mauriello JA Jr, Dhillon S, Leone T, Pakeman B, Mostafavi R, Yepez MC. Treatment selections of 239 patients with blepharospasm and Meige syndrome over 11 years. Br J Ophthalmol 1996;80:1073-6.

Blomstedt P, Tisch S, Hariz MI. Pallidal deep brain stimulation in the treatment of Meige syndrome. Acta Neurol Scand 2008;118:198-202.

Volkmann J, Wolters A, Kupsch A et al. Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 2012;11:1029-38.

Volkmann J, Mueller J, Deuschl G et al. Pallidal neurostimulation in patients with medication-refractory cervical dystonia: a randomised, sham-controlled trial. Lancet Neurol 2014;13:875-84.

Cao C, Pan Y, Li D et al. Subthalamus deep brain stimulation for primary dystonia patients: a long-term follow-up study. Mov Disord 2013;28:1877-82.

Fonoff ET, Campos WK, Mandel M et al. Bilateral subthalamic nucleus stimulation for generalized dystonia after bilateral pallidotomy. Mov Disord 2012;27:1559-63.

Ostrem JL, Marks WJ Jr, Volz MM et al. Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome). Mov Disord 2007;22:1885-91.

Foote KD, Sanchez JC, Okun MS. Staged deep brain stimulation for refractory cra- niofacial dystonia with blepharospasm: case report and physiology. Neurosurgery 2005;56:E415.

Hebb MO, Chiasson P, Lang AE et al. Sustained relief of dystonia following cessation of deep brain stimulation. Mov Disord 2007;22:1958-62.

Muta D, Goto S, Nishikawa S et al. Bilateral pallidal stimulation for idiopathic seg- mental axial dystonia advanced from Meige syndrome refractory to bilateral thala- motomy. Mov Disord 2001;16:774-7.

Vercueil L, Pollak P, Fraix V et al. Deep brain stimulation in the treatment of severe dystonia. J Neurol 2001;248:695-700.

Capelle HH, Weigel R, Krauss JK. Bilateral pallidal stimulation for blepharospasm-oro- mandibular dystonia (Meige syndrome). Neurology 2003;60:2017-8.

Houser M, Waltz T. Meige syndrome and pallidal deep brain stimulation. Mov Disord 2005;20:1203-5.

Loher TJ, Capelle HH, Kaelin-Lang A et al. Deep brain stimulation for dystonia: out- come at long-term follow-up. J Neurol 2008;255:881-4.

Berman BD, Starr PA, Marks WJ et al. Induction of bradykinesia with pallidal deep brain stimulation in patients with cranialcervical dystonia. Stereotact Funct Neuro- surg 2009;87:37-44.

Sensi M, Cavallo MA, Quatrale R et al. Pallidal stimulation for segmental dystonia: long term follow up of 11 consecutive patients. Mov Disord 2009;24:1829-35.

Woehrle JC, Blahak C, Kekelia K et al. Chronic deep brain stimulation for segmental dystonia. Stereotact Funct Neurosurg 2009;87:379–384.

Ghang JY, Lee MK, Jun SM et al. Outcome of pallidal deep brain stimulation in Meige syndrome. J Korean Neurosurg Soc 2010;48:134-8.

Inoue N, Nagahiro S, Kaji R, Goto S. Long-term suppression of Meige syndrome after pallidal stimulation: a 10-year follow-up study. Mov Disord 2010;25:1756-8.

Lyons MK, Birch BD, Hillman RA et al. Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus 2010;29:E5.

Markaki E, Kefalopoulou Z, Georgiopoulos M et al. Meige’s syndrome: a cranial dys- tonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 2010;112:344-6.

Romito LM, Elia AE, Franzini A, Bugiani O, Albanese A. Low-voltage bilateral pallidal stimulation for severe meige syndrome in a patient with primary segmental dystonia: case report. Neurosurgery 2010;67 (3 Suppl. Operative):onsE308, discussion onsE.

Reese R, Gruber D, Schoenecker T et al. Long-term clinical outcome in meige syn- drome treated with internal pallidum deep brain stimulation. Mov Disord 2011;26: 691-8.

Sako W, Morigaki R, Mizobuchi Y et al. Meige syndrome: bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord 2011;17:123-5.

Tai CH, Wu RM, Liu HM et al. Meige syndrome relieved by bilateral pallidal stimula- tion with cycling mode: case report. Neurosurgery 2011;69:E1333–E-7.

Ostrem JL, Markun LC, Glass GA et al. Effect of frequency on subthalamic nucleus deep brain stimulation in primary dystonia. Parkinsonism Relat Disord 2014;20:432-8.

Sobstyl M, Zabek M, Mossakowski Z et al. Meige Syndrome: pallidal deep brain stim- ulation in the treatment of Meige syndrome. Neurol Neurochir Pol 2014;48:196-9.

Vidailhet M, Vercueil L, Houeto JL et al. Bilateral, pallidal, deepbrain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 2007;6:223–229.

Vidailhet M, Yelnik J, Lagrange C et al. Bilateral pallidal deep brain stimulation for the treatment of patients with dystoniachoreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol 2009;8:709-17.

Schjerling L, Hjermind LE, Jespersen B et al. A randomized doubleblind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia. J Neu- rosurg 2013;119:1537-45.

Ostrem JL, Starr PA. Treatment of dystonia with deep brain stimulation. Neurother 2008;5:320-30.

Vidailhet M, Vercueil L, Houeto JL et al. Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 2005;352:459-67.

Coubes P, Cif L, El Fertit H et al. Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results. J Neurosurg 2004; 101:189-94.

Kupsch A, Benecke R, Muller J et al. Pallidal deep-brain stimulation in primary gener- alized or segmental dystonia. N Engl J Med 2006;355:1978-90.

Markun LC, Starr PA, Air EL et al. Shorter disease duration correlates with improved long-term deep brain stimulation outcomes in young-onset DYT1 dystonia. Neuro- surgery 2012;71:325-30.

Isaias IU, Alterman RL, Tagliati M. Outcome predictors of pallidal stimulation in patients with primary dystonia: the role of disease duration. Brain 2008;131 (Pt 7): 1895-902.

Holloway KL, Baron MS, Brown R et al. Deep brain stimulation for dystonia: a meta- analysis. Neuromodulation 2006;9:253-61.

Koy A, Hellmich M, Pauls KA et al. Effects of deep brain stimulation in dyskinetic cer- ebral palsy: a meta-analysis. Mov Disord 2013;28:647-54.

Published

2017-11-01

How to Cite

1.
Wang X, Zhang C, Wang Y, Liu C, Zhao B, Zhang J- guo, et al. Deep Brain Stimulation for Craniocervical Dystonia (Meige Syndrome): A Report of Four Patients and a Literature-Based Analysis of Its Treatment Effects.: Originally published in the Neuromodulation journal 2016; 19: 818-23. Translation: Dr. Juan Carlos Andreani. NeuroTarget [Internet]. 2017 Nov. 1 [cited 2024 Nov. 21];11(4):54-61. Available from: https://neurotarget.com/index.php/nt/article/view/141

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Special Papers