Jurnal Internasional Stimulasi otak dalam untuk neurodegenerasi terkait kinase-kinase: Sebuah meta-analisis

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Jurnal Internasional Stimulasi otak dalam untuk neurodegenerasi terkait kinase-kinase: Sebuah meta-analisis

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Abstract

Background

Neurodegeneration associated Pantothenate kinase kinases are rare autosomal recessive disorders characterized by degeneration of progressive neurodegenerations associated with brain iron accumulation. DBS has been tested to treat related movement disorders, especially dystonia. The aim of this study was to determine the outcome and safety of DBS for neurodegeneration related to pantothenate kinase.

Method

We conducted a meta-analysis using independent participant data (n = 99) from 38 articles. The main results were changes in movement and disability scores on the Dystonia Burke-Fahn-Marsden Rating Scale 1 year postoperatively. Secondary outcomes were response rates and complications.

Results

Patients with classic types (n = 58) and atypical types (n = 15) neurodegeneration related to pantothenate kinase were operated at an average age of 11 and 31 years. year, respectively ( P P P = 0.04). Higher preoperative scores and atypical types predict greater improvement. GPi-DBS increases the dystonia disability score in unusual cases (-31%; 95% confidence interval, -49% to -13%) but not classic (-5%; 95% confidence interval, -17% to 8%) case. The prevalence of surgical infections (6%) and hardware failure (7%) is similar to that of other dystonia. Two patients died within 3 months. There is not enough data to describe results> 1 year after GPi-DBS or with other DBS targets. Overall, small sample sizes are limited to generalization.

Conclusion

This meta-analysis provides level 4 evidence that GPi-DBS for neurodegeneration associated with pantothenate kinase can increase the classic type and atypical type movement score and atypical type defect score . 1 year postoperatively. © 2019 International Parkinson and Movement Disorder Society

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