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Outcome of Pallidal Deep Brain Stimulation in Meige Syndrome

  • Received : 2010.04.21
  • Accepted : 2010.08.03
  • Published : 2010.08.28

Abstract

Objective : Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome. Methods : Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was $23.1{\pm}6.4$ months. The mean age at time of surgery was $58.0{\pm}7.8$ years. The mean duration of symptoms was $8.7 {\pm}7.6$ years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After $2.4{\pm}1.3$ days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Results : BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant. Conclusion : The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.

Keywords

References

  1. Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J : Validity and reliability of a rating scale for the primary torsion dystonias. Neurology 35 : 73-77, 1985 https://doi.org/10.1212/WNL.35.1.73
  2. Jankovic J : Clinical features, differential diagnosis and pathogenesis of blepharospam and cranial-cervical dystonia in Bosniak L (ed) : Blepharospasm advances in ophthalmic plastic reconstructive surgery. New York : Pergamon Press, 1998, pp 67-82
  3. Jeong SG, Lee MK, Kang JY, Jun SM, Lee WH, Ghang CG : Pallidal deep brain stimulation in primary cervical dystonia with phasic type : clinical outcome and postoperative course. J Korean Neurosurg Soc 46 : 346-350, 2009 https://doi.org/10.3340/jkns.2009.46.4.346
  4. Jung HH, Chang JW : Surgical management of hemifacial spasm and Meige syndrome in Krames ES, Hunter Peckham P, Rezai AR (eds) : Neuromodulation, ed 1. Elsevier, 2009, Vol 2, pp587-597
  5. Kupsch A, Benecke R, Müller J, Trottenberg T, Schneider GH, Poewe W, et al. : Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 335 : 1978-1990, 2006
  6. Meige H : Les convulsions de la face, une forme clinique de convulsion faciale, bilatérale et médiane. Rev Neurologique (Paris) 20 : 437-443, 1910
  7. Ostrem JL, Marks WJ Jr, Volz MM, Heath SL, Starr PA : Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome). Mov Disord 22 : 1885-1891, 2007 https://doi.org/10.1002/mds.21580
  8. Tan EK, Jankovic J : Botumlinum toxin A in patients with oromandibular dystonia : long-term follow-up. Neurology 53 : 2102-2107, 1999 https://doi.org/10.1212/WNL.53.9.2102
  9. Vercueil L, Pollak P, Fraix V, Caputo E, Moro E, Benazzouz A, et al. : Deep brain stimulation in the treatment of severe dystonia. J Neurol 248 : 695-700, 2001 https://doi.org/10.1007/s004150170116
  10. Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Benabid AL, Cornu P, et al. : Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352 : 459-467, 2005 https://doi.org/10.1056/NEJMoa042187

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