Outcome of pallidal deep brain stimulation in meige syndrome

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.

Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was 23.1 ± 6.4 months. The mean age at time of surgery was 58.0 ± 7.8 years. The mean duration of symptoms was 8.7 ± 7.6 years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After 2.4 ± 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).

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.

The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.