Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation

OBJECTIVEPrimary Meige syndrome is characterized by blepharospasm and orofacial–cervical dystonia. Deep brain stimulation (DBS) is recognized as an effective therapy for patients with this condition, but previous studies have focused on clinical effects. This study explored the predictors of clinical outcome in patients with Meige syndrome who underwent DBS.METHODSTwenty patients who underwent DBS targeting the bilateral subthalamic nucleus (STN) or globus pallidus internus (GPi) at the Chinese People’s Liberation Army General Hospital from August 2013 to February 2018 were enrolled in the study. Their clinical outcomes were evaluated using the Burke–Fahn–Marsden Dystonia Rating Scale at baseline and at the follow-up visits; patients were accordingly divided into a good-outcome group and a poor-outcome group. Putative influential factors, such as age and course of disease, were examined separately, and the factors that reached statistical significance were subjected to logistic regression analysis to identify predictors of clinical outcomes.RESULTSFour factors showed significant differences between the good- and poor-outcome groups: 1) the DBS target (STN vs GPi); 2) whether symptoms first appeared at multiple sites or at a single site; 3) the sub-item scores of the mouth at baseline; and 4) the follow-up period (p < 0.05). Binary logistic regression analysis revealed that initial involvement of multiple sites and the mouth score were the only significant predictors of clinical outcome.CONCLUSIONSThe severity of the disease in the initial stage and presurgical period was the only independent predictive factor of the clinical outcomes of DBS for the treatment of patients with Meige syndrome.

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Long-term Thalamic Deep Brain Stimulation for Essential Tremor: Clinical Outcome and Stimulation Parameters

Movement Disorders Clinical Practice ◽

10.1002/mdc3.12337 ◽

2016 ◽

Vol 3 (6) ◽

pp. 567-572 ◽

Cited By ~ 15

Author(s):

Pedro M. Rodríguez Cruz ◽

Antonio Vargas ◽

Carlos Fernández-Carballal ◽

Jose Garbizu ◽

Beatriz De La Casa-Fages ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Clinical Outcome ◽

Essential Tremor ◽

Brain Stimulation ◽

Stimulation Parameters ◽

Deep Brain

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Similar Long‐Term Clinical Outcomes of Deep Brain Stimulation With Different Electrode Targets for Primary Meige Syndrome: One Institution's Experience of 17 Cases

Neuromodulation Technology at the Neural Interface ◽

10.1111/ner.13304 ◽

2020 ◽

Author(s):

Han Tian ◽

Nan‐xiang Xiong ◽

Nian Xiong ◽

Xiao‐ming Liu ◽

Jing Rao ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Clinical Outcomes ◽

Brain Stimulation ◽

Meige Syndrome ◽

Deep Brain

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Long-term clinical outcome of deep brain stimulation in PKAN syndrome

European Journal of Paediatric Neurology ◽

10.1016/j.ejpn.2017.04.1112 ◽

2017 ◽

Vol 21 ◽

pp. e179

Author(s):

Victoria Gonzalez ◽

Laura Cif ◽

Christophe Milesi ◽

Gilles Cambonie ◽

Isabelle de Antonio Rubio ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Clinical Outcome ◽

Brain Stimulation ◽

Deep Brain

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Long-Term Clinical Outcome of Internal Globus Pallidus Deep Brain Stimulation for Dystonia

PLoS ONE ◽

10.1371/journal.pone.0146644 ◽

2016 ◽

Vol 11 (1) ◽

pp. e0146644 ◽

Cited By ~ 11

Author(s):

Hye Ran Park ◽

Jae Meen Lee ◽

Gwanhee Ehm ◽

Hui-Jun Yang ◽

In Ho Song ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Clinical Outcome ◽

Globus Pallidus ◽

Brain Stimulation ◽

Internal Globus Pallidus ◽

Deep Brain

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Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement

Journal of Clinical Medicine ◽

10.3390/jcm8122163 ◽

2019 ◽

Vol 8 (12) ◽

pp. 2163 ◽

Cited By ~ 3

Author(s):

Annika Danielsson ◽

Miryam Carecchio ◽

Laura Cif ◽

Anne Koy ◽

Jean-Pierre Lin ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Clinical Outcome ◽

Brain Stimulation ◽

Rating Scale ◽

Case Series ◽

Long Term Follow Up ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patients, the clinical outcome of pallidal deep brain stimulation in DYT-THAP1 dystonia, was evaluated. The Burke Fahn Marsden Dystonia Rating Scale served as an outcome measure. Nine females and 5 males were enrolled, with a median follow-up of 4 years and 10 months after implant. All benefited from surgery: dystonia severity was reduced by a median of 58% (IQR 31-62, p = 0.001) at last follow-up, as assessed by the Burke Fahn Marsden movement subscale. In the majority of individuals, there was no improvement of speech or swallowing, and overall, the effect was greater in the trunk and limbs as compared to the cranio-cervical and orolaryngeal regions. No correlation was found between disease duration before surgery, age at surgery, or preoperative disease burden and the outcome of deep brain stimulation. Device- and therapy-related side-effects were few. Accordingly, pallidal deep brain stimulation should be considered in clinically impairing and pharmaco-resistant DYT-THAP1 dystonia. The method is safe and effective, both short- and long-term.

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Long-term follow-up of deep brain stimulation for Meige syndrome

Neurosurgical FOCUS ◽

10.3171/2010.4.focus1067 ◽

2010 ◽

Vol 29 (2) ◽

pp. E5 ◽

Cited By ~ 31

Author(s):

Mark K. Lyons ◽

Barry D. Birch ◽

Renee A. Hillman ◽

Orland K. Boucher ◽

Virgilio Gerald H. Evidente

Keyword(s):

Deep Brain Stimulation ◽

Parkinson Disease ◽

Brain Stimulation ◽

Long Term Results ◽

Meige Syndrome ◽

Pallidal Stimulation ◽

Long Term Follow Up ◽

Deep Brain

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.

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