Disease Stabilization of DYT1-Positive Primary Generalized Dystonia With Deep Brain Stimulation of the Globus Pallidus Interna: A 15-Year Follow-up

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.

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

2020 ◽  
pp. 105-108
Author(s):  
Mariana Moscovich

Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a safe and long-term effective treatment for medication-refractory dystonia. However, complications and side effects may occur. Freezing of gait (FOG) is a rare phenomenon in patients with dystonia, although very frequently this complication is observed in patients with Parkinson disease (PD). FOG can be disabling and may severely impair quality of life, even when episodic. This chapter reports on a case of a 49-year-old left-handed man presenting with FOG, impairment in balance, and walking difficulty. These issues emerged 3 years after successful bilateral GPi DBS for primary generalized dystonia.


2017 ◽  
Vol 41 ◽  
pp. 58-65 ◽  
Author(s):  
Zheng-Dao Deng ◽  
Dian-you Li ◽  
Chen-cheng Zhang ◽  
Yi-Xin Pan ◽  
Jin Zhang ◽  
...  

2010 ◽  
Vol 25 (3) ◽  
pp. 289-299 ◽  
Author(s):  
Laura Cif ◽  
Xavier Vasques ◽  
Victoria Gonzalez ◽  
Patrice Ravel ◽  
Brigitte Biolsi ◽  
...  

2017 ◽  
Vol 21 (2) ◽  
pp. 197-202 ◽  
Author(s):  
Anouk Y.J.M. Smeets ◽  
Annelien A. Duits ◽  
Albert F.G. Leentjens ◽  
Koen Schruers ◽  
Vivianne van Kranen-Mastenbroek ◽  
...  

2014 ◽  
Vol 5 (9) ◽  
pp. 416 ◽  
Author(s):  
Domenico Servello ◽  
Edvin Zekaj ◽  
Christian Saleh ◽  
Claudia Menghetti ◽  
Mauro Porta

2012 ◽  
Vol 154 (11) ◽  
pp. 2029-2041 ◽  
Author(s):  
M. Porta ◽  
D. Servello ◽  
C. Zanaboni ◽  
F. Anasetti ◽  
C. Menghetti ◽  
...  

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