{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T04:17:18Z","timestamp":1789705038955,"version":"4.0.1"},"reference-count":46,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T00:00:00Z","timestamp":1547164800000},"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 Disorders"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pantothenate kinase\u2010associated neurodegeneration is a rare autosomal\u2010recessive disorder, characterized by progressive neurodegeneration associated with brain iron accumulation. DBS has been trialed to treat related movement disorders, particularly dystonia. The objective of this study was to determine the outcome and safety of DBS for pantothenate kinase\u2010associated neurodegeneration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a meta\u2010analysis using independent participant data (n\u2009=\u200999) from 38 articles. Primary outcome was change in movement and disability scores of the Burke\u2010Fahn\u2010Marsden Dystonia Rating Scale 1 year postoperatively. Secondary outcomes were response rate and complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with classic\u2010type (n\u2009=\u200958) and atypical\u2010type (n\u2009=\u200915) pantothenate kinase\u2010associated neurodegeneration were operated on at a median age of 11 and 31 years, respectively (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). GPi was primarily targeted (n\u2009=\u200987). Mean dystonia movement score improved 1 year following GPi\u2010DBS (\u201026%; 95% confidence interval, \u201037% to \u201015%), particularly in atypical versus classic cases (\u201045% vs \u201016%; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). At least 30% improvement was observed in 34% of classic versus 73% of atypical cases (<jats:italic>P<\/jats:italic>\u2009=\u20090.04). Higher preoperative score and atypical type predicted larger improvement. GPi\u2010DBS improved dystonia disability score in atypical (\u201031%; 95% confidence interval, \u201049% to \u201013%) but not classic (\u20105%; 95% confidence interval, \u201017% to 8%) cases. Prevalence of surgical infections (6%) and hardware failure (7%) was similar to other dystonia etiologies. Two patients died within 3 months. There was insufficient data to describe outcome\u2009&gt;\u20091 year following GPi\u2010DBS or with other DBS targets. Overall, small sample sizes limited generalizability.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This meta\u2010analysis provides level 4 evidence that GPi\u2010DBS for pantothenate kinase\u2010associated neurodegeneration may improve dystonia movement scores in classic type and atypical type and disability scores in atypical type 1 year postoperatively. \u00a9 2019 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.27563","type":"journal-article","created":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T14:24:19Z","timestamp":1547216659000},"page":"264-273","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Deep brain stimulation for pantothenate kinase\u2010associated neurodegeneration: A meta\u2010analysis"],"prefix":"10.1002","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3280-6503","authenticated-orcid":false,"given":"Philippe","family":"De Vloo","sequence":"first","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery Toronto Western Hospital, University of Toronto  Toronto Ontario Canada"},{"name":"Department of Neurosurgery University Hospitals Leuven, KU Leuven  Leuven Belgium"},{"name":"Department of Neurosurgery Great Ormond Street Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darrin J.","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery Toronto Western Hospital, University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert F.","family":"Dallapiazza","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery Toronto Western Hospital, University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammad","family":"Rohani","sequence":"additional","affiliation":[{"name":"Division of Neurology Iran University of Medical Sciences  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfonso","family":"Fasano","sequence":"additional","affiliation":[{"name":"Edmond J. Safra Program in Parkinson's Disease Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, and Division of Neurology, Toronto Western Hospital University of Toronto  Toronto Ontario Canada"},{"name":"Krembil Brain Institute  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renato P.","family":"Munhoz","sequence":"additional","affiliation":[{"name":"Edmond J. Safra Program in Parkinson's Disease Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, and Division of Neurology, Toronto Western Hospital University of Toronto  Toronto Ontario Canada"},{"name":"Krembil Brain Institute  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George M.","family":"Ibrahim","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Neurosurgery, The Hospital for Sick Children University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mojgan","family":"Hodaie","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery Toronto Western Hospital, University of Toronto  Toronto Ontario Canada"},{"name":"Krembil Brain Institute  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andres 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