gms | German Medical Science

72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

06.06. - 09.06.2021

Magnetic resonance imaging-guided focused ultrasound thalamotomy in spinocerebellar ataxia 12 associated tremor – a case report

Thalamotomie mittels Magnetresonanztomographie-geführtem fokussierten Ultraschall bei mit der Spinocerebellären Ataxie 12 assoziiertem Tremor – ein Case Report

Meeting Abstract

  • presenting/speaker Valeri Borger - Universitätsklinikum Bonn, Klinik und Poliklinik für Neurochirurgie, Bonn, Deutschland
  • Veronika Purrer - Universitätsklinikum Bonn, Klinik und Poliklinik für Neurologie, Bonn, Deutschland; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Bonn, Deutschland
  • Neeraj Upadhyay - Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Bonn, Deutschland
  • Claus Pieper - Universitätsklinikum Bonn, Klinik für Diagnostische und Interventionelle Radiologie, Bonn, Deutschland
  • Hartmut Vatter - Universitätsklinikum Bonn, Klinik und Poliklinik für Neurochirurgie, Bonn, Deutschland
  • Jaroslaw Maciaczyk - Universitätsklinikum Bonn, Klinik und Poliklinik für Neurochirurgie, Bonn, Deutschland
  • Ullrich Wüllner - Universitätsklinikum Bonn, Klinik und Poliklinik für Neurologie, Bonn, Deutschland; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Bonn, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie. sine loco [digital], 06.-09.06.2021. Düsseldorf: German Medical Science GMS Publishing House; 2021. DocP019

doi: 10.3205/21dgnc307, urn:nbn:de:0183-21dgnc3071

Published: June 4, 2021

© 2021 Borger et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

Objective: Spinocerebellar ataxias (SCAs) are progressive, neurodegenerative disorders generally associated with oculomotor dysfunction, dysarthria and ataxia. The SCA12 is typically associated with action tremor of the upper extremity. The treatment of SCA12 is challenging. Single case series have previously demonstrated the effective use of deep brain stimulation (DBS) for the treatment of tremor in SCAs. Beside DBS, MRI-guided focused ultrasound (MRgFUS) is a promising technique to ablate deep brain targets such as ventrointermedius nucleus (ViM) or cerebello-thalamic tract (CTT) resulting in significant tremor reduction in essential tremor (ET) and Parkinson’s disease (PD). However, to our knowledge no patient with SCA has been treated with MRgFUS so far. Herein, we report on a patient suffering from SCA12 with severe action tremor and his outcome after lesioning of the CTT using MRgFUS.

Methods: A 65-year-old man presented with severe bilateral action tremor of the upper extremity accompanied by a moderate head and voice tremor. Therapeutic strategies, including propranolol, primidone, topiramate and cannabinoids showed either little benefit or had to be stopped due to side effects. In 2014, a genetic analysis confirmed the diagnosis of SCA12. The clinical evaluation regarding the Scale for the Assessment and Rating for Ataxia (SARA) revealed a score of 18,5/40 and on the Fahn-Tolosa-Marin Tremor Scale (TRS) 90/260. The aim was to relieve the severely disabling tremor. Therefore, we performed unilateral lesioning of CTT with MRgFUS using coordinates based on tractography of CTT.

Results: A total of 5 ablative sonications (peak temperature above 56°C) at two different locations (1st target: 1 mm superior to AC-PC line, 14 mm lateral to midline, 25% of the AC-PC line anterior to PC; 2nd target: movement of 0.5mm laterally and 0.5mm anteriorly) were performed. During the treatment a nearly total tremor reduction in the right arm was observed, side effects did not occur. SARA score was 24/40 after 3 days and improved to 17/40 after 3 months. TRS score improved to 51/260 after 3 days and 48/260 after 3 months.

Conclusion: Our experience shows that unilateral lesioning of CTT using MRgFUS might also be effective and safe in terms of tremor reduction in patients with SCA. However, the safety and efficacy of MRgFUS in ET and other tremor conditions as well as the clinical value of diffusion tensor imaging for target determination within the thalamus needs to be further investigated.