Long-term clinical outcome of bilateral pallidal stimulation for intractable craniocervical dystonia (Meige syndrome). Report of 6 patients

2017 ◽  
Vol 383 ◽  
pp. 153-157 ◽  
Author(s):  
Michał Sobstyl ◽  
Grażyna Brzuszkiewicz-Kuźmicka ◽  
Artur Zaczyński ◽  
Tomasz Pasterski ◽  
Marta Aleksandrowicz ◽  
...  
2018 ◽  
Vol 130 (1) ◽  
pp. 84-89 ◽  
Author(s):  
Shiro Horisawa ◽  
Taku Ochiai ◽  
Shinichi Goto ◽  
Takeshi Nakajima ◽  
Nobuhiko Takeda ◽  
...  

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.


2010 ◽  
Vol 25 (11) ◽  
pp. 1756-1758 ◽  
Author(s):  
Nobuhiro Inoue ◽  
Shinji Nagahiro ◽  
Ryuji Kaji ◽  
Satoshi Goto

2011 ◽  
Vol 26 (4) ◽  
pp. 691-698 ◽  
Author(s):  
René Reese ◽  
Doreen Gruber ◽  
Thomas Schoenecker ◽  
Hansjörg Bäzner ◽  
Christian Blahak ◽  
...  

2010 ◽  
Vol 29 (2) ◽  
pp. E5 ◽  
Author(s):  
Mark K. Lyons ◽  
Barry D. Birch ◽  
Renee A. Hillman ◽  
Orland K. Boucher ◽  
Virgilio Gerald H. Evidente

Object Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The medical treatment of this condition is largely unsuccessful over time and is a major source of decreased quality of life in those patients suffering from this disease. Recent advances in the application of deep brain stimulation (DBS) surgery techniques for many disorders have prompted several recent reports of DBS for medically refractory cases of Meige syndrome. While the etiology for this disorder is unknown, it is considered by many investigators to be a form of idiopathic torsion dystonia. Pallidal stimulation is widely considered to be effective for dystonia. Methods The authors report the long-term results of bilateral globus pallidus internus (GPi) or subthalamic nucleus (STN) stimulation in 3 patients with Meige syndrome and 1 patient with Parkinson disease and associated craniofacial dystonia treated at their center. Results Initial 12-month and long-term follow-up Burke-Fahn-Marsden scores were substantially improved in all 4 patients compared with preoperative scores. Conclusions Bilateral GPi DBS may be an effective and safe treatment for medically refractory Meige syndrome. The results are comparable with those reported in the literature. Sustained and long-term improvement in symptoms does appear to be reproducible across reports. The authors' patient with Parkinson disease and associated craniofacial dystonia syndrome undergoing bilateral STN DBS noted immediate and sustained improvement in his symptoms. Further study is required, but these results, along with the other reports, suggest that bilateral GPi DBS is an effective treatment for medically refractory Meige syndrome.


2010 ◽  
Vol 67 (3) ◽  
pp. ons-ons ◽  
Author(s):  
Luigi M. Romito ◽  
Antonio E. Elia ◽  
Angelo Franzini ◽  
Orso Bugiani ◽  
Alberto Albanese

Abstract BACKGROUND AND IMPORTANCE: Meige syndrome (MS) is an adult-onset segmental dystonia characterized by the combination of upper and lower cranial involvement. Its treatment is challenging and the use of oral medication or of botulinum neurotoxin treatment is not decisive. Deep brain stimulation of the globus pallidus internum (GPi DBS) has been used occasionally in severe cases. CLINICAL PRESENTATION: We report the long-term efficacy of low-voltage chronic bilateral GPi DBS in a patient with segmental dystonia featuring severe MS and cervical brachial involvement. The patient received a bilateral ventroposterolateral GPi implant. Postoperative 3-dimensional reconstruction allowed checking of the definitive position of the electrode and contacts within the targeted nucleus. Following implant, the patient received bilateral low-voltage stimulation, consisting in amplitude of 1.3 V with a pulse width of 90 microseconds and a frequency of 130 Hz, yielding a current of 23 µA. Clinical follow-up for 38 months showed a progressive and sustained improvement of dystonia with unchanged electrical settings throughout the observation period. The patient again undertook normal life activities. CONCLUSION: Bilateral low-voltage GPi stimulation allowed efficient control of MS symptoms and the associated brachial cervical dystonia.


2001 ◽  
Vol 120 (5) ◽  
pp. A747-A748
Author(s):  
S DRESNER ◽  
A IMMMANUEL ◽  
P LAMB ◽  
S GRIFFIN

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