Case Report: Effective management of a Meige syndrome patient with subthalamic stimulation-induced dyskinesia through timed stimulation programming of different contacts

TL;DR

A new stimulation strategy helped manage severe side effects in a patient with Meige syndrome after brain stimulation treatment.

Contribution

A novel timed stimulation programming strategy using different contacts to manage refractory stimulation-induced dyskinesia in Meige syndrome.

Findings

Timed alternation between ventral and dorsal contacts reduced stimulation-induced dyskinesia without worsening symptoms.

Volume of tissue activated reconstruction suggested pallidothalamic fiber modulation suppressed dyskinesia.

The strategy offers a viable alternative when conventional DBS programming fails in Meige syndrome patients.

Abstract

Meige syndrome is a rare adult-onset segmental dystonia characterized by blepharospasms and oromandibular dystonia. Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an established treatment, but it can lead to stimulation-induced dyskinesia (SID) in some patients. Refractory SID in Meige syndrome after STN-DBS is clinically challenging. We report a case of a Meige syndrome patient who developed refractory SID following STN-DBS and was successfully managed using a novel timed-stimulation programming strategy employing different contacts.

A 47-year-old female with a two-year history of Meige syndrome developed refractory SID after the treatment of STN-DBS. Various programming strategies were attempted, including monopolar stimulation, interleaved stimulation, bipolar stimulation et al., but none achieved a balance between symptom control and SID. A novel approach involving timed alternation between ventral contacts (contacts 3 and 7) and dorsal contacts (contacts 4 and 8) was implemented. The stimulation was gradually programmed, the duration of ventral stimulation was increased while decreasing dorsal stimulation. Eventually, the patient achieved significant symptom improvement without SID. The reconstruction of the volume of tissue activated (VTA) revealed that this stimulation strategy likely modulates the neural circuits of pallidothalamic fibers (PTF) to suppress SID.

This case demonstrates that this noval timed stimulation programming can effectively manage refractory SID in Meige syndrome patients, offering a viable alternative when conventional methods fail. The findings suggest that PTF stimulation plays a key role in SID suppression, and this strategy warrants further investigation in larger cohorts.

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