Summary

Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an established procedure to improve generalised, segmental craniofacial dystonia. However, no studies have addressed the applicability of DBS for blepharospasm as a focal craniofacial dystonia. A 52-year-old man developed medically intractable involuntary eye closure. Because the abnormal movement was observed exclusively in the eyelids, he was diagnosed not with Meige's syndrome but with blepharospasm as a focal craniofacial dystonia. He underwent stereotactic surgery under general anaesthesia for bilateral GPi-DBS. Continuous GPi stimulation almost completely abolished the blepharospasm. 15 months after the operation, his preoperative scores on the Burke-Fahn-Marsden Dystonia Rating Scale (=8 points) decreased to 1 (87.5% improvement). The present study demonstrates the applicability of GPi-DBS for treating blepharospasm presenting as focal dystonia. Further studies with accumulated case series are needed to confirm the effect of DBS on blepharospasm and other focal craniofacial dystonias.