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  • Pallidal Deep Brain Stimulation In the Treatment of Meige Syndrome”

    Final Number:

    Michael Roman Sobstyl MD, PhD; Miroslaw Zabek MD, PhD; Grazyna Brzuszkiewicz-Kuzmicka MD, PhD; Tomasz Pasterski MD

    Study Design:
    Clinical Trial

    Subject Category:
    Functional Neurosurgery

    Meeting: 2016 ASSFN Biennial Meeting

    Introduction: Meige syndrome (MS) is characterized by blepharospasm, facial, oromandibular, and cervical dystonia. The medical treatment of this condition is challenging and unsuccessful over long time. Recent case reports and small clinical series showed that bilateral deep brain stimulation (DBS) of globus pallidus pars interna (GPi) improves dystonic features of MS validated by Burk-Fahn-Marsden Dystonia Rating Scale (BFMDRS) (1-5).

    Methods: We report on our experience in using bilateral GPi DBS in 5 cases of MS. We present short-term (3 months) follow-up as well long-term (from 24 months to 48 months) results. Preoperative and postoperative BFMDRS assessments were performed on each patient. The postoperative BFMDRS scores was done when both stimulators were switched on and compared to baseline scores.

    Results: Bilateral GPi DBS reduced the BFMDRS total movement score by 75 % at short-term follow-up, and by 87 % at long-term follow-up when compared to baseline scores. The BFMDRS total disability score was reduced by 46 % at short-term follow-up, and by 56 % at long-term follow-up when compared to baseline scores.

    Conclusions: Our results showed that bilateral GPi DBS in MS is effective and safe, if conservative treatment options failed. The benefit is not only observed at short but also at long-term follow-up ranging from 24 to 48 months postoperatively.

    Patient Care: The presented study proves the clinical efficacy of patients with disabling Meige syndrome. Clinical improvement is observed not only at 3 months postoperatively but at longer follow-up covering the period up to 48 months after surgery. This study supports other reports that bilateral pallidal stimulation is effective in resistant to pharmacotherapy Meige syndrome patients. The results may encourage the neurologists for referral of patients with this disabling dystonic condition for functional neurosurgical procedures.

    Learning Objectives: The following learning objectives of the study are: 1) Patients with disabling Meige syndrome may benefit from bilateral GPI stimulation. 2) The response to stimulation is fast and is present at 3 months follow-up period. (early postoperative period). 3) The clinical improvement is seen at longer follow-up ranging from 24 to 48 months with still good control of Meige syndrome.

    References: [1] Capelle HH, Weigel R, Krauss JK. Bilateral pallidal stimulation for blepherospasm-oromandibular dystonia (Meige syndrome). Neurology 2003;60: 2017- 2018. [2] Markaki E, Kefalopoulou Z, Georgiopoulous M, Paschali A, Constantoyannis C. Meige syndrome: a cranial dystonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 2010;112: 344-346. [3] Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VG. Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus. 2010 Aug; 29(2):1-5. [4] Ostrem JL, Marks WJ, Volz MM, Heath SL, Starr Ph. Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome) Mov Disord. 2007; 22:1885-1891. [5] Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, Falk D, Herzog J, Pinsker MO, Schneider GH, Schrader C, Deuschl G, Mehdorn HM, Kupsch A, Volkmann J, Krauss JK. Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord. 2011;26:691-698.

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