Stereotactic neurosurgery for Meige syndrome: two cases reports and literature review

Abstract

Objective Meige syndrome (MS) is the combination of blepharospasm and oromandibular dystonia.We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) and unilateral pallidotomy in patients with medically refractory MS.2 case reports are presented,and the diagnostic,therapeutic and etiology issues outlined by reviewing the literature.Methods Symptoms were evaluated according to the Burke - Fahn - Marsden dystonia rating scale (BFMDRS). We report a case of a 48 - year - old man with bilateral manifestation of Meige syndrome (MS) successfully treated with Bilateral GPi - DBS.Right - side unilateral pallidotomy has been used with another 45 - year - old man. Results Stereotactic bilateral GPi - DBS of the pallidum led to a dramatic clinical improvement.BFMDRS score showed an improvement of 83.7％ and 69.1％ on the 12 and 24month follow - up.The patient of unilateral pallidotomy BFMDRS score showed an improvement of 78.6％ and 46.2％ on the 1 week and 3 month follow - up. Symptoms were no improvement in 6month follow - up.Conclusions Pallidotomy or thalamotomy is recommended for the treatment of MS in patients on whom deep brain stinulation could not be performed.Stereotactic bilateral GPi DBS may be an effective and safe treatment for primary Meige syndrome who obtain disappointing results from conventional treatment. Key words: Meige syndrome; Globus pallidus intemus; Deep brain stimulation; Pallidotomy; Dystonia