{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T02:18:04Z","timestamp":1790993884181,"version":"4.1.0"},"reference-count":38,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,10,9]],"date-time":"2018-10-09T00:00:00Z","timestamp":1539043200000},"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":[[2019,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Good short\u2010term results of pallidal deep brain stimulation have been reported in myoclonus\u2010dystonia. Efficacy and safety in the long term remain to be established. In addition, the actual impact of DBS treatment on social inclusion is unknown. The objective of this study was to assess the long\u2010term clinical outcome, quality of life, and social adjustment of GPi\u2010DBS in patients with \u03b5\u2010sarcoglycan (DYT11)\u2010positive myoclonus\u2010dystonia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Consecutive myoclonus\u2010dystonia patients with \u03b5\u2010sarcoglycan mutations who underwent GPi\u2010DBS were evaluated at least 5 years postoperatively. Motor symptoms were assessed using the Burke\u2010Fahn\u2010Marsden Dystonia Rating Scale including the Disability Scale, a composite score combining the rest and action parts of the Unified Myoclonus Rating Scale and modified Abnormal Involuntary Movement Scale. Standardized video\u2010protocols were assessed by a blinded and external movement disorder specialist. Social adjustment, cognition, and mood were evaluated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Nine patients (5 women) with long\u2010term GPi\u2010DBS (8.7\u2009\u00b1\u20093.1 years) were included. There was significant improvement in the composite myoclonus score (94.1%\u2009\u00b1\u20094% improvement;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.008). Dystonia severity was also markedly improved (71.4%\u2009\u00b1\u200928.33% improvement;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.008) as well as motor disability (88.3%\u2009\u00b1\u200920% improvement;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.008) and abnormal involuntary movement score (71.1%\u2009\u00b1\u200915.0% improvement;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.008). No patients experienced postoperative speech or gait problems or any permanent adverse effects. Eight of the 9 patients had fully enhanced social adjustment and personal achievement, with little or no mood or behavioral disorders.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>GPi\u2010DBS seems to be a safe and efficacious treatment for medically refractory \u025b\u2010sarcoglycan myoclonus\u2010dystonia, with sustained motor benefit, good quality of life, and social adjustment in long\u2010term follow\u2010up. \u00a9 2018 International Parkinson and Movement Disorder Society<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mds.27474","type":"journal-article","created":{"date-parts":[[2018,10,10]],"date-time":"2018-10-10T01:01:17Z","timestamp":1539133277000},"page":"87-94","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":63,"title":["Long\u2010term GPi\u2010DBS improves motor features in myoclonus\u2010dystonia and enhances social adjustment"],"prefix":"10.1002","volume":"34","author":[{"given":"Zuzana","family":"Kosutzka","sequence":"first","affiliation":[{"name":"Sorbonne Universit\u00e9, Facult\u00e9 de M\u00e9decine; CNRS UMR 7225, UMR S 1127 Institut du Cerveau et de la Moelle \u00e9pini\u00e8re  Paris France"},{"name":"Second Department of Neurology, Faculty of Medicine Comenius University  Bratislava Slovakia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Tisch","sequence":"additional","affiliation":[{"name":"Department of Neurology, St Vincent's Hospital University of New South Wales Sydney  Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Bonnet","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, Facult\u00e9 de M\u00e9decine; CNRS UMR 7225, UMR S 1127 Institut du Cerveau et de la Moelle \u00e9pini\u00e8re  Paris France"},{"name":"APHP, H\u00f4pital Salp\u00eatri\u00e8re D\u00e9partement de Neurologie  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ruiz","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, Facult\u00e9 de M\u00e9decine; CNRS UMR 7225, UMR S 1127 Institut du Cerveau et de la Moelle \u00e9pini\u00e8re  Paris France"},{"name":"APHP, H\u00f4pital Salp\u00eatri\u00e8re D\u00e9partement de Neurologie  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elodie","family":"Hainque","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, Facult\u00e9 de M\u00e9decine; CNRS UMR 7225, UMR S 1127 Institut du Cerveau et de la Moelle \u00e9pini\u00e8re  Paris France"},{"name":"APHP, H\u00f4pital Salp\u00eatri\u00e8re D\u00e9partement de Neurologie  Paris 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