Long-term outcome of pallidal stimulation for Meige syndrome

Author:

Horisawa Shiro1,Ochiai Taku2,Goto Shinichi1,Nakajima Takeshi3,Takeda Nobuhiko1,Kawamata Takakazu1,Taira Takaomi1

Affiliation:

1. Department of Neurosurgery, Neurological Institute, Tokyo Women’s Medical University, Tokyo;

2. Ochiai Brain Clinic, Saitama; and

3. Department of Neurosurgery, Jichi Medical University, Tochigi, Japan

Abstract

OBJECTIVEMeige syndrome is characterized by blepharospasm and varied subphenotypes of craniocervical dystonia. Current literature on pallidal surgery for Meige syndrome is limited to case reports and a few small-scale studies. The authors investigated the clinical outcomes of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in patients with Meige syndrome.METHODSSixteen patients who underwent GPi DBS at the Tokyo Women’s Medical University Hospital between 2002 and 2015 were included in this study. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement subscale (BFMDRS-M) scores (range 0–120) obtained at the following 3 time points were included in this analysis: before surgery, 3 months after surgery, and at the most recent follow-up evaluation.RESULTSThe patients’ mean age (± SD) at symptom onset was 46.7 ± 10.1 years, and the mean disease duration at the time of the authors’ initial evaluation was 5.9 ± 4.1 years. In 12 patients, the initial symptom was blepharospasm, and the other 4 patients presented with cervical dystonia. The mean postoperative follow-up period was 66.6 ± 40.7 months (range 13–150 months). The mean total BFMDRS-M scores at the 3 time points were 16.3 ± 5.5, 5.5 ± 5.6 (66.3% improvement, p < 0.001), and 6.7 ± 7.3 (58.9% improvement, p < 0.001).CONCLUSIONSThe results indicate long-term efficacy for GPi DBS for the majority of patients with Meige syndrome.

Publisher

Journal of Neurosurgery Publishing Group (JNSPG)

Subject

Genetics,Animal Science and Zoology

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