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 ± 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). 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.
ABSTRACT
OBJECTIVE: The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). METHODS: Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for 18.7 +/- 11.1 months. The mean duration of the CD was 5.8 +/- 3.4 years. The mean age at time of surgery was 54.2 +/- 10.2 years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. RESULTS: The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p = 0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged 81.7 +/- 6.8% at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. CONCLUSIONS: Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.
