{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T20:31:27Z","timestamp":1789590687899,"version":"build-2803163510"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurol Surg A Cent Eur Neurosurg"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:p>\n            Introduction\u2003A 28-year-old man presented with a history of sensorineural deafness since early childhood treated with bilateral cochlear implants (CIs). He showed signs of debilitating dystonia that had been present since puberty. Dystonic symptoms, especially a protrusion of the tongue and bilateral hand tremor, had not responded to botulinum toxin therapy. We diagnosed Mohr-Tranebjaerg syndrome (MTS).<\/jats:p><jats:p>\n            Methods and Material\u2003Deep brain stimulation (DBS) of the bilateral globus pallidus internus was performed predominantly with stereotaxic computed tomography angiography guidance under general anesthesia. Electrophysiology was used to identify the target regions and to guide DBS electrode placement.<\/jats:p><jats:p>\n            Results\u2003In the immediate postoperative course and stimulation, the patient showed marked improvement of facial, extremity, and cervical dystonia. More than 2 years after implantation, his dystonic symptoms had dramatically improved by 82%.<\/jats:p><jats:p>\n            Discussion\u2003MTS is a rare genetic disorder leading to sensorineural deafness, dystonia, and other symptoms. The use of DBS for the dystonia in MTS was previously described but not in the presence of bilateral CIs.<\/jats:p><jats:p>\n            Conclusion\u2003DBS in MTS may be a viable option to treat debilitating dystonic symptoms. We describe successful DBS surgery, despite the presence of bilateral CIs, and stimulation therapy over 2 years.<\/jats:p>","DOI":"10.1055\/s-0038-1669472","type":"journal-article","created":{"date-parts":[[2018,10,5]],"date-time":"2018-10-05T19:16:08Z","timestamp":1538766968000},"page":"044-048","source":"Crossref","is-referenced-by-count":6,"title":["Bilateral Globus Pallidus Internus Deep Brain Stimulation in a Case of Progressive Dystonia in Mohr-Tranebjaerg Syndrome with Bilateral Cochlear Implants"],"prefix":"10.1055","volume":"80","author":[{"given":"Michel","family":"Rijntjes","sequence":"first","affiliation":[{"name":"Department of Neurology and Neurophysiology, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bastian","family":"Sajonz","sequence":"first","affiliation":[{"name":"Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Piroth","sequence":"first","affiliation":[{"name":"Department of Neurology and Neurophysiology, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Prokop","sequence":"first","affiliation":[{"name":"Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Jost","sequence":"first","affiliation":[{"name":"Parkinson-Klinik Wolfach, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Trippel","sequence":"first","affiliation":[{"name":"Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Horst","family":"Urbach","sequence":"first","affiliation":[{"name":"Department of Neuroradiology, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Reinacher","sequence":"first","affiliation":[{"name":"Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volker","family":"Coenen","sequence":"additional","affiliation":[{"name":"Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany"},{"name":"Medical Faculty, Freiburg University, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,10,5]]},"container-title":["Journal of Neurological Surgery Part A: Central European Neurosurgery"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0038-1669472.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,1,18]],"date-time":"2019-01-18T04:44:27Z","timestamp":1547786667000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0038-1669472"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10,5]]},"references-count":0,"journal-issue":{"issue":"01","published-online":{"date-parts":[[2019,1,10]]},"published-print":{"date-parts":[[2019,1]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0038-1669472","relation":{},"ISSN":["2193-6315","2193-6323"],"issn-type":[{"value":"2193-6315","type":"print"},{"value":"2193-6323","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10,5]]}}}