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|Year : 2017 | Volume
| Issue : 1 | Page : 99-102
“I swear, I can't stop it!” – A case of severe Tourette's syndrome treated with deep brain stimulation of anteromedial globus pallidus interna
S Dwarakanath1, A Hegde2, J Ketan3, P Chandrajit4, R Yadav3, K Keshav5, S Sampath1, PK Pal3, Y C J Reddy2
1 Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
3 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
4 Department of Neuroradiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
5 Department of Clinical Psychology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
|Date of Web Publication||12-Jan-2017|
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
Introduction: Tourette's syndrome (TS) is a complex neuropsychiatric disorder characterized by the presence of multiple motor and vocal tics. Here, we report the case of a young man with severe TS refractory to multiple medications who underwent deep brain stimulation (DBS), which was successful in substantially ameliorating his tics. To our knowledge, this is the first such report from India and South Asia.
Case Report: An 18-year-old right-handed male patient was diagnosed with TS at the age of 10 years. He had facial and ocular tics. He was also hitting his fist against his chest and shouting obscenities. The manifestations would be present in every waking hour with a maximum tic free interval of 15–20 minutes. They would often result in self-injury or damage to objects. He would have frequent crying spells, anger outbursts, and death wishes. As tics became highly conspicuous and socially inappropriate, he dropped out of school and remained almost completely house-bound for the preceding year. On evaluation, he scored 96 (46 on tic-severity subscale and 50 on impairment subscale) of a maximum of 100 on the Yale Global Tic Severity Scale. (YGTSS).
Management: After failure of multiple combinations of medicines, repetitive transcranial magnetic stimulation, and behavioural therapies, he successfully underwent DBS to bilateral anteromedial globus pallidus interna.
Conclusion: Tic severity reduced substantially post-surgery, with the YGTSS score improving by more than 72%. These improvements were sustained on follow-up visits with the patient successfully returning to join college. To our knowledge, this is the first such report from India and South Asia.
Keywords: Anteromedial globus pallidus interna, deep brain stimulation (DBS), Tourette's syndrome Yale global tic severity scale
The first case in India/South Asia that demonstrates that anteromedial globus pallidus interna can be an useful target for deep brain stimulation in the case of Tourette′s syndrome.
|How to cite this article:|
Dwarakanath S, Hegde A, Ketan J, Chandrajit P, Yadav R, Keshav K, Sampath S, Pal P K, Reddy Y. “I swear, I can't stop it!” – A case of severe Tourette's syndrome treated with deep brain stimulation of anteromedial globus pallidus interna. Neurol India 2017;65:99-102
|How to cite this URL:|
Dwarakanath S, Hegde A, Ketan J, Chandrajit P, Yadav R, Keshav K, Sampath S, Pal P K, Reddy Y. “I swear, I can't stop it!” – A case of severe Tourette's syndrome treated with deep brain stimulation of anteromedial globus pallidus interna. Neurol India [serial online] 2017 [cited 2017 Sep 24];65:99-102. Available from: http://www.neurologyindia.com/text.asp?2017/65/1/99/198188
Tourette's syndrome (TS) is a complex neuropsychiatric disorder characterized by the presence of multiple motor and vocal tics. Behavioral therapies and medications are effective for many patients with TS. However, symptoms may persist and cause severe debility in a subset of patients despite these treatments. Repetitive transcranial magnetic stimulation (rTMS) and deep brain stimulation (DBS) have been shown to be promising treatment options in this group of patients., Here, we report the case of a young man with severe TS refractory to multiple medications, behavioural treatments, and rTMS, who underwent DBS to bilateral anteromedial globus pallidus interna (AM-GPi), which was successful in substantially ameliorating his tics. To our knowledge, this is the first such report from India and South Asia.
| » Case Report|| |
Our patient, an 18-year old right-handed male, was born of a full-term normal delivery, and had attained age appropriate developmental milestones. Early in his childhood, hyperactivity, inattention, and impulsivity were notable, although the family had not sought formal treatment, thinking that he would grow out of them. He came to us with complaints of sudden, repeated body movements and utterances since the age of 10 years. The onset was insidious, with the symptoms following a fluctuating, but gradually worsening course. It had started with the patient making repeated sounds as if he was clearing his throat. Over the years, he developed repetitive blinking of eyes, facial grimacing, broadening of nostrils, and jerky movements (flexion followed by extension) of the neck, elbow, and ankle joints. He was hitting his fists against his chest, constricting his anal sphincter, grunting, and shouting obscene words. Common to all these occurrences, he described an uncontrollable urge which would “force” him to perform these acts. Their frequency and intensity tended to increase in situ ations which provoked anxiety. The conditon also got aggravated following physical exertion but slightly decreased when he was calm, immersed in computer/mobile-phone games (which he enjoyed), and disappeared during sleep. Diagnosed with TS, he was treated with multiple medications [Table 1] but had no improvement. The tics would be present in every waking hour of each day, with a maximum tic free interval of 15–20 minutes. Often, the tics would result in self-injury or damage to objects. He would have frequent crying spells, anger outbursts, and death wishes. As tics became highly conspicuous and socially inappropriate, he dropped out of school and remained almost completely house-bound for the preceding year.
On evaluation, he scored 96 (46 on tic-severity subscale and 50 on impairment subscale) out of a maximum of 100 on the Yale Global Tic Severity Scale (YGTSS) [Table 2]. Having no improvement with first-line treatments, rTMS was attempted with no significant benefit. Six months after discharge, despite medications, he continued to be severely ill, now rating 98 on the YGTSS [Figure 1]. Meeting the selection criteria for DBS, discussion was carried out with the patient and family on the available evidence, associated pros and cons, and cost of the procedure. Informed written consent was obtained from both the patient and his parents. The required pre-surgical assessments were carried out [Table 3], Video 1 (Preoperative Video)].
Surgical procedure and stimulation calibration
Magnetic resonance imaging (MRI) was then performed 1 day prior to the procedure using the 3T Achieva, Philips Medical Systems, Best, Netherlands. Both T1-weighted [three-dimensional (3D) magnetization-prepared rapid gradient-echo (MP-RAGE) sequence with 1 mm slice thickness] and T2-weighted images (2-mm slice thickness) were obtained. The images were transferred to the Medtronic Framelink (R) planning system, and preplanning was performed. On the day of the surgery, frame fixation using the Leksell G stereotactic frame (Elekta Instruments, Stockholm, Sweden) was performed under general anesthesia. MRI was performed on the 1.5-T scanner; Siemens Aera, Erlangen, Germany). T1-weighted images (3D MP-RAGE sequence with 1 mm slice thickness) were obtained. The images were transferred to the Medtronic Framelink (R) planning system and fused with the 3-T images and the planning was completed. The anteromedial region of globus pallidus internus (AM-GPi) was targeted based on visual anatomy, as described by Ashwin et al. The final coordinates were as follows; left side (x = −12.84, y = 5.73, and z = 7.28 from the mid-commissural point), right side (x = 14.25, y = 6, and z = −6.34 from the mid-commissural point). Both the procedures were performed under general anesthesia because the severity of tics did not permit surgery under local anesthesia. Postoperatively, the patient recovered uneventfully. We performed a postoperative computed tomography (CT) scan to check for the accuracy of the electrode position. The programming parameters used were 3.5 V at 60 µs and 180 Hz.
Complete clinical and neuropsychological evaluation was repeated at follow-up visit and are detailed in [Table 2], Video 2 (Postoperative Video). At 6 months postoperatively, his functioning considerably improved; it was observed that his hyperactivity, inattention, and impulsivity were causing substantial difficulties. Tab chlorpromazine was cross-tapered, and tab amisulpiride 400 mg, and tab atomoxetine up to 50 mg/day were added. At follow-up visit 9 months postoperatively, tic severity was even lesser, although the previously mentioned behavioural problems continued to be severe. Tab fluoxetine 40 mg/day was added to the existing medication regimen for the behavioural symptoms.
| » Discussion|| |
It has been postulated that the basal ganglia-thalamocortical circuits play a role in the generation of tics and obsessive compulsive disorders (OCDs). Thus, a variety of targets have been used, the main being the centromedian-parafascicular–ventrooralis complex (CM-Pf-VO) of the thalamus and the GPi (posteroventral (PV-GPi) segment or the anteromedial (AM-GPi) segment). The CM-Pf complex and the AM-GPi have been the most popular choices as the targets during DBS. While the CM-Pf complex is able to modulate both the sensorimotor and the limbic circuitry, the AM-GPi may be able to target both the motor and comorbid symptoms of TS because it is the critical relay station for translation of limbic signals to motor output. DBS for Tourette's syndrome was first performed in 1999, and since then atleast 135 patients have been operated in 23 centers in 13 countries worldwide., While the introductory guidelines were first published in 2005, a revised set of guidelines were published in 2015; the main difference between the two being the removal of age criteria. A variety of targets (7 in all) have been described, the most common being the CM-Pf complex in 70 cases, GPi in 46 cases (both the PV-GPi and AM-GPi), and the anterior limb of the internal capsule-nucleus accumbens in 6 cases. Servello et al., reported a series of 31 cases of DBS in Gilles de la Tourette syndrome mainly targeting the CM-Pfc-VOa complex. They had an average of 47% improvement in the YGTSS score, 17.3% improvement in the Yale–Brown Obsessive Compulsive Scale (Y-BOCS), and a 30.7 improvement in the Visual Analog Scale (VAS) for self-evaluation of the impact of TS on social integration of the patient. Porta et al., in a series of 18 cases (Target – CM-Pfc-Voa complex), reported a 73% mean improvement in the YGTSS and 42% improvement in the YBOCS score. Ackerman et al., in a series of 8 cases, reported an average improvement of 49.3% in the YGTSS and no improvement on the YBOCS scale. Martinez- Fernandez et al., in a series of 6 cases targeting the GPi (3 PV-GPi and 3 AM-GPi), reported a 29% improvement in the YGTSS, 26% improvement in YBOCS, and a 40% improvement in the VAS-QOL scale. They also concluded that patients who received AM-GPi DBS had a better response when compared with patients who received PV-GPi DBS (38% vs. 20% on the YGTSS). Sachdev et al., in a series of 17 patients targeting the AM-GPi, reported a 48.3% reduction in motor tics and a 41.3% reduction in phonic tics. Based on the above mentioned literature and the expertise available, we preferred the AM-GPi as the target in our patient.
| » Conclusion|| |
The improvements were sustained up to 9 months post-surgery and no major adverse effects were noted. The report supports the notion that DBS may be considered a safe and effective treatment for tics. A rigorous selection process, experience with DBS, as well as a comprehensive baseline and follow-up assessment are essential for successful outcomes. To our knowledge, this is the first report of TS being successfully treated with DBS in India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]