Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from meige syndrome refractory to bilateral thalamotomy

Muta, Daisuke; Goto, Satoshi; Nishikawa, Sadakatsu; Hamasaki, Tadashi; Ushio, Yukitaka; Inoue, Nobuhiro; Mita, Shuji

doi:10.1002/mds.1122

Cited by 91 publications

(69 citation statements)

References 27 publications

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“…Since Vercueil and coworkers 3 first reported their results of bilateral GPi DBS in a patient with Meige syndrome, several studies have shown that segmental dystonia responds to GPi DBS. 2,3,6,9,11,13,[18][19][20][21][22][23][24][25][29][30][31] The reports on Meige syndrome and its response to DBS are primarily single cases, aside from the excellent report by Ostrem and colleagues 25 ( Table 3). The follow-up time frame has been somewhat limited in most reports, except the cases of Loher et al 21 at 7 years, Hebb et al 11 at 5 years, and Capelle and associates 6 at 2 years.…”

Section: Discussionmentioning

confidence: 99%

“…15 Meige syndrome has become the most common eponym current used in the literature. 2,3,6,9,11,13,21,23,24,27,30 Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The underlying cause of Meige syndrome is unknown, but most investigators consider it a variant of idiopathic torsion dystonia.…”

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confidence: 99%

“…13 Some patients can develop spasmodic dysphonia as well as dystonic involvement of the limbs. 23 The disorder is more common in females by a 3:1 ratio. 14 Medical treatment of this condition has generally been unsuccessful and often limited by partial response and adverse side effects.…”

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confidence: 99%

“…18,19,25,29,31 There have been several recent reports on the efficacy of GPi DBS in selected patients with Meige syndrome. 2,3,6,9,11,12,[21][22][23][24][25]30 We report on our experience in using DBS in 3 cases of Meige syndrome and 1 case of Parkinson disease and associated craniofacial dystonia, with long-term follow-up, and we review the current literature. Object.…”

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confidence: 99%

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

Lyons¹,

Birch²,

Hillman

et al. 2010

FOC

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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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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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confidence: 99%

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

Lyons¹,

Birch²,

Hillman

et al. 2010

FOC

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“…Remarkably, the remaining group of patients showed good benefit from DBS and eventually no longer needed to turn on the neurostimulator. For some DBS patients with dystonia, discontinuing stimulation resulted in a clinical rebound effect with acutely severe symptoms, 109,117,118 whereas in other patients motor signs took hours, days, months, and in some cases years to return (B. Walter, personal communication). Although it is possible that stimulation could form a functional lesion in the tissue surrounding the active electrode contact or contacts, post mortem histology of DBS patients does not support this argument.…”

Section: Variability In Long-term Outcomementioning

confidence: 99%

Mechanisms and Targets of Deep Brain Stimulation in Movement Disorders

et al. 2008

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Summary:Chronic electrical stimulation of the brain, known as deep brain stimulation (DBS), has become a preferred surgical treatment for medication-refractory movement disorders. Despite its remarkable clinical success, the therapeutic mechanisms of DBS are still not completely understood, limiting opportunities to improve treatment efficacy and simplify selection of stimulation parameters. This review addresses three questions essential to understanding the mechanisms of DBS. 1) How does DBS affect neuronal tissue in the vicinity of the active electrode or electrodes? 2) How do these changes translate into therapeutic benefit on motor symptoms? 3) How do these effects depend on the particular site of stimulation? Early hypotheses proposed that stimulation inhibited neuronal activity at the site of stimulation, mimicking the outcome of ablative surgeries. Recent studies have challenged that view, suggesting that although somatic activity near the DBS electrode may exhibit substantial inhibition or complex modulation patterns, the output from the stimulated nucleus follows the DBS pulse train by direct axonal excitation. The intrinsic activity is thus replaced by high-frequency activity that is time-locked to the stimulus and more regular in pattern. These changes in firing pattern are thought to prevent transmission of pathologic bursting and oscillatory activity, resulting in the reduction of disease symptoms through compensatory processing of sensorimotor information. Although promising, this theory does not entirely explain why DBS improves motor symptoms at different latencies. Understanding these processes on a physiological level will be critically important if we are to reach the full potential of this powerful tool.

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Familial aggregation of genetically heterogeneous hypertrophic cardiomyopathy: A boy with LEOPARD syndrome due to PTPN11 mutation and his nonsyndromic father lacking PTPN11 mutations

Digilio

Pacileo

Sárközy

et al. 2004

Birth Defects Research

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Aggregation of syndromic and nonsyndromic HCM in the same family is an unusual pattern of recurrence. Although genetic heterogeneity of LEOPARD and nonsyndromic HCM is not disputed, the existence of peculiar interactions linking genes causing nonsyndromic HCM and HCM in LEOPARD syndrome can be hypothesized. Different genes can work together, and a more severe cardiac phenotype can be due to additive effects. The involvement of familial susceptibility to specific cardiac malformations based on the presence of common predisposing factors can also be considered. Further molecular studies may shed light on these observations.

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Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from meige syndrome refractory to bilateral thalamotomy

Cited by 91 publications

References 27 publications

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

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

Mechanisms and Targets of Deep Brain Stimulation in Movement Disorders

Familial aggregation of genetically heterogeneous hypertrophic cardiomyopathy: A boy with LEOPARD syndrome due to PTPN11 mutation and his nonsyndromic father lacking PTPN11 mutations

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