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Levodopa responsiveness has been shown to be the best predictor of improvement after subthalamic nucleus (STN) deep brain stimulation (DBS) for Parkinson’s disease (PD). The objective of this study was to assess the effect of STN DBS on PD patients intolerant to levodopa due to severe acute side effects such as intolerable nausea. There were 10 patients in the study who received STN DBS for PD. Five patients who were intolerant to levodopa were matched based on age, disease duration, sex and presurgical disease severity to 5 patients taking levodopa and demonstrating a good levodopa response. Both groups had a significant improvement in Unified Parkinson’s Disease Rating Scale activities of daily living and motor subscales as well as tremor, rigidity and bradykinesia scores at 3, 6 and 12 months after surgery compared to baseline, and these improvements were equivalent between the two groups. Patient global ratings also indicated significant improvements at all follow-up visits. There were no differences in stimulator settings between the two groups at the 3-, 6- or 12-month follow-up visits. In conclusion, although levodopa responsiveness is the best predictor for outcome after STN DBS, carefully selected PD patients intolerant to levodopa can have significant improvement.

The relative accuracy of starting point algorithms in microelectrode-guided stereotactic pallidotomy and thalamotomy was evaluated using postoperative magnetic resonance imaging (MRI) data. Multiplanar reformations were performed to align postoperative MRI in anterior-posterior, dorsal-ventral and mediolateral planes. Three-dimensional distance and direction from the pallidal and thalamic stereotactic starting points to the respective radiofrequency lesions were measured. Similar magnitude of microelectrode refinement in pallidotomy and thalamotomy suggested similar accuracy of algorithms used to set the stereotactic starting point. Fewer microelectrode-recording tracts were required to identify optimal lesioning sites in thalamotomy compared to pallidotomy. Lesions were consistently localized anterior and superior to the starting point and a refined starting point algorithm may reduce the number of microelectrode recording tracts.

Accuracy of pallidotomy lesion placement was assessed by comparing actual lesion locations with expected pallidotomy lesion locations based on stereotaxy. Actual and expected lesions were compared in anteroposterior, dorsoventral and lateral axes. In 22 pallidotomies, actual lesion locations were determined using axial MR images. Expected lesion locations were calculated using a starting point derived from preoperative computerized tomography, displacements from the starting point based on microelectrode-driven electrophysiological refinement, and the trajectory angle of the lesioning tract relative to the anterior-posterior commissural plane. On average, actual lesion locations were found 2.91 ± 2.23 mm posterior, 3.22 ± 2.49 mm ventral, and 0.05 ± 1.80 mm lateral compared to the expected lesion location. Discrepancies between the actual lesion and expected lesion locations may be mostly accounted for by posterior and ventral lesion spread from the exposed electrode tip, in-plane and volume averaging effects associated with MR images, and possible brain shifting during surgery. However, despite the remaining small differences between actual and expected lesion location, good clinical outcome of reduced dyskinesias and ‘off’ time along with UPDRS-based improvement in mentation, motor and activity of daily living measures was observed.

Acute thalamotomy and pallidotomy lesion volumes based on magnetic resonance (MR) images were measured in 22 patients (11 thalamotomy and 11 pallidotomy patients). Thalamotomy inner lesion volumes (0.06 ± 0.04 ml; thermocoagulative zone) were smaller than pallidotomy inner lesion volumes (0.14 ± 0.08 ml) as determined using T1-weighted 3D-MPRAGE (1.5-mm slice spacing). Similar results were found using T1-weighted (6-mm slice spacing) image sets (0.09 ± 0.05 ml, thalamotomy; 0.13 ± 0.05 ml, pallidotomy). No differences were found when comparing thalamic or pallidal inner lesion volumes when the comparison was based on T2 weighted images. Thalamotomy total lesion volumes (thermocoagulative and surrounding edematous zones) were less than pallidotomy total lesion volumes independent of the MR protocol. The difference in thalamotomy and pallidotomy lesion volumes is most likely based on the distance between each discrete lesion placed along the lesioning tracts. In 7 of 11 thalamotomies, this distance was 1 mm with the remaining having 2 mm between each discrete lesion. All pallidotomy discrete lesions were 2 mm apart. More overlap between discrete lesioning sites for thalamotomies is likely to produce reduced lesion volumes.

Background: Younger age and a response to a supra-threshold levodopa challenge have been reported to predict a favorable response to bilateral subthalamic nucleus (STN) stimulation. Objective: To identify and evaluate clinical factors which predict a positive outcome after bilateral STN stimulation in patients with Parkinson’s disease, using each patient’s typical doses of antiparkinsonian medication rather than a supra-threshold levodopa challenge. Methods: Forty-five Parkinson’s disease patients who underwent bilateral STN implantation were included in the study. There were 33 men and 12 women. Mean age and disease duration were 59.4 and 12.7 years, respectively. All patients underwent Unified Parkinson Disease Rating Scale (UPDRS) evaluations in the medication off and on states at baseline and postsurgically with stimulation on at 3, 6 and 12 months. The percentage improvement in UPDRS motor scores at baseline (medication off vs. on) was compared with the percentage improvement after stimulation optimization (baseline medication off vs. follow-up medication off/stimulation on). Results: The preoperative percentage improvement in UPDRS motor scores with antiparkinsonian medications was predictive of the postoperative percentage improvement in the UPDRS motor scores in the medication off/stimulation on state (r = 0.53, p < 0.001). However, preoperative UPDRS motor scores in the medication on state were more strongly correlated with the postsurgical effect of stimulation (r = 0.64, p < 0.001). The postoperative improvement in UPDRS motor scores with stimulation did not correlate with age, disease duration or time to programming optimization. Conclusions: The preoperative percentage improvement in UPDRS motor scores with antiparkinsonian medications and UPDRS motor score in the medication on state at baseline are the strongest clinical predictors of responsiveness to bilateral STN stimulation.

Glutaric aciduria type 1 is an inborn error of metabolism due to deficiency of glutaryl-CoA dehydrogenase. This disorder mainly affects children. The majority of patients develop a dystonic-dyskinetic syndrome. The dystonia is painful and can cause significant disability. This report documents an 18-month-old child, the youngest reported, who underwent pallidotomy for disabling dystonia. The surgery improved dystonic symptoms, especially pain in this child with minor complications related to the procedure. Pallidotomy is a reasonable option for children with dystonic symptoms secondary to glutaric aciduria.

Background/Aims: Subthalamic deep brain stimulation (STN-DBS) for Parkinson’s disease (PD) improves motor symptoms and quality of life (QOL). Because depression is a potent correlate of QOL, and STN-DBS may be associated with changes in mood, this study sought to determine whether QOL improvement is a direct or indirect consequence of motor improvement. Methods: 26 patients with PD, free of dementia and major depression, who consecutively underwent bilateral, microelectrode-guided STN-DBS, underwent preoperative and 3-month postoperative neuropsychological evaluation, including measures of QOL (PD Questionnaire –39) and depressive symptoms (Beck Depression Inventory). Results: Motor score in the Unified Parkinson’s Disease Rating Scale (UPDRS Part III) improved significantly with STN-DBS relative to preoperative ‘on’ and ‘off’ scores, as did QOL and depressive symptoms. Extent of QOL improvement tended to be associated with improvement in motor score from presurgical on to postsurgical on stimulation and on medication state. QOL improvement was significantly related to amelioration of depressive symptoms. Partial correlations revealed that the association between QOL improvement and depression remained significant when influence of motor improvement on QOL and depression was controlled for. The motor-QOL association was no longer significant when effects of depression were controlled for. Conclusions: Significant QOL improvements after STN-DBS are associated with improved motor ‘on’ state and depressive symptoms. The influence of motor improvement on QOL may be largely indirect by reducing depression.

Postoperative lesion volume and clinical outcome were assessed in 19 Parkinson’s disease (PD) patients who received posteroventral pallidotomy, and in 14 essential tremor (ET) patients who received ventrolateral thalamotomy. Before and after surgery, PD patients were evaluated using the Unified PD Rating Scale (UPDRS), and ET patients were evaluated using the Fahn-Tolosa-Marin (FTM) tremor rating scale. Inner and total lesion volumes were determined with postoperative MR imaging and three-dimensional data segmentation. Lesion volumes were compared to percent improvement in UPDRS and FTM scores, using Spearman’s rank-order correlation test. No rank-order correlations were found between lesion volume and clinical improvement in either the PD or the ET patients. In performing stereotactic surgery for movement disorders, any lesion volume within a prescribed range may be equally effective in relieving symptoms associated with PD or ET.