Tourette syndrome (TS) is a childhood-onset neuropsychiatric disorder characterized by the presence of multiple motor and vocal tics. TS usually co-occurs with one or multiple psychiatric disorders. Although behavioral and pharmacological treatments for TS are available, some patients do not respond to the available treatments. For these patients, TS is a severe, chronic, and disabling disorder. In recent years, deep brain stimulation (DBS) of basal ganglia-thalamocortical networks has emerged as a promising intervention for refractory TS with or without psychiatric comorbidities. Three major challenges need to be addressed to move the field of DBS treatment for TS forward: (1) patient and DBS target selection, (2) ethical concerns with treating pediatric patients, and (3) DBS treatment optimization and improvement of individual patient outcomes (motor and phonic tics, as well as functioning and quality of life). The Tourette Association of America and the American Academy of Neurology have recently released their recommendations regarding surgical treatment for refractory TS. Here, we describe the challenges, advancements, and promises of the use of DBS in the treatment of TS. We summarize the results of clinical studies and discuss the ethical issues involved in treating pediatric patients. Our aim is to provide a better understanding of the feasibility, safety, selection process, and clinical effectiveness of DBS treatment for select cases of severe and medically intractable TS.
ABSTRACT:Objective:To evaluate the short-term and long-term clinical effectiveness and safety of subthalamic nucleus deep brain stimulation (STN-DBS) for medically intractable pediatric isolated dystonia.Methods:Using a longitudinal retrospective design, we assessed the clinical outcomes of nine patients who underwent STN-DBS for treatment-refractory pediatric isolated dystonia one decade ago (mean age at surgery: 15.9 ± 4.5 years). The primary clinical outcome used was assessed by retrospective video analyses of patients’ dystonia symptoms using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Clinical assessments were performed at baseline, 1-year follow-up (1-yr FU), and 10-year follow-up (10-yr FU). Adverse side effects, including surgery-related, device-related, and stimulation-related effects, were also documented.Results:After STN-DBS surgery, the mean improvement in the BFMDRS motor score was 77.1 ± 26.6% at 1-yr FU and 90.4 ± 10.4% at 10-yr FU. Similarly, the mean BFMDRS disability score was improved by 69.5 ± 13.6% at 1-yr FU and by 86.5 ± 13.9% at 10-yr FU. The clinical improvements gained at 10-yr FU were significantly larger than those observed at 1-yr FU. Negative correlations were found between the duration of disease to age at surgery ratio (DD/AS) and the improvements in the BFMDRS motor score and total score at 1-yr FU and 10-yr FU.Conclusion:To our knowledge, this study provides the first clinical evidence for the short- and long-term effectiveness and safety of STN-DBS for pediatric isolated dystonia. Additionally, putative evidence is provided that earlier STN-DBS intervention in patients with refractory pediatric isolated dystonia may improve short- and long-term clinical outcomes.
Background: Deep brain stimulation (DBS) is an established treatment for hyperkinetic movement disorders. Patients undergoing DBS can choose between the use of a rechargeable or non-rechargeable battery for implanted pulse generators (IPG).Objectives: In this study, we aimed to evaluate patient preferences and satisfaction with rechargeable and non-rechargeable batteries for IPGs after undergoing DBS.Methods: Overall, 100 patients with hyperkinetic movement disorders (dystonia: 79, Tourette syndrome: 21) who had undergone DBS took a self-designed questionnaire to assess their satisfaction and experience with the type of battery they had chosen and the factors influencing their choice.Results: Of the participants, 87% were satisfied with the stimulating effects of the treatment as well as the implanted device; 76% had chosen rechargeable devices (r-IPGs), 71.4% of whom recharged the battery themselves. Economic factors were the main reason for choosing both r-IPG and non-rechargeable IPG (nr-IPG). The questionnaire revealed that 66% of the patients checked their r-IPG battery every week. The mean interval for battery recharge was 4.3 days.Conclusions: The majority of the patients were satisfied with their in-service-IPG, regardless of whether it was a r-IPG or nr-IPG. Affordability was the main factor influencing the choice of IPG. The majority of the patients were confident in recharging the battery of their r-IPG themselves; only 11% of patients experienced difficulties. Understanding the recharge process remains difficult for some patients and increasing the number of training sessions for the device may be helpful.
Background Deep brain stimulation (DBS) is a promising novel approach for managing refractory Gilles de la Tourette’s syndrome (GTS). The subthalamic nucleus (STN) is the most common DBS target for treating movement disorders, and smaller case studies have reported the efficacy of bilateral STN-DBS treatment for relieving tic symptoms. However, management of GTS and treatment mechanism of STN-DBS in GTS remain to be elucidated. Methods Ten patients undergoing STN-DBS were included. Tics severity was evaluated using the Yale Global Tic Severity Scale. The severities of comorbid psychiatric symptoms of obsessive–compulsive behavior (OCB), attention-deficit/hyperactivity disorder, anxiety, and depression; social and occupational functioning; and quality of life were assessed. Volumes of tissue activated were used as seed points for functional connectivity analysis performed using a control dataset. Results The overall tics severity significantly reduced, with 62.9% ± 26.2% and 58.8% ± 27.2% improvements at the 6- and 12-months follow-up, respectively. All three patients with comorbid OCB showed improvement in their OCB symptoms at both the follow-ups. STN-DBS treatment was reasonably well tolerated by the patients with GTS. The most commonly reported side effect was light dysarthria. The stimulation effect of STN-DBS might regulate these symptoms through functional connectivity with the thalamus, pallidum, substantia nigra pars reticulata, putamen, insula, and anterior cingulate cortices. Conclusions STN-DBS was associated with symptomatic improvement in severe and refractory GTS without significant adverse events. The STN is a promising DBS target by stimulating both sensorimotor and limbic subregions, and specific brain area doses affect treatment outcomes.
<b><i>Introduction:</i></b> Globus pallidus internus (GPi) deep brain stimulation (DBS) combined with anterior capsulotomy offers a promising treatment option for severe medication-refractory cases of Tourette’s syndrome (TS) with psychiatric comorbidities. Several patients treated with this combined surgery experienced sustained relief after discontinuation of stimulation over the course of treatment. <b><i>Methods:</i></b> Retrospectively, the medical records and clinical outcomes were reviewed of 8 patients (6 men; 2 women with mean age of 20.3 years) who had undergone bilateral GPi-DBS combined with anterior capsulotomy for medically intractable TS and psychiatric comorbidities. All patients had experienced an accidental interruption or intentional withdrawal of pallidal stimulation during treatment. <b><i>Results:</i></b> The widespread clinical benefits achieved during the combined treatment were fully maintained after intentional or accidental DBS discontinuation. The improvement in overall tic symptoms achieved was on average 78% at the follow-up or close to the DBS discontinuation, while it was 83% at last follow-up (LFU). At LFU, most patients had functionally recovered; exhibited only mild tics; displayed minor or no obsessive-compulsive disorder symptoms, anxiety, or depression; and experienced a much better quality of life. <b><i>Conclusion:</i></b> Bilateral GPi-DBS combined with anterior capsulotomy appears to result in marked and sustained improvements in TS symptoms and psychiatric comorbidities, which are fully maintained over time, even without pallidal stimulation.
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