Long-Term Efficacy and Safety of Chronic Globus Pallidus Internus Stimulation in Different Types of Primary Dystonia

Abstract

Background: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) offers a very promising therapy for medically intractable dystonia. However, little is known about the long-term benefit and safety of this procedure. We therefore performed a retrospective long-term analysis of 18 patients (age 12–78 years) suffering from primary generalized (9), segmental (6) or focal (3) dystonia (minimum follow-up: 36 months). Methods: Outcome was assessed using the Burke-Fahn-Marsden (BFM) scores (generalized dystonia) and the Tsui score (focal/segmental dystonia). Follow-up ranged between 37 and 90 months (mean 60 months). Results: Patients with generalized dystonia showed a mean improvement in the BFM movement score of 39.4% (range 0–68.8%), 42.5% (range –16.0 to 81.3%) and 46.8% (range –2.7 to 83.1%) at the 3- and 12-month, and long-term follow-up, respectively. In focal/segmental dystonia, the mean reduction in the Tsui score was 36.8% (range 0–100%), 65.1% (range 16.7–100%) and 59.8% (range 16.7–100%) at the 3- and 12-month, and long-term follow-up, respectively. Local infections were noted in 2 patients and hardware problems (electrode dislocation and breakage of the extension cable) in 1 patient. Conclusion: Our data showed Gpi-DBS to offer a very effective and safe therapy for different kinds of primary dystonia, with a significant long-term benefit in the majority of cases.

Early Globus Pallidus Internus Stimulation in Pediatric Patients With Generalized Primary Dystonia: Long-Term Efficacy and Safety

“…[8][9][10][11][12][13][14][15][16][17] In detail, the present analysis showed that the Dystonia Rating Scale scores, which were significantly improved 3 months (movement: 67.4% + 18.7%, disability: 54.4% + 22.4%) and 12 months (movement: 75.4% + 13.5%, disability: 73.4% + 16.9%) after the start of deep brain stimulation, remained stable during long-term follow-up in all patients with a mean motor improvement of 82.9% at a 36-month follow-up and beyond. These convincing results exceed the long-term outcome data available of recently published studies on cohorts consisting mainly of adult patients that report a persistent mean Dystonia Rating Scale motor improvement at 36 months between 50% and 66% 16,27,28 and correspond well with the long-term results in the pediatric subgroup of the study by Cif et al 23 Together with current data clearly supporting the role of early …”

“…[15][16][17] In addition, the long-term benefit of this approach has also been shown. 16,23,27,28 However, although primary generalized dystonia presents mainly at a young age (<10 years) and is usually severely disabling, globus pallidus internus deep brain stimulation is still often not applied in the early course of the disease 1,3,4,10 -even in the absence of effective medical treatment options 4,6,21 and the presence of convincing data on this neurosurgical procedure in pediatric patients (eg, the pioneering work by Coubes and coworkers 8,9,23 ). This phenomenon might in part be due to the persisting lack of long-term data on the efficacy and safety of early globus pallidus internus deep brain stimulation in pediatric and juvenile patients and the averseness to a ''neurosurgical procedure'' on the part of parents and primary caregivers/neurologists.…”

“…18,22,[28][29][30] The beneficial results of the globus pallidus internus deep brain stimulation procedure performed at young age and at an early stage of the disease allowed for the patients to return to a life of much higher quality, restoring social integration and participation: all patients were able to attend school and interact with their peer group with almost no or little restrictions (patient 1 plays soccer and skis, patient 4 plays various sports, and patient 5 rides a regular bicycle; see Table 1). The absence of predictive factors for long-term outcome (at 36 months) in this series, apart from the low number of cases, might well be explained by the fact that all patients were operated on at a young age, and response was excellent in all cases.…”

Deep Brain Stimulation in Children with Dystonia: Experience from a Tertiary Care Center

“…GPi DBS has been an accepted model for the treatment of primary generalized dystonia and its long-term benefit has been well documented [12,27,28,29,30,31]. There has been a reluctance to consider DBS at an early stage in children with primary generalized dystonia despite the recent literature corroborating to the efficacy and safety of early DBS in pediatric patients [16].…”

Laser-evoked synaptic transmission in cultured hippocampal neurons expressing channelrhodopsin-2 delivered by adeno-associated virus

“…Therefore, rAAV-mediated delivery of ChR2 should not only help to investigate functional brain circuits in living animals but it may also provide a plausible approach to treat neurological diseases which require deep brain stimulation (Gradinaru et al, 2007;Mehrkens et al, 2008;Obeso et al, 1997).…”