Stimulation of the globus pallidus internus in a patient with DYT1-positive primary generalized dystonia: a 10-year follow-up

2010 ◽  
Vol 29 (2) ◽  
pp. E16 ◽  
Author(s):  
Dunbar Alcindor ◽  
Michael Y. Oh ◽  
Susan Baser ◽  
Cindy Angle ◽  
Boyle C. Cheng ◽  
...  

The authors report the case of DYT1-positive primary generalized dystonia refractory to medical management that was successfully treated with continuous deep brain stimulation of the internal segment of the globus pallidus. Prior studies have shown that neuromusculoskeletal deficits can remain permanent if early surgical intervention is not undertaken. The authors report prolonged efficacy and safety over a 10-year period in a 28-year-old man.

2008 ◽  
Vol 109 (1) ◽  
pp. 130-132 ◽  
Author(s):  
Brigitte Biolsi ◽  
Laura Cif ◽  
Hassan El Fertit ◽  
Santiago Gil Robles ◽  
Philippe Coubes

Deep brain stimulation is now accepted as a safe and efficient treatment for movement disorders including selected types of dystonia and dyskinesia. Very little, however, is known about its effect on other movement disorders, particularly for “choreic” movements. Huntington disease is a fatal autosomal-dominant neurodegenerative disorder characterized by movement disorders, progressive cognitive impairment, and psychiatric symptoms. Bilateral chronic stimulation of the internal globus pallidus was performed to control choreic movements in a 60-year-old man with a 10-year history of Huntington disease. Chronic deep brain stimulation resulted in remarkable improvement of choreic movements. Postoperative improvement was sustained after 4 years of follow-up with a marked improvement in daily quality of life.


2008 ◽  
Vol 50 (12) ◽  
pp. 1055-1059 ◽  
Author(s):  
Mikhail F. Chernov ◽  
Taku Ochiai ◽  
Takaomi Taira ◽  
Yuko Ono ◽  
Ryoichi Nakamura ◽  
...  

2021 ◽  
Vol 201 ◽  
pp. 106449
Author(s):  
Mona Ramezani Ghamsari ◽  
Shadi Ghourchian ◽  
Maziar Emamikhah ◽  
Mahdi Safdarian ◽  
Gholamali Shahidi ◽  
...  

2017 ◽  
Vol 14 (5) ◽  
pp. E53-E57 ◽  
Author(s):  
Derrick A Dupre ◽  
Raj Nangunoori ◽  
Sravanthi Koduri ◽  
Cindy Angle ◽  
Diane Cantella ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Primary generalized dystonia (PGD) is a genetic form of dystonia that frequently displays pharmacological resistance and progresses quickly after onset. Deep brain stimulation (DBS) has been used successfully to treat refractory dystonia, specifically globus pallidus interna (GPi) DBS for DYT1-positive PGD patients. Long-term follow-up of the safety and efficacy falls short of the longevity seen in other diseases treated with DBS. CASE PRESENTATION A male patient presented for neurosurgical evaluation with scapular winging, hand contractures, and violent truncal spasms, which forced him to be bedridden. After failing conservative therapy, the 18-yr-old patient was implanted with bilateral GPi-DBS. DBS parameter adjustments were made primarily within the first 3 yr after implantation, with nominal changes thereafter. Initial settings were contact of 3 + 0−, amplitude of 4.9 V, frequency of 185 Hz, and pulse width of 270 μsec on the left and 3 + 0−, 2.8 V, 185 Hz, and 120 μsec on the right. Current settings are 3 + 2 + 1−, 5.2 V, 130 Hz, 330 μsec on the left and 3 + 0−, 3.5 V, 185 Hz, and 180 μsec on the right and have been relatively unchanged in the past 4 yr. Unified dystonia rating scale scores reveal a significant decrease in dystonic symptoms. CONCLUSION While prior reports have shown that GPi-DBS is effective for dystonia, this is the first with 15 yr of long-term follow-up showing disease stabilization, suggesting that stimulation is efficacious and can potentially prevent disease progression. This report reaffirms previous reports that recommend early surgical intervention before the onset of permanent musculoskeletal deficits.


2005 ◽  
Vol 352 (5) ◽  
pp. 459-467 ◽  
Author(s):  
Marie Vidailhet ◽  
Laurent Vercueil ◽  
Jean-Luc Houeto ◽  
Pierre Krystkowiak ◽  
Alim-Louis Benabid ◽  
...  

2009 ◽  
Vol 24 (16) ◽  
pp. 2363-2369 ◽  
Author(s):  
David Grabli ◽  
Claire Ewenczyk ◽  
Maria-Clara Coelho-Braga ◽  
Christelle Lagrange ◽  
Valerie Fraix ◽  
...  

2018 ◽  
Vol 128 (3) ◽  
pp. 897-902 ◽  
Author(s):  
Shikun Zhan ◽  
Fafa Sun ◽  
Yixin Pan ◽  
Wei Liu ◽  
Peng Huang ◽  
...  

OBJECTIVESubthalamic nucleus deep brain stimulation has been shown to be effective in reducing symptoms of primary Meige syndrome. However, assessments of its efficacy and safety have been limited to several case reports and small studies.METHODSThe authors performed a retrospective study to assess the efficacy and safety of bilateral subthalamic nucleus stimulation in 15 patients with primary Meige syndrome who responded poorly to medical treatments or botulinum toxin injections. Using the movement and disability subscores of the Burke-Fahn-Marsden Dystonia Rating Scale, the authors evaluated the severity of patients’ dystonia and related before surgery and at final follow-up during neurostimulation. The movement scale was assessed based on preoperative and postoperative video documentation by an independent rater who was unaware of each patient’s neurostimulation status. Quality of life was assessed with the Medical Outcomes Study 36-Item Short-Form General Health Survey.RESULTSThe dystonia movement subscores in 14 consecutive patients improved from 19.3 ± 7.6 (mean ± standard deviation) before surgery to 5.5 ± 4.5 at final follow-up (28.5 ± 16.5 months), with a mean improvement of 74% (p < 0.05). The disability subscore improved from 15.6 ± 4.9 before surgery to 6.1 ± 3.5 at final follow-up (p < 0.05). In addition, the postoperative SF-36 scores increased markedly over those at baseline. The authors also found that bilateral stimulation of the subthalamic nucleus immediately improved patient symptoms after stimulation and required lower stimulation parameters than those needed for pallidal deep brain stimulation for primary Meige syndrome. Four adverse events occurred in 3 patients; all of these events resolved without permanent sequelae.CONCLUSIONSThese findings provide further evidence to support the long-term efficacy and safety of subthalamic nucleus stimulation as an alternative treatment for patients with medically intractable Meige syndrome.


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