{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T11:57:15Z","timestamp":1779883035132,"version":"3.53.1"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,12,4]],"date-time":"2021-12-04T00:00:00Z","timestamp":1638576000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Early evidence suggests good response to pallidal deep brain stimulation (DBS) in DYT\u2010<jats:italic>KMT2B<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>We aimed to conduct a systematic review and meta\u2010analysis to assess outcomes and identify predictors of good outcome following GPi\u2010DBS in DYT\u2010<jats:italic>KMT2B<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We searched MEDLINE, Cochrane and MDS\u2010abstracts databases using the MeSH terms \u201c<jats:italic>KMT2B<\/jats:italic> and DYT28\u201d. We included studies that reported objective outcomes following GPi\u2010DBS in DYT\u2010<jats:italic>KMT2B<\/jats:italic>. The BFMDRS\u2010M (Burke\u2010Fahn\u2010Marsden Dystonia Rating Scale\u2010 Movement) total scores pre\u2010 and post\u2010surgery were used to quantify outcomes. We calculated pooled effects using a random effects meta\u2010analysis and used meta\u2010regression to identify potential effect modifiers. Multiple linear regression using individual patient data was used to identify predictors of good outcome (&gt;50% improvement from baseline on BFMDRS\u2010M).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Initial searches screened 132 abstracts of which 34 full\u2010text 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\u2009\u00b1\u20095.7\u2009years and 40% were male. The median follow\u2010up was 12\u00a0months (range: 1\u2013264\u00a0months). GPi\u2010DBS resulted in median BFMDRS\u2010M improvement of 42.7% (range: \u2212103.5% to 95.9%) postoperatively. Pooled proportion of patients experiencing clinical improvement &gt;50% on BFMDRS\u2010M was 41% (95% CI: 27%\u201357%). Male gender [\u03b2: 22.6, 95% CI: 8.0\u201337.3, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.004), and higher pre\u2010operative BFMDRS\u2010M score [\u03b2: 0.62, 95% CI: 0.36\u20130.87, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001) were independently associated with better outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p><jats:italic>KMT2B<\/jats:italic>\u2010associated dystonia responds effectively to pallidal stimulation. The outcome is better in males and those with more severe dystonia at baseline.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mdc3.13374","type":"journal-article","created":{"date-parts":[[2021,11,17]],"date-time":"2021-11-17T02:24:20Z","timestamp":1637115860000},"page":"31-37","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["<scp>GPi\u2010DBS<\/scp> for <scp><i>KMT2B<\/i><\/scp>\u2010Associated Dystonia: Systematic Review and Meta\u2010Analysis"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4987-7252","authenticated-orcid":false,"given":"Roopa","family":"Rajan","sequence":"first","affiliation":[{"name":"Department of Neurology All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1195-3558","authenticated-orcid":false,"given":"Kanwaljeet","family":"Garg","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5223-9206","authenticated-orcid":false,"given":"Arti","family":"Saini","sequence":"additional","affiliation":[{"name":"Department of Neurology All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0641-7398","authenticated-orcid":false,"given":"Divya M.","family":"Radhakrishnan","sequence":"additional","affiliation":[{"name":"Department of Neurology All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0755-5477","authenticated-orcid":false,"given":"Miryam","family":"Carecchio","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Department of Neuroscience University of Padua  Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Binukumar","family":"BK","sequence":"additional","affiliation":[{"name":"CSIR\u2010Institute of Genomics and Integrative Biology  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manmohan","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4590-7947","authenticated-orcid":false,"given":"Achal K.","family":"Srivastava","sequence":"additional","affiliation":[{"name":"Department of Neurology All India Institute of Medical Sciences (AIIMS)  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,12,4]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13255"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.4103\/0028-3886.302472"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00702-016-1656-9"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001312"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02410-7"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2010.207993"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10143-019-01233-x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2020.630391"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm8122163"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2019-322169"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11910-019-1007-y"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3740"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.27026"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.28485"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awaa304"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.12862"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.27771"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s10038-019-0625-1"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10377"},{"key":"e_1_2_6_21_1","unstructured":"R: The R Project for Statistical Computing [Internet]. 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