Generalized Dystonia Treated With Deep Brain Stimulator: An Institutional Single Surgeon Experience

Hammad Ghanchi; Jacob E Bernstein; Taha M Taka; Tye Patchana; Samir Kashyap; Omid R Hariri; Ali O Jamshidi; Ajay K Ananda

doi:10.7759/cureus.10992

Generalized Dystonia Treated With Deep Brain Stimulator: An Institutional Single Surgeon Experience

Cureus. 2020 Oct 16;12(10):e10992. doi: 10.7759/cureus.10992.

Authors

Hammad Ghanchi¹, Jacob E Bernstein¹, Taha M Taka², Tye Patchana¹, Samir Kashyap¹, Omid R Hariri³, Ali O Jamshidi⁴, Ajay K Ananda⁴

Affiliations

¹ Neurosurgery, Riverside University Health System Medical Center, Moreno Valley, USA.
² Neurosurgery, University of California Riverside, Riverside, USA.
³ Neurosurgery, Kaiser Permanente-Orange County, Anaheim, USA.
⁴ Neurosurgery, Kaiser Permanente, Los Angeles, USA.

Abstract

Introduction Dystonia can cause severe disability when left untreated. Once a patient has exhausted medical management, surgical intervention may be the only treatment option. Although not curative, deep brain stimulation has been shown to be beneficial for patients affected by this condition. Our study sought to review patients undergoing deep brain stimulation for medically refractory dystonia to assess outcomes. Methods Our institution's operative database was reviewed retrospectively for all patients undergoing deep brain stimulator placement over the last six years. These medical records were reviewed for the severity of dystonia preoperatively and followed postoperatively for 24 months, focusing on the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Patients with less than two-year postoperative follow-up were excluded from the study. The patients were further stratified by age into Group A, consisting of patients less than 40 years old, and Group B, patients greater than or equal to 40 years old. Other attributes such as age, sex, age of disease onset, disease duration at the time of surgery, genetic tests for dystonia-related genes, and any complication associated with surgery were also reviewed. Results Four hundred fifty-five operative cases for deep brain stimulator placement were reviewed, and 16 patients met inclusion criteria for the study. The mean age for our patient cohort was 43.75 years, with four males and 12 females. The average time from the age of disease onset to time of surgery was 9.7 years for Group A and 10.8 years for Group B; the overall average was 10.3 years. All patients had globus pallidus interna (GPi) as their surgical target. The first incidence of a statistically significant decrease in BFMDRS score was noted at three months postoperatively (p<0.001) when compared to preoperative values. Fourteen patients in our cohort underwent preoperative genetic testing for DYT gene mutations, out of which four were found to have a mutation. Conclusion Our review of outcomes for primary generalized dystonia at our institution found that deep brain stimulator targeting the GPi is safe and effective. We found an overall 88% response rate with younger patients (< 40-year-old) showing a better response at two years than older patients.

Keywords: deep brain stimulation; dystonia.