Burke-Fahn-Marsden Dystonia Rating Scale Movement Scores Research Articles

Pallidal versus subthalamic deep brain stimulation for Meige syndrome: A systematic review and meta-analysis

BackgroundGlobus pallidus internus (GPi) and subthalamic nucleus (STN) are two common deep brain stimulation (DBS) targets. This meta-analysis was to compared the efficacy and safety of these two DBS targets for the treatment of Meige syndrome (MS). MethodsA systematic search was performed using EMBASE, MEDLINE, the Cochrane Library, and ClinicalTrials.gov to identify DBS trials for MS. Review Manager 5.3 was used to perform meta-analysis and the mean difference (MD) was analyzed and calculated with a random effect model. Pearson's correlation coefficients and meta-regression analyses were utilized to identify relevant predictive markers. ResultsTwenty trials involving 188 participants with GPi-DBS and 110 individuals with STN-DBS were eligible. Both groups showed improvement of the Burke-Fahn-Marsden Dystonia Rating Scale-Movement (BFMDRS-M) and Disability (BFMDRS-D) scores (BFMDRS-M: MD = 10.57 [7.74–13.41] for GPi-DBS, and MD = 8.59 [4.08–13.11] for STN-DBS; BFMDRS-D: MD = 5.96 [3.15–8.77] for GPi-DBS, and MD = 4.71 [1.38–8.04] for STN-DBS; all P < 0.001) from baseline to the final follow-up, while no notable disparity in improvement rates was observed between them. Stimulation-related complications occurrence was also similar between two groups (38.54 ± 24.07% vs. 43.17 ± 29.12%, P = 0.7594). Simultaneously, preoperative BFMDRS-M score and disease duration were positively connected with the relative changes in BFMDRS-M score at the final visit. ConclusionBoth GPi-DBS and STN-DBS are effective MS therapies, with no differences in efficacy or the frequency of stimulation-related problems. Higher preoperative scores and longer disease duration probably predict greater improvement.

Metabolic Imaging of Deep Brain Stimulation in Meige Syndrome.

ObjectivesThe subthalamic nucleus (STN) has been shown to be a safe and effective deep brain stimulation (DBS) surgical target for the treatment of Meige syndrome. The aim of this study was to compare changes in brain metabolism before and 6 months after STN-DBS surgery.MethodsTwenty-five patients with primary Meige syndrome underwent motor function assessment, including the Burke–Fahn–Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability subscale (BFMDRS-D) and positron emission tomography with an 18[F]-fluorodeoxyglucose scan before and 6 months after STN-DBS surgery. For the voxelwise metabolic change assessment, the p-value was controlled for multiple comparisons using the familywise error rate.ResultsThere was a significant decrease in BFMDRS-M scores 6 months after STN-DBS, from 10.02 ± 3.99 to 4.00 ± 2.69 (p < 0.001). The BFMDRS-D scores also decreased significantly from 4.52 ± 2.90 to 0.64 ± 1.29 (p < 0.001). In the left hemisphere, hypermetabolism was found in the occipital lobe, superior parietal gyrus, postcentral gyrus and thalamus. In the right hemisphere, hypermetabolism was found in the lentiform nucleus, precuneus and precentral gyrus in patients with Meige syndrome receiving DBS. In addition, the bilateral inferior temporal gyrus and middle frontal gyrus exhibited glucose hypermetabolism.ConclusionOur findings indicate that STN-DBS has a significant effect on metabolic level in the brain, which may be an important mechanism for the treatment of Meige syndrome using STN-DBS.

GPi-DBS for KMT2B-Associated Dystonia: Systematic Review and Meta-Analysis.

Early evidence suggests good response to pallidal deep brain stimulation (DBS) in DYT-KMT2B. We aimed to conduct a systematic review and meta-analysis to assess outcomes and identify predictors of good outcome following GPi-DBS in DYT-KMT2B. We searched MEDLINE, Cochrane and MDS-abstracts databases using the MeSH terms "KMT2B and DYT28". We included studies that reported objective outcomes following GPi-DBS in DYT-KMT2B. The BFMDRS-M (Burke-Fahn-Marsden Dystonia Rating Scale- Movement) total scores pre- and post-surgery were used to quantify outcomes. We calculated pooled effects using a random effects meta-analysis and used meta-regression to identify potential effect modifiers. Multiple linear regression using individual patient data was used to identify predictors of good outcome (>50% improvement from baseline on BFMDRS-M). Initial searches screened 132 abstracts of which 34 full-text articles were identified to be of potential interest. Ten studies reporting 42 individual patients, met the inclusion/exclusion criteria and were included in the final review. The mean age at onset was 6.4 ± 5.7 years and 40% were male. The median follow-up was 12months (range: 1-264months). GPi-DBS resulted in median BFMDRS-M improvement of 42.7% (range: -103.5% to 95.9%) postoperatively. Pooled proportion of patients experiencing clinical improvement >50% on BFMDRS-M was 41% (95% CI: 27%-57%). Male gender [β: 22.6, 95% CI: 8.0-37.3, P=0.004), and higher pre-operative BFMDRS-M score [β: 0.62, 95% CI: 0.36-0.87, P < 0.001) were independently associated with better outcome. KMT2B-associated dystonia responds effectively to pallidal stimulation. The outcome is better in males and those with more severe dystonia at baseline.

Subthalamic Nucleus Deep Brain Stimulation in Primary Meige Syndrome: A 1-Year Follow-Up Study

ObjectiveTo investigate the efficacy of bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) in patients with Meige syndrome. Materials and MethodsFifteen consecutive patients who underwent STN-DBS at the Peking University People’s Hospital between September 2017 and June 2018 were included in this study. The Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS) movement score and the BFMDRS disability score were obtained prior to surgery, and at specific time points after surgery. Patients’ sleep status was also assessed before and after surgery. ResultsThe BFMDRS movement scores decreased from 15.3 ± 4.6 to 5.2 ± 6.2 after STN-DBS, with a mean improvement of 68.6% (p < 0.05). The BFMDRS disability scores were also significantly decreased, from 6.9 ± 3.3 to 3.5 ± 2.9, with a mean improvement of 51.7% (p < 0.05). The eye, mouth, speech, and swallowing movement scores also decreased significantly after STN-DBS compared to baseline (p < 0.05). The sleep quality of the patients was also improved after surgery. ConclusionsThese findings demonstrate that the STN is an effective brain target for the treatment of patients with Meige syndrome. STN-DBS was not only able to improve patients’ motor symptoms, but also their sleep status.

Pallidal deep brain stimulation in primary Meige syndrome: clinical outcomes and psychiatric features

ObjectivesTo study the efficacy and safety of bilateral globus pallidus internus deep brain stimulation (GPi-DBS) in refractory Meige syndrome (MS) and evaluate the psychiatric disorders before and after surgery.MethodsTwenty-two patients...

Deep brain stimulation for Meige syndrome: a meta-analysis with individual patient data.

Deep brain stimulation (DBS) is an effective intervention for Meige syndrome, a type of dystonia characterized by blepharospasm, facial, and oromandibular dystonia. This individual patient-level data meta-analysis was to identify the potential outcome predictors, compare the stimulation targets and summarize the efficacy of DBS for Meige syndrome. Three electronic databases (PubMed, Web of Science and Embase) were searched with no publication data restriction to identify studies regarding DBS for Meige syndrome. The primary outcome was the improvement in BFMDRS-M score. Pearson's correlation coefficients and a stepwise multivariate regression analysis were used to identify the potential prognostic factors. Twenty-three studies (115 patients, 94 with pallidal stimulation and 21 with subthalamic stimulation) were eligible. Patients showed significant improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) (21.5 ± 11.0 vs 8.6 ± 6.9, P < 0.001) and disability (BFMDRS-D) (6.4 ± 5.1 vs 2.9 ± 2.4, P < 0.001) scores at the last follow-up visit (31.9 ± 30.7months), compared with scores at baseline. Preoperative BFMDRS-M and BFMDRS-D scores were positively correlated with the relative changes in BFMDRS-M score at the last follow-up visit. On the stepwise multivariate regression, only the preoperative BFMDRS remained significant in the best predictive model. Based on the existing evidence, pallidal/subthalamic stimulation is an effective therapy for even the refractory Meige syndrome. Higher preoperative scores probably indicate larger improvement. Stimulation targets or other clinical factors do not constitute the outcome predictive factors.

Comparison of Short-Term Stimulation of the Globus Pallidus Interna and Subthalamic Nucleus for Treatment of Primary Dystonia

To compare the efficacy and side effects of bilateral globus pallidus internus (GPi) and subthalamic nucleus (STN) deep brain stimulation (DBS) in the same patient with primary dystonia. Patients with primary dystonia from the department of functional neurosurgery in Beijing Tiantan Hospital were recruited for the study. Four electrodes were bilaterally implanted in the GPi and STN. A trial stimulation was applied to determine the preliminary therapeutic effects. Five evaluations were conducted: preoperative, postoperative (before stimulation), after sham stimulation, and after stimulation for 24 hours of GPi and STN using optimal parameters, judged by the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). The BFMDRS movement score decreased after both short-term GPi stimulation (from 15.3 ± 6.9 to 7.6 ± 4.2, P < 0.05) and short-term STN stimulation (from 15.3 ± 6.9 to 8.6 ± 5.0, P < 0.05). There were significant reductions in facial (eyes and mouth) movement scores after short-term GPi and STN DBS compared with baseline (P < 0.05), but not in cervical symptoms (P > 0.05). The cervical symptoms of tonic dystonia had an improvement after long-term DBS treatment (P < 0.05). There were more adverse events with STN DBS; however, most side effects could be ameliorated by adjusting stimulation parameters. Both short-term GPi and STN stimulation improved the motor symptoms of dystonia, but there was no significant difference between GPi DBS and STN DBS. There were more side effects associated with STN stimulation.

Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN)

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature. All patients received neurological evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS) scoring system before and after surgery. Patients were then subject to regular clinical follow-ups (ranging from 22 to 44 months). The mean stimulation amplitude, pulse width and frequency was 2.65 ± 0.45 V, 91.7 ± 21.9 μs, and 146.7 ± 12.5 Hz, respectively. BFMDRS scores were improved in all patients after surgery, ranging from 41.6 to 73.1%. Improvements of appendicular symptoms ranged from 46.2 to 94.1%, and improvements of axial symptoms ranged from 27.3 to 33.3%. No side effects were reported in patients 1 and 2; whereas patient 3 exhibited a mild decline in verbal fluency one year after surgery. STN stimulation could serve as a candidate DBS target in the treatment of PKAN, especially for patients with prominent appendicular symptoms.

Long-term follow-up study on deep brain stimulation for post-traumatic dystonia

Background Deep brain stimulation (DBS) offers a very promising therapy for medically intractable dystonia. Among different dystonia subtypes, the surgical outcome of primary dystonia is most convincing, while that of post-traumatic dystonia is uncertain. This paper aims to evaluate the effect of DBS on post-traumatic dystonia. Methods Four patients of post-traumatic dystonia treated with DBS on globus pallidus internus (GPi) or subthalamic nucleus (STN) were reviewed and their surgical effect was evaluated. Outcome assessments were based on Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement and disability scores preoperatively and one month, 6 months, one year and 2 years after surgery. Improvement rate was counted to evaluate the curative effect. Results BFMDRS movement scores were improved by 38.35%, 47.28%, 62.74% and 68.69% respectively, and disability scores were improved by 35.36% , 46.83% , 59.60% and 67.01% respectively. Imaging features of these patients were reviewed. Although the location and size of encephalomalacia differed among these patients, the anatomical features of basal ganglia remained intact. Conclusions With strict selection, DBS may be a promising treatment to ameliorate the symptoms of post-traumatic dystonia. The surgical effect may be sustainable in long term. Anatomical integrity of basal ganglia may be an important factor to predict good outcome. DOI: 10.3969/j.issn.1672-6731.2015.10.006

PP12.1 – 2938: Comparison of Burke-Fahn-Marsden dystonia severity, Gross Motor, Manual Abilities and Communication Function classification scales in childhood hyperkinetic movement disorders including cerebral palsy: a Rosetta Stone study

Objective The Burke-Fahn-Marsden Dystonia Rating Scale-Movement (BFM-M) evaluates dystonia impairment, but may not reflect functional ability in paediatric movement disorders (PMD). The Gross Motor Classification System (GMFCS), Manual Ability Classification System (MACS) and Communication Function Classification System (CFCS) are widely used in the cerebral palsy (CP) literature to classify functional ability. This study explores the concordance of the three functional scales in a large sample of PMD and the impact of dystonia severity on these functional scales. Methods Children with PMD (n=161, median age 10y3mo SD 4y) were assessed using the BFM-M, and classified using the 3 scales. Data analysis was performed using the R language and environment for statistical computing, version 3.01 RDev. Overall relationships between BFM-M and categorical variables were evaluated with the Kruskal-Wallis Rank Sum Test and the proportion variation explained (R b ). Relationships between two categorical variables were assessed using Pearson's chi-squared test and Pearson residuals. Spearman correlations (rs) were also provided as an estimate of the strength of the association between variables. The information provided by these scales is compared. Results Of the 161 children included, secondary dystonias (n=125, 78%) included those of both static (n=99, 61%), including dystonic/dyskinetic CP (n=75, 47%), and progressive (n=26, 16%) dystonia. All four scales were strongly associated (all rS>0.72, p Conclusion The BFM-M is strongly linked with the GMFCS, MACS and CFCS, irrespective of aetiology. PMD other than CP could be effectively evaluated using these scales. The ability of the BFM-M scale to discriminate between GMFCS levels in this sample offers the possibility to compare samples across different studies. However, the similarity between MACS I-III in terms of BFM-M scores indicates that non-motor components such as process skills and planning are required for functional activities and cannot be explained by dystonia alone.

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS-M). Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8-17 years), and the mean follow-up was 26.6 months (range 2-42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5-29.0) and 20.0 ± 5.5 (range 14.5-28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5-102.0) and 52.4 ± 21.5 (range 34.0-80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.

A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia

The authors' aim was to compare the subthalamic nucleus (STN) with the globus pallidus internus (GPi) as a stimulation target for deep brain stimulation (DBS) for medically refractory dystonia. In a prospective double-blind crossover study, electrodes were bilaterally implanted in the STN and GPi of 12 patients with focal, multifocal, or generalized dystonia. Each patient was randomly selected to undergo initial bilateral stimulation of either the STN or the GPi for 6 months, followed by bilateral stimulation of the other nucleus for another 6 months. Preoperative and postoperative ratings were assessed by using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and video recordings. Quality of life was evaluated by using questionnaires (36-item Short Form Health Survey). Supplemental Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were assessed for patients with focal dystonia (torticollis) by examining the video recordings. On average for all patients, DBS improved the BFMDRS movement scores (p < 0.05) and quality of life physical scores (p < 0.01). After stimulation of the STN, the mean 6-month improvement in BFMDRS movement score was 13.8 points; after stimulation of the GPi, this improvement was 9.1 points (p = 0.08). Quality of life did not differ significantly regardless of which nucleus was stimulated. All 12 patients accepted 6 months of stimulation of the STN, but only 7 accepted 6 months of stimulation of the GPi. Among those who rejected stimulation of the GPi, 3 accepted concomitant stimulation of both the STN and GPi for 6 months, resulting in improved quality of life physical and mental scores and BFMDRS movement scores. Among the 4 patients who were rated according to TWSTRS, after 6 months of stimulation of both the STN and GPi, TWSTRS scores improved by 4.7% after stimulation of the GPi and 50.8% after stimulation of the STN (p = 0.08). The STN seems to be a well-accepted, safe, and promising stimulation target in the treatment of dystonia, but further studies are necessary before the optimal target can be concluded. Simultaneous stimulation of the STN and GPi should be further investigated. Clinical trial registration no.: KF 01-110/01 (Committees on Biomedical Research Ethics of the Capital Region of Denmark).

Long-Term Benefit Sustained after Bilateral Pallidal Deep Brain Stimulation in Patients with Refractory Tardive Dystonia

Background/Aims: Tardive dystonia (TD) can be a highly disabling, permanent condition related to the use of dopamine-receptor-blocking medications. Our aim was to evaluate the long-term effect of bilateral pallidal deep brain stimulation (DBS) for TD. Methods: Five consecutive patients with disabling TD who underwent stereotactic placement of bilateral globus pallidus internus DBS leads were included. All patients had a history of mood disorder or schizophrenia previously treated with neuroleptic medication, with a mean duration of motor symptoms of 10.2 years. Dystonia severity was measured using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score by a blinded neurologist reviewing pre- and postoperative videotaped examinations. Results: The mean baseline movement BFMDRS score was 49.7 (range 20–88). Overall, we observed a mean reduction of 62% in the BFMDRS movement score within the first year after surgery. Persistent improvement in dystonia (71%) was seen at the last follow-up ranging from 2 to 8 years after surgery. Conclusion: Our experience suggests that pallidal DBS can be an effective therapy with long-term benefits for patients with TD.