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An underlying monogenic etiology is increasingly recognized as an important predictor of DBS outcomes. Moreover, the genetic background of dystonia is continuously expanding, posing new challenges in the tailored counseling of patients regarding advanced therapies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>To improve the quality of available evidence on the efficacy of DBS for treating monogenic dystonia, we conducted a systematic review in accordance with PRISMA guidelines. We applied a rigorous methodology and maximized the amount of information provided by including all patients, regardless of age or applied rating scale.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Our findings confirm the high probability of a good DBS outcome in patients harboring\n                      <jats:italic>TOR1A, SGCE, PANK2<\/jats:italic>\n                      , and\n                      <jats:italic>TAF1<\/jats:italic>\n                      variants. An intermediate response was associated with\n                      <jats:italic>KMT2B<\/jats:italic>\n                      and\n                      <jats:italic>THAP1<\/jats:italic>\n                      variants. A particularly favorable outcome with &gt;\u200980% improvement in dystonia symptoms was associated with a subset of DYT\u2010\n                      <jats:italic>TOR1A<\/jats:italic>\n                      patients and few cases with\n                      <jats:italic>SGCE\u2010, KMT2B\u2010, THAP1<\/jats:italic>\n                      \u2010,\n                      <jats:italic>GNAO1<\/jats:italic>\n                      \u2010, and\n                      <jats:italic>TAF1<\/jats:italic>\n                      \u2010related disease. Poor study quality, non\u2010systematic assessment of DBS response, and pooling of patients with different genetic etiologies were among the encountered limitations.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Based on the collected evidence, we formulated recommendations for applying DBS in monogenic dystonia. Our findings, together with the cumulative literature, advocate the introduction of genetic testing in the pre\u2010DBS work\u2010up. They furthermore highlight the need to implement and report on systematic assessments of DBS outcomes, including mandatory patient\u2010reported outcomes. These steps will ensure optimal counseling and continuous improvement in the care of patients with monogenic dystonia.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.70490","type":"journal-article","created":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T12:37:11Z","timestamp":1768567031000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Efficacy of Deep Brain Stimulation for the Treatment of Monogenic Dystonia Symptoms: A Systematic Review"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0217-8630","authenticated-orcid":false,"given":"Elisabetta","family":"Indelicato","sequence":"first","affiliation":[{"name":"Center for Rare Movement Disorders Innsbruck Medical University Innsbruck  Innsbruck 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