Globus Pallidus Interna DBS Improves Function in Refractory Meige Syndrome

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Meige syndrome is a craniofacial dystonia characterized by involuntary muscle contractions of the jaw and around the eyes.
Meige syndrome is a craniofacial dystonia characterized by involuntary muscle contractions of the jaw and around the eyes.

According to a 2-patient case study published in Clinical Neurology and Neurosurgery, deep brain stimulation (DBS) of the globus pallidus interna (GPi) may improve motor function and quality of life in individuals with medically refractory Meige syndrome (MS), a craniofacial dystonia characterized by involuntary muscle contractions of the jaw and around the eyes.

Two women ages 68 and 76 years were included in the case analysis. The patients had insidious onset of blepharospasm (ie, involuntary contractions around the eyes) and progressive oromandibular dystonia (ie, involuntary contractions of the jaw and tongue). Additionally, both patients reported difficulties with eating and verbal communication. Researchers evaluated improvements in motor function, disability, and quality of life following GPi-DBS. Additionally, the investigators compared the outcomes of these patients with those reported in previously published literature.

Following GPi-DBS, both patients exhibited significant improvements in motor symptoms and disability. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-M), which measures dystonia activity, improved by 92% and 77% in patient 1 and patient 2, respectively, at 24-month follow-up. In addition, patient 1 required injections of botulinum toxin to manage blepharospasm, whereas patient 2 did not require additional therapy following 24 months of continuous stimulation.

A total of 23 studies reporting BFMDRS in medically refractory MS were included in the analysis, of which 82 cases of MS were analyzed. In the pooled analysis of these studies, the researchers found a 60% improvement in BFMDRS-M subscores at a mean follow-up of 59.7±27.7 months after GPi-DBS (baseline vs follow-up, 25.5±11.2 vs 9.66±7.28, respectively). Some studies reported a benefit associated with GPi-DBS that lasted approximately 10 years.

The case study portion is limited by the small patient cohort, and the literature review portion is also limited by the inclusion of a small number of studies.

Based on the findings from the cases as well as the available literature, the researchers note that “GPi-DBS can improve craniocervical dystonia, giving back to patients several lost skills, which makes us believe that it may be a remarkable tool to decrease disability and improve quality of life.”

Reference

Aires A, Gomes T, Linhares P, Cunha F, Rosas MJ, Vaz R. The impact of deep brain stimulation on health related quality of life and disease-specific disability in Meige Syndrome (MS). Clin Neurol Neurosurg. 2018;171:53-57.

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