The International Deep Brain Stimulation Registry and Database for Gilles de la Tourette Syndrome: How Does It Work?

Abstract

Tourette Syndrome (TS) is a neuropsychiatric disease characterized by a combination of motor and vocal tics. Deep brain stimulation (DBS), already widely utilized for Parkinson's disease and other movement disorders, is an emerging therapy for select and severe cases of TS that are resistant to medication and behavioral therapy. Over the last two decades, DBS has been used experimentally to manage severe TS cases. The results of case reports and small case series have been variable but in general positive. The reported interventions have, however, been variable, and there remain non-standardized selection criteria, various brain targets, differences in hardware, as well as variability in the programming parameters utilized. DBS centers perform only a handful of TS DBS cases each year, making large-scale outcomes difficult to study and to interpret. These limitations, coupled with the variable effect of surgery, and the overall small numbers of TS patients with DBS worldwide, have delayed regulatory agency approval (e.g., FDA and equivalent agencies around the world). The Tourette Association of America, in response to the worldwide need for a more organized and collaborative effort, launched an international TS DBS registry and database. The main goal of the project has been to share data, uncover best practices, improve outcomes, and to provide critical information to regulatory agencies. The international registry and database has improved the communication and collaboration among TS DBS centers worldwide. In this paper we will review some of the key operation details for the international TS DBS database and registry.

Neuroethics of neuromodulation: An update

“…In 2012, cooperation among an international body of stakeholders led to the creation of a registry and database for DBS in Tourette’s syndrome. It stores several types of data, including: demographic data, pre-operative clinical scales, surgical data, device parameters, follow-up assessment and scales, and adverse events[1]. As the registry and database develop, stakeholders emphasize ensuring data quality, data uniformity, accessibility, and transparency[1].…”

Proceedings of the Fourth Annual Deep Brain Stimulation Think Tank: A Review of Emerging Issues and Technologies

“…DBS has been explored in a subset of TS subjects with severely disabling symptoms. An international TS DBS registry and database was established in 2012 by investigators in the TS DBS field and the Tourette Association of America (TAA; previously the Tourette Syndrome Association, TSA; Deeb et al, 2016). The need for the registry and database was based on the relatively low number of cases of TS patients who have received DBS.…”

Gilles de la Tourette syndrome

“…She also highlighted, however, the paucity of evidence-based publications (5 reports on a total of 32 patients worldwide), the heterogeneity of results, and the lack of consensus on "best" brain target, all of which point to the fact that DBS for GTS remains anything but "established". Deeb and colleagues documented recently the International GTS DBS registry, totalling 157 patients to date: of these 58.5% had DBS in the thalamus and 39% in the GPi 164 .…”

“…The recent initiative of the Tourette Association of America to launch an international GTS DBS registry and database to share data, uncover best practices, improve outcomes, and to provide information to regulatory agencies, is a step in the right direction 164 . Despite 17 years of surgery for GTS, there is still no consensus about the best brain target for DBS, and this procedure remains investigational, and is still far from "established", although it may be legitimate to offer DBS to patients where other options have been exhausted, especially in patients in whom the severity of refractory motor tics threatens their life or risks inducing irreversible neurological deficit 167 .…”