Objective: We describe marked improvement of severe and complex adult onset idiopathic truncal dystonia, highlighting the challenging programing to achieve the best outcome.
Background: Few case reports and cases series have been reported the response of truncal dystonia to deep brain stimulation of Globus Pallidus (GPi-DBS).
Method: 46 years old (at surgery) female patient, presented by generalized dystonia, with prominent truncal dystonia. She had irrelevant past history and no family history of similar conditions. The dystonia started at right shoulder and arm, followed by trunk (on action), and progressed gradually to severe mobile complex axial dystonia within 1 year, composed of flexion, lateral and axial twisting (torsion) positions (The Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS)=67).
Results: The patient underwent bilateral GPi-DBS (Activa RC, Medtronic) in 2017, with intraoperative microelectrode monitoring. Proper localization of electrodes was confirmed by neuroimaging after surgery. The patient showed marked improvement 1 month after initial programing (left electrode (C+10-) 4V/60us/180hz & right (C+3-) 3.8V/60us/180hz), but with persistent axial twisting of the trunk (forward tilt of right shoulder and backward tilt of left shoulder (anti-clockwise)). Several approaches of programing were tried to improve this twist. Resolution of the twist was achieved by increasing amplitude and using more ventral contact of right electrode (ipsilateral to anteriorly tilted shoulder) (left electrode (C+10-) 3.9V/90us/130Hz & right (C+3-) 3.9V/90us/130hz)(BFMDRS=7). Total and truncal improvement of FBMDRS were 95.3% and 87.5% respectively. No side effects were reported after surgery or stimulation.
Conclusion: Programing of this case of complex adult onset truncal dystonia was challenging to ameliorate different abnormal positions. Increasing amplitude and changing contact of right electrode might improve anticlockwise rotation of the truncal dystonia, for further reports.
References: Picillo M, Lozano AM, Kou N, Munhoz RP, Fasano A. Programming deep brain stimulation for tremor and dystonia: the toronto western hospital algorithms. Brain Stimul. (2016) 9:438–52. doi: 10.1016/j.brs.2016.02.003 Volkmann J, Herzog J, Kopper F, Deuschl G. Introduction to the programming of deep brain stimulators. Mov Disord. (2002) 17(Suppl. 3):S181–7. doi: 10.1002/mds.10162
To cite this abstract in AMA style:
A. Shalash, Z. Fayed. Improved Severe Complex Idiopathic Adult Onset Truncal Dystonia with Axial Twisting by Bilateral Pallidal Stimulation: Case Report [abstract]. Mov Disord. 2020; 35 (suppl 1). https://www.mdsabstracts.org/abstract/improved-severe-complex-idiopathic-adult-onset-truncal-dystonia-with-axial-twisting-by-bilateral-pallidal-stimulation-case-report/. Accessed September 7, 2024.« Back to MDS Virtual Congress 2020
MDS Abstracts - https://www.mdsabstracts.org/abstract/improved-severe-complex-idiopathic-adult-onset-truncal-dystonia-with-axial-twisting-by-bilateral-pallidal-stimulation-case-report/