gms | German Medical Science

59th Annual Meeting of the German Society of Neurosurgery (DGNC)
3rd Joint Meeting with the Italian Neurosurgical Society (SINch)

German Society of Neurosurgery (DGNC)

1 - 4 June 2008, Würzburg

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Significant therapeutic effect of chronic Globus pallidus internus stimulation on dystonic dysphonia accompanying cervical dystonia

Signifikanter therapeutischer Effekt von Globus pallidus internus Stimulation auf dystone Dysphonie bei zervikaler Dystonie

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  • corresponding author J.H. Mehrkens - Neurochirurgische Universitätsklinik, Ludwig-Maximilians-Universität, München
  • K. Joussen - Praxis für Hals-Nasen-Ohren-Heilkunde, Stimm- und Sprachstörungen, München
  • K. Bötzel - Neurologische Universitätsklinik, Ludwig-Maximilians-Universität, München

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocDI.07.02

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/dgnc2008/08dgnc190.shtml

Published: May 30, 2008

© 2008 Mehrkens et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

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Objective: In dystonic dysphonia, which may accompany and aggravate facial and cervical dystonia, abnormal or dystonic muscle contractions can affect the muscles that close (adduction) or open (abduction) the laryngeal mechanism causing the voice to break or to have a tight, strained or strangled quality. Treatment usually consists of local Botolinum-Toxin-A (BTX-A) injections. We here present the immediate post-operative results and clinical follow for 2 adult patients undergoing Globus pallidus internus (Gpi) - deep brain stimulation (DBS) for cervical dystonia who also suffered from severely disabling dystonic dysphonia.

Methods: Both patients (pat. I: female, 56 yrs.; pat. II: male, 52 yrs.) were suffering from intractable cervical dystonia accompanied by severe spasmodic dysphonia that had been treated with moderate success by local BTX-A injections over an extended period of time. Electrode-implantation (DBS 3387, Medtronic) was performed under propofol anaesthesia with MRI-guided stereotaxy using a modified Leksell/Lerch system. Electrode position was verified by post-operative magnetic resonance imaging (MRI) and clinical outcome was assessed using the Tsui-score and phonetic analysis including digital-videolaryngostroboscopy.

Results: Post-op MRI confirmed the correct electrode position within the Gpi in both patients, with the lowest contact just above the optic tract (18 mm (pat. I) and 20 mm (pat II) lateral of the mid-commisure line). There was a significant improvement of the Tsui-score in both patients (reduction of 70% and 80%, at 10 and 22 months follow-up, respectively). Along with the improvement of the cervical dystonia, the speech-/vocal-abilities returned to near normal function without further BTX-A injections post-operatively: Both patients are able to speak for extended periods of time without exhaustion with patient I being able to give talks and patient II even having been able to return to his profession as a high-school teacher without any limitations.

Conclusions: Gpi deep brain stimulation has been shown to offer a very effective and safe therapy in patients suffering from facial and cervical dystonia. Despite contrarian reports in literature, there also seems to be a significant positive effect on accompanying dystonic dysphonia in selected patients.