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Rescue Subthalamic Deep Brain Stimulation for Refractory Meige Syndrome
Veronica Tavares Aragão1, Sara Carvalho Barbosa Casagrande1, Clarice Listik1
1Department of Neurology, Movement Disorders Center, School of Medicine, University of São Paulo, São Paulo, Brazil.
Stereotactic and Functional Neurosurgery
|April 25, 2021
Summary
Meige syndrome, a disabling dystonia, may require alternative deep brain stimulation (DBS) targets when standard globus pallidus internus (GPi) DBS is ineffective. This case shows subthalamic nucleus (STN) DBS can be a successful rescue therapy.
Area of Science:
- Neurology
- Neurosurgery
- Movement Disorders
Background:
- Meige syndrome is a disabling segmental dystonia.
- Botulinum toxin is a primary treatment, but some patients are refractory.
- Deep brain stimulation (DBS), particularly targeting the globus pallidus internus (GPi), is an established therapy with good efficacy.
Observation:
- Some patients exhibit suboptimal responses to bilateral GPi DBS.
- Rescue DBS may be considered in such cases.
- This case report details a patient with Meige syndrome who experienced a good outcome following subthalamic nucleus (STN) DBS, but not GPi DBS.
Findings:
- The patient's GPi DBS was well-positioned and adequately programmed, yet yielded suboptimal results.
- Conversely, STN DBS provided a favorable therapeutic response.
- Potential explanations for differential outcomes include the larger dimension of the GPi and its specific somatotopic organization.
Implications:
- Subthalamic nucleus (STN) DBS may represent a viable alternative or rescue therapy for Meige syndrome when GPi DBS is insufficient.
- Understanding the neuroanatomy and somatotopic organization of targets is crucial for optimizing DBS outcomes in dystonia.
- Further research is warranted to elucidate the optimal DBS targets for refractory Meige syndrome.
