Reversal of Status Dystonicus after Relocation of Pallidal Electrodes in DYT6 Generalized Dystonia

D.L. Marinus Oterdoom; Martje E. van Egmond; Luisa Cassini Ascencao; J. Marc C. van Dijk; Assel Saryyeva; Martijn Beudel; Joachim Runge; Tom J. de Koning; Mahmoud Abdallat; Hendriekje Eggink; Marina A.J. Tijssen; Joachim K. Krauss

doi:10.5334/tohm.409

Figures & Tables

Table 1

BFMDRS Scores at Different Time Points

BFMDRS Scores	May 2014	June 2015	December 2015	January 2016	February 2016	October 2017
	Before 1st Surgery	1 Year after 1st Surgery	Status Dystonicus	Before 2nd Surgery	After 2nd Surgery	3 Years after 1st Surgery
Disability	26	14	29	30	27	15
Movement	71	69	90	108	73	64
Total	97	83	119	138	100	79

[i] The first deep brain stimulation implantation was in May 2015, the second in February 2016. For privacy reasons, the patient and his parents did not give permission to provide supplemental videos.

Schematic Depiction of the Electrode Positions. (A) Anterior coronal three-dimensional view of initial electrode positions (right 1, left 2) and electrode positions after second surgery (right 3, left 4). Note the position outside the right GPi (R) and barely inside the left GPi (L) of initial electrodes and the improved position after revision surgery. (B) Sagittal view from the right. (C) Sagittal view from the left. Note the improved position of 2 and 4 with at least two contacts within both internal parts of the globus pallidus (GPis). This is achieved by a more frontal burr hole facilitating a more oblique trajectory through the GPi. Anatomical structures and DBS electrodes were drawn into the patients using computed tomography and magnetic resonance imaging in SureTune2 software (Medtronic, MN, USA). R, GPi right; L, GPi left; OT, optic tract; 1, initial electrode right; 2, revised electrode right; 3, initial electrode left; 4, revised electrode left.

Table 2

Electrode Positions Relative to the Midcommisural Point

	X left	Y left	Z left	X right	Y right	Z right
First surgery	22.4¹	2.7	–2.9	22.6¹	3.1	–2.8
Second surgery	20	3	–4	20	3	–4

Target coordinates relative to anterior commissure - posterior commissure line midpoint in millimeters.

1 Realized lateral coordinate left 23.1 mm and right 24.4 mm.

Reversal of Status Dystonicus after Relocation of Pallidal Electrodes in DYT6 Generalized Dystonia

Figures & Tables

Table 1

BFMDRS Scores at Different Time Points

Figure 1

Table 2

Electrode Positions Relative to the Midcommisural Point

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