A Case of Deep Brain Stimulation for Tourette's Complicated by Twiddler's Syndrome

Gilles de la Tourette's Syndrome (TS) is defined by the presence of motor and phonic tics.1 Most cases can be managed medically, but a small percentage of cases are so severe that they cause significant disability despite all attempts to achieve medical optimization. For these cases, DBS has been performed with varying results.2 We review a case of thalamic DBS for severe TS in an adolescent that resulted in significant improvement, but was complicated by compulsive twiddling of the generator that ultimately necessitated removal of the right stimulator. The patient experienced reemergence of tics predominantly on the nonstimulated left body, which improved after reimplantation of electrodes.

Case Report

A 16‐year‐old woman with TS presented for consideration of DBS for severe, treatment‐refractory motor tics, including head banging resulting in concussions and forceful neck and shoulder jerking leading to numerous bilateral shoulder dislocations. She underwent simultaneous bilateral DBS of the medial thalamus (ventral oral internus, centro‐median/parafascicular nuclei), as described elsewhere,3, 4 and experienced dramatic improvement in her motor and phonic tics (see Table 1). Ten months postoperatively, she developed acute worsening of tics after repetitive rotation of the right implantable pulse generator (IPG). Impedances were abnormal and a chest X‐ray revealed a fractured extension lead (see Fig. 1). An infection of the replaced IPG necessitated complete removal of the right‐sided system. During the 3‐month period with only left thalamic stimulation, she experienced a worsening of motor tics, predominantly on the nonstimulated side of her body, comprised of chorea‐like and hemiballistic flinging movements of the left arm and leg as well as forceful shoulder rotation that resulted in dislocation. After a course of antibiotics, the right stimulator was replaced with the IPG moved to the abdomen to reduce the risk of manipulation. She experienced subsequent improvement, approaching her previous bilateral DBS level (see Table 1; Video 1).

Table 1.

Pre‐ and post‐DBS outcomes including pre‐ and postrevision scores

	Yale Global Tic Severity Scale				YBOCS	Medications	Settingsa
	Total	Motor	Phonic	Impairment
Pre‐DBS	88	24	19	45	17	Pimozide 4 mg, clomipramine 175 mg, diazepam 4 mg	N/A
9‐month DBS	40	13	12	15	14	Pimozide 2.5 mg, clomipramine 150 mg, atomoxetine 80 mg	L IPG C+2 – 3.0/90/130 R IPG C+2 – 2.0/90/130
Unilateral DBS	67	16	11	40	17	No change	L IPG C+2 – 4.0/90/130
Post‐revision 14‐month DBS	45	15	10	20	11	No change	L IPG C+2 – 4.0/90/130 R IPG C+2 – 2.7/90/130

^aL/R IPG, left/right implantable pulse generator. C, case and denotes monopolar stimulation with the IPG as the anode. Stimulation parameters are listed as polarity followed by amplitude in voltage/pulse width in microseconds/frequency in Hertz.

YBOCS, Yale‐Brown Obsessive Compulsive Scale.

Figure 1.

Radiograph demonstrating a flipped right implantable pulse generator with fractured distal extension lead.

Discussion

DBS remains an experimental, but promising, intervention for the management of severe, treatment‐refractory TS. This case underscores the potential for significant tic improvement, but also the potential for residual obsessive‐compulsive behaviors to undermine that improvement. Intentional or unintentional flipping of IPGs has been described in both cardiac and DBS patients.5, 6 This is, to our knowledge, the first report of “twiddler's syndrome” in a TS patient. We speculate that patients with TS and obsessive‐compulsive disorder may be at higher risk for this syndrome. Placement of the IPG in the abdomen should be considered in such instances given that the generator may be less prone to movement and manipulation in patients with prominent shoulder and neck tics. This is also the second report of hemi tics after removal of unilateral DBS in TS,7 but the first involving DBS targeting the thalamus. The presence of asymmetric tics after removal of the contralateral stimulator in 2 cases further supports lateralizing dysfunction in TS.

Author Roles

(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the First Draft, B. Review and Critique.

M.H.P.: 1A, 1B, 3A

C.L.B.: 3B

A.Y.M.: 1B, 3B

Disclosures

Funding Sources and Conflicts of Interest: The authors report no sources of funding and no conflicts of interest.

Financial Disclosures for previous 12 months: M.P. has received teaching honoraria from Medtronic, Inc., Teva Pharmaceuticals, and US World Meds as well as consultation compensation from St. Jude Medical, Inc., and grant support from Boston Scientific, Inc. A.M. has received consultation compensation from Medtronic, Inc., and St. Jude Medical, Inc., and has received grant support from Boston Scientific, Inc., Medtronic, Inc., and St. Jude Medical, Inc. C.B. has received research funding from Otsuka Pharmaceuticals.

Supporting information

A video accompanying this article is available in the supporting information here.

Video 1. Emergence of hemi tics after removal of contralateral deep brain stimulator with subsequent improvement after reimplantation.