501 - Therapeutic Effects of Deep Brain Stimulation in a Case of Refractory Meige Syndrome

Case Diagnosis: A 55-year-old male presented with several year history of squinting and repetitive eye blinking, worse with bright light and better in low light. He was initially diagnosed with blepharospasm, however symptoms worsened following anesthesia for cardiac catheterization. He began experiencing worsening eyelid dystonia, speech articulation difficulties, and cognitive fog. Standard treatments with botulinum toxin injections, low-dose benzodiazepines, and psychiatric interventions provided minimal relief. Eyelid dystonia progressively worsened over several years, leading to difficulty completing household tasks, inability to work in customer facing roles, and cessation of driving. He subsequently developed oromandibular involvement with spasms and dyskinesia, leading to a diagnosis of Meige syndrome.

Case Description: The patient trialed multiple therapies including benzodiazepines, SSRIs, tetrabenazine, acupuncture, and continued botulinum toxin injections, with limited success. Five years after symptom onset, he underwent deep brain stimulation (DBS) placement. Initial stimulation improved blepharospasm with limited success. The patient necessitated electrode exchange surgeries and ongoing adjustment of stimulation parameters. As his DBS therapy was optimized over several years, he reported significant improvements in phonation, neck tension, facial grimacing, and blepharospasm, though symptoms exacerbated with stress and prolonged speaking.

Discussions: Meige syndrome is a rare form of dystonia involving blepharospasm and oromandibular dystonia. Initial treatments, including botulinum toxin and oral medications, often provide limited relief. DBS targeting the globus pallidus internus and subthalamic nucleus can improve functional outcomes in cases refractory to pharmacotherapies. Symptom variability with position, stress, fatigue, and circadian variation further underscores the complex neurophysiology of this dystonia. Early recognition and a multidisciplinary approach integrating neurology, neurosurgery, psychiatry, and rehabilitation are critical for functional recovery.

Conclusions: This case demonstrates that DBS can meaningfully improve severe, medication-refractory Meige syndrome with persistent blepharospasm and oromandibular dystonia. Long-term management requires frequent device adjustments, and continued adjunctive therapies. Multimodal interventions remain essential for maximizing patient independence and quality of life.