Tag: dystonia

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Deep Brain Stimulation in DYT1 Dystonia: A 10-Year Experience

Electrode-Implantation-for-Deep-Brain-Stimulation-small1

Neurosurgery 73:86–93, 2013

Globus Pallidus Interna (GPi) deep brain stimulation (DBS) is an effective treatment for DYT1-associated dystonia, but long-term results are lacking.

OBJECTIVE: To evaluate the long-term effects of GPi DBS in patients with DYT1 dystonia.

METHODS: A retrospective chart review (cohort study) of 47 consecutive DYT11 patients treated by a single surgical team over a 10-year period and followed for up to 96 months (mean, 46 months) was performed. Symptom severity was quantified with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (M) and disability (D) sub-scores.

RESULTS: As measured with the BFMDRS (M), symptom severity was reduced to less than 20% of baseline after 2 years of DBS therapy (P = .001). The disability scores were reduced to ,30% of baseline (P = .001). Symptomatic improvement was durable throughout available follow-up. Sixty-one percent of patients had discontinued all dystonia-related medications at their last follow-up. Ninety-one percent had discontinued at least 1 class of medication. Infections requiring removal and later reimplantation of hardware occurred in 4 of 47 patients (8.5%). Hardware malfunction including lead fractures occurred in 4 of 47 cases (8.5%). Lead revision to address poor clinical response was performed in 2 of 92 implanted leads (2.2%).

CONCLUSION: GPi DBS is an effective therapy for DYT1-associated torsion dystonia. Statistically significant efficacy is maintained for up to 7 years. Neurologic complications are rare, but long-term hardware-related complications can be significant.

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Accuracy of stimulating electrode placement in paediatric pallidal deep brain stimulation for primary and secondary dystonia

DBS in pediatric dystonia

Acta Neurochir (2013) 155:823–836

Accuracy of electrode placement is an important determinant of outcome following deep brain stimulation (DBS) surgery. Data on accuracy of electrode placement into the globus pallidum interna (GPi) in paediatric patients is limited, particularly those with non-primary dystonia who often have smaller GPi. Pallidal DBS is known to bemore effective in the treatment of primary dystonia compared with secondary dystonia.

Objectives We aimed to determine if accuracy of pallidal electrode placement differed between primary, secondary and NBIA (neuronal degeneration and brain iron accumulation) associated dystonia and how this related to motor outcome following surgery.

Methods A retrospective review of a consecutive cohort of children and young people undergoing DBS surgery in a single centre. Fused in frame preoperative planning magnetic resonance imaging (MRI) and postoperative computed tomography (CT) brain scans were used to determine the accuracy of placement of DBS electrode tip in Leskell stereotactic system compared with the planned target. The differences along X, Y, and Z coordinates were calculated, as was the Euclidean distance of electrode tip from the target. The relationship between proximity to target and change in Burke-Fahn-Marsden Dystonia Rating Scale at 1 year was also measured.

Results Data were collected from 88 electrodes placed in 42 patients (14 primary dystonia, 18 secondary dystonia and 10 NBIA associated dystonia). Median differences between planned target and actual position were: left-side X-axis 1.05 mm, Y-axis 0.85 mm, Z-axis 0.94 mm and Euclidean difference 2.04 mm; right-side X-axis 1.28 mm, Y-axis 0.70 mm, Z-axis 0.70 mm and Euclidean difference 2.45 mm. Accuracy did not differ between left and right-sided electrodes. No difference in accuracy was seen between primary, secondary or NBIA associated dystonia. Dystonia reduction at 1 year post surgery did not appear to relate to proximity of implanted electrode to surgical target across the cohort.

Conclusions Accuracy of surgical placement did not differ between primary, secondary or NBIA associated dystonia. Decreased efficacy of pallidal DBS in secondary and NBIA associated dystonia is unlikely to be related to difficulties in achieving the planned electrode placement.

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High-Frequency Pallidal Stimulation for Camptocormia in Parkinson Disease: Case Report

Neurosurgery 68:E1501–E1505, 2011 DOI: 10.1227/NEU.0b013e318210c859

Camptocormia is characterized by abnormal flexion of the thoracolumbar spine that increases during upright posture and abates in the recumbent position and has been reported to occur in patients with Parkinson disease. Camptocormia causes significant spinal and abdominal pain, impairment of balance, and social stigma.

CLINICAL PRESENTATION: A 57-year-old woman with Parkinson disease developed severe camptocormia, which did not improve with trials of antiparkinsonian and muscle relaxant medications. The patient was successfully treated with bilateral globus pallidus interna deep brain stimulation surgery under general anesthesia. High-frequency neuromodulation afforded relief of camptocormia and improvement in Parkinson disease symptoms.

CONCLUSION: Camptocormia in Parkinson disease may represent a form of dystonia and can be treated effectively with chronic pallidal neuromodulation.

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Best surgical practices: a stepwise approach to the University of Pennsylvania deep brain stimulation protocol

Neurosurg Focus 29 (2):E3, 2010. (DOI: 10.3171/2010.4.FOCUS10103)

Deep brain stimulation (DBS) is the treatment of choice for otherwise healthy patients with advanced Parkinson disease who are suffering from disabling dyskinesias and motor fluctuations related to dopaminergic therapy. As DBS is an elective procedure, it is essential to minimize the risk of morbidity. Further, precision in targeting deep brain structures is critical to optimize efficacy in controlling motor features. The authors have already established an operational checklist in an effort to minimize errors made during DBS surgery. Here, they set out to standardize a strict, step-by-step approach to the DBS surgery used at their institution, including preoperative evaluation, the day of surgery, and the postoperative course. They provide careful instruction on Leksell frame assembly and placement as well as the determination of indirect coordinates derived from MR images used to target deep brain structures. Detailed descriptions of the operative procedure are provided, outlining placement of the stereotactic arc as well as determination of the appropriate bur hole location, lead placement using electrophysiology, and placement of the internal pulse generator. The authors also include their approach to preventing postoperative morbidity. They believe that a strategic, step-by-step approach to DBS surgery combined with a standardized checklist will help to minimize operating room mistakes that can compromise targeting and increase the risk of complication.

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