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J Neurol Surg A Cent Eur Neurosurg 2019; 80(01): 044-048
DOI: 10.1055/s-0038-1669472
Surgical Technique
Georg Thieme Verlag KG Stuttgart · New York

Bilateral Globus Pallidus Internus Deep Brain Stimulation in a Case of Progressive Dystonia in Mohr-Tranebjaerg Syndrome with Bilateral Cochlear Implants

Volker Arnd Coenen
1   Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Michel Rijntjes
2   Department of Neurology and Neurophysiology, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Bastian Sajonz
1   Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Tobias Piroth
2   Department of Neurology and Neurophysiology, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Thomas Prokop
1   Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Wolfgang Jost
5   Parkinson-Klinik Wolfach, Germany
,
Michael Trippel
1   Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Horst Urbach
3   Department of Neuroradiology, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
,
Peter Christoph Reinacher
1   Department of Stereotactic and Functional Neurosurgery, University Hospital Freiburg, Freiburg, Germany
4   Medical Faculty, Freiburg University, Freiburg, Germany
› Author Affiliations
Further Information

Publication History

13 March 2018

03 May 2018

Publication Date:
05 October 2018 (online)

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Abstract

Introduction A 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).

Methods and Material Deep 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.

Results In 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%.

Discussion MTS 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.

Conclusion DBS 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.

Keywords

cochlear implant - deafness dystonia - deep brain stimulation - Mohr-Tranebjaerg syndrome
 

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