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This study presents data on the characteristics and treatment outcomes of a dystonia study population, analyzing their responses to various medical treatments before surgery and over a period of three to five years post-treatment. Key metrics include the use of medication like anticholinergics and botulinum toxin injections, along with scores from standardized assessments such as the Beck Depression Inventory and SF-36. Findings highlight significant changes in patient health status and quality of life over time, with particular emphasis on the role of medical interventions.

Reliability of the Burke-Fahn-Marsden scale in a multicenter trial for dystonia

Movement Disorders, 2007

Background: The multicenter SPIDY trial (pallidal stimulation for generalized, idiopathic dystonia) recently reported a marked improvement in dystonia which was assessed by the Burke-Fahn-Marsden (BFM) scale. However, the reliability of this tool has rarely been evaluated and its use in a multicenter study has never been assessed prospectively. Purpose: To evaluate the concordance between three unblinded clinical raters and one single-blinded rater for 10 prospective series of ratings on the BFM scale in 22 dystonic patients of the SPIDY study. Methods: Ten assessments on the BFM scale were performed under various stimulation conditions at different time points (before surgery and 1, 3, 6, and 12 months afterwards). Patients were first evaluated by three unblinded clinical raters (one per center). All assessments were videotaped and sent to a blinded rater. Intra-and inter-rater reliability was assessed using intraclass correlation coefficients. Results: The intra-rater reliability at inclusion was better for the blinded rater than for the clinical raters. The inter-rater reliability (comparing the blinded rater with each clinical rater) was "very good" at inclusion, "fair" at month 1 and was "good" at month 3, month 6, and month 12. Conclusion: Blinding (rather than video) is probably the key factor in better intra-rater reliability and can produce more accurate rating than clinical rating. Consequently, a blind procedure should be performed systematically in multicenter studies. As inter-rater reliability is good in trained unblinded raters, the BFM scale may also be used in the follow up of dystonic patients in movement disorders centers, in clinical practice.

Treatment of Dystonia Caused by Several Etiologies with DBS

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA

Introduction: This meta-analysis of reported cases of deep brain stimulation (DBS) for dystonia evaluates the effect using the globus pallidus internus (GPi) as target, and the factors that significantly have influenced the outcome related to the target. The Burke-Fahn-Marsden (BFM) movement scale, the most reported measure, was chosen as the primary outcome measure for this analysis. Material and Methods: MEDLINE searches on English literature identified 137 patients who underwent DBS for dystonia in 24 studies that had individual BFM scores. The study was done with statistical analysis by intention to treat. Statistical analysis was made with a significant p-value of 0.05. For the comparison of pre- and postoperative scores, a Wilcoxon signed-rank test was used. Results: The mean BFM percentage change (improvement in postoperative score from baseline) was 46.3% (range 34% to 100%). At last follow-up, disease severity and degree of disability and pain on the BFM were significantly ...

Table 2

We also made available the raw data of the miRNA profiling obtained from the prefrontal cortex of Alzheimer's patients. This will allow data re-analysis of the nCounter experiment shown in the EMM paper.

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