Deep brain stimulation for Meige syndrome

Meige syndrome is a cranial dystonia which presents with blepharospasm and oromandibular dystonia, identified in patients with Parkinson's disease, atypical Parkinsonism, and essential tremor. Treatment of Meige syndrome involves both pharmacological and surgical interventions including botulinum toxin injections, muscle relaxants, dopamine receptor antagonists, and anticholinergic drugs. Deep brain stimulation (DBS) has also been used to treat these patients with medically refractory Meige syndrome with a high degree of efficacy. Herein, we present a case of medically refractory Meige syndrome treated with bilateral globus pallidus internus (GPi) DBS with improvement of dystonia.

This is a 74-year-old female diagnosed with blepharospasm 15 years ago, after she noted increased blinking frequency and difficulty keeping her eyelids open. She was initially managed with extraocular Botox injections, but her symptoms progressed and began to include shoulder shrugging, jaw clenching, and uncontrolled head turning. A diagnosis of Meige syndrome was made and the patient responded appropriately to pharmacological management with benzodiazepines, baclofen, and carbidopa-levodopa. The patient eventually developed severe dystonia secondary to her Meige syndrome diagnosis and her symptoms were now refractory to botulinum toxin injections and medications. The patient underwent DBS of the bilateral GPi. After 52 weeks of follow-up, significant improvements in involuntary blinking, squinting, photophobia, teeth clenching, and neck tightness were noted.

DBS is a safe and effective means of treating dystonia related to Meige syndrome. Further studies are needed to elucidate optimal targeting for these patients and programming specifics for long-term outcomes.