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

Lyons, Mark; Birch, Barry D.; Hillman, Renee; Boucher, Orland K.; Evidente, Virgilio Gerald H.

doi:10.3171/2010.4.focus1067

Cited by 54 publications

(33 citation statements)

References 28 publications

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“…In recent years, DBS has been tested in localized forms of dystonia, including patients affected by blepharospasm . No study dealt with patients with isolated blepharospasm.…”

Section: Management Of Blepharospasmmentioning

confidence: 99%

“…112 In recent years, DBS has been tested in localized forms of dystonia, including patients affected by blepharospasm. [113][114][115][116][117][118][119][120][121][122][123][124][125] No study dealt with patients with isolated blepharospasm. Most patients underwent bilateral DBS of the GPi, and the benefits associated with DBS for dystonia often develop gradually over several months.…”

Section: E F a Z I O E T A Lmentioning

confidence: 99%

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Blepharospasm 40 years later

et al. 2017

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Forty years ago, C.D. Marsden proposed that blepharospasm should be considered a form of adult-onset focal dystonia. In the present paper, we provide a comprehensive overview of the findings regarding blepharospasm reported in the past 40 years. Although prolonged spasms of the orbicularis oculi muscles remain the clinical hallmark of blepharospasm, patients with blepharospasm may be characterized by various types of involuntary activation of periocular muscles. In addition to motor features, blepharospasm patients may also have nonmotor manifestations, including psychiatric, mild cognitive, and sensory disturbances. The various motor and nonmotor symptoms are not present in all patients, suggesting that blepharospasm is phenomenologically a heterogeneous condition. This emphasizes the need for tools for severity assessment that take into account both motor and nonmotor manifestations. The cause of blepharospasm remains elusive, but several lines of evidence indicate that blepharospasm is a multifactorial condition in which one, or several, as yet unknown genes together with epigenetic and environmental factors combine to reach the threshold of the disease. Although blepharospasm was originally believed to be solely a basal ganglia disorder, neurophysiological and neuroimaging evidence point to anatomical and functional involvement of several brain regions. The contribution of multiple areas has led to the hypothesis that blepharospasm should be considered as a network disorder, and this might reflect the varying occurrence of motor and nonmotor manifestations in blepharospasm patients. Despite advances in the aetiology and pathophysiology, treatment remains symptomatic.

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“…In recent years, DBS has been tested in localized forms of dystonia, including patients affected by blepharospasm . No study dealt with patients with isolated blepharospasm.…”

Section: Management Of Blepharospasmmentioning

confidence: 99%

Section: E F a Z I O E T A Lmentioning

confidence: 99%

Blepharospasm 40 years later

et al. 2017

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“…Since alternative approaches have not been well studied, it may be reasonable to consider a staged or alternatively a simultaneous GPi-DBS implantation approach to medication refractory cranio-facial and cranio-cervical dystonia. Patient selection and patient counseling concerning the anticipated surgical outcome will be an important issue in these patients as outcomes can be mixed [7][8][9][10][20][21][22][23].…”

Section: Discussionmentioning

confidence: 99%

Mixed results for GPi-DBS in the treatment of cranio-facial and cranio-cervical dystonia symptoms

Limotai

Oyama

et al. 2011

J Neurol

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The aim of the study is to determine clinical outcomes in patients undergoing Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) for cranio-facial and cranio-cervical dystonia (Meige) symptoms. A total of 6 patients seen between 2002 and 2010 with cranio-facial and cranio-cervical dystonia symptoms were identified from the University of Florida Institutional Review Board approved database. Patients were videotaped using a standardized protocol, and tapes were randomized and blindly reviewed by a movement disorders neurologist. The Unified Dystonia Rating Scale improved 31.6 ± 23.2% (range: 3.4-63.2%) at 6 months and 63.7 ± 35.3% (range: 6.3-100%) at 12 months. The Burke-Fahn-Marsden Dystonia Rating Scale improved 45.3 ± 29.5% (range: 4.7-75.0%) at 6 months and 61.8 ± 30.9% (range: 16.6-100%) at 12 months. One patient significantly had a very large improvement with little evidence of residual dystonia. Blepharospasm improved in all patients, whereas speech and swallowing did not improve in this cohort. Two patients improved with unilateral GPi-DBS, although one required a contralateral DBS later in the disease course. Two patients were managed with low frequency stimulation (<100 Hz). Two patients had less than 20% benefit. GPi-DBS for cranio-facial and cranio-cervical symptoms is an effective strategy to manage a subset of patients who remain unresponsive to optimized medical management. Unilateral stimulation may be an option for some patients, but it remains unclear whether response to single-sided stimulation will be sustainable. The mixed results of this GPi-DBS case series highlight the need for a careful re-examination of selection criteria, alternative brain targets, and possibly rescue leads for patients who are non-responders to the GPi target.

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“…Recent case reports with long-term follow-up (1 to 4 years) show sustained improvement in cranio-facio-cervical dystonia by GPi DBS. Improvement in terms of the BFMDRS movement score was 53% in one report and 82 -86% in the other reports (Ghang et al, 2010;Lyons et al, 2010;Reese et al, 2011;Sako et al, 2011). However, in a recent case series, the effect of GPi DBS on Meige syndrome was variable with some patients having less than 20% improvement (Limotai et al, 2011).…”

Section: Craniofacial and Craniocervical Dystonia (Meige Syndrome)mentioning

confidence: 93%