Objective: To report the first cranio- cervical dystonia (Meige syndrome) patient who received Deep Brain Stimulation (DBS) implant in Hong Kong.

Background: Meige syndrome is an idiopathic dystonia that involves craniofacial and often cervical muscles. It manifests as bleharospasm and oromandibuar dystonia. Botulinum toxin injection is the standard treatment, but its effect is transient and repeated injection is required. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a treatment option for dystonia. The mean improvement of Burke – Fahn – Marsden dystonia rating – motor (BFMDRS- M) in a recent review is 66.9% with bilateral GPi DBS for Meige syndrome. Most of the reports are single or small series.1

Method: A 53 year-old lady, suffered from insidious onset of blepharospasm and oromandibular dystonia since 2011. All investigations were unremakable. She had functional blindness secondary to blepharospasm that required wearing special eyeglasses with eyelid retractor for daily living. She received botulinum toxin injection without much improvement in dystonia control. Her BFMDRS- M total score was 14 with a score of 8 for the eye- part, in the preoperative workup. She agreed and was scheduled for GPi DBS surgery at end of 2017 after detailed discussion with patient and her family.

Results: Programming of DBS started one week after surgery. At the first 2 months after surgery, there was no significant improvement in blepharospasm and oromandibular dystonia. At 3 month, by using high voltage with double monopolar parameter of GPi, patient ‘s oromandibular dystonia had 30 % improvement but blepharospasm remained the same. Further, patient ‘s blehparospasm symptom had 20 % improvement at 4 month after surgery. At 6 month after surgery, she obtained 57% improvement in BFMDRS. She does not require wearing special eyeglass with eyelid retractor for daily living. She only reported having occasional blepharospasm after prolonged conversation and when using phone.

Conclusion: Our case showed that bilateral GPi DBS for Meige syndrome is safe and effective. The improvement was similar to the literature. The case report contributes to the literature of GPi DBS for Meige syndrome.

References: Wang X, Chao Zhang C, Wang Y, et al, Deep Brain Stimulation for Craniocervical Dystonia (Meige Syndrome): A Report of Four Patients and a Literature-Based Analysis of Its Treatment Effects. Neuromodulation 2016; 19: 818-823

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MDS Abstracts - https://www.mdsabstracts.org/abstract/bilateral-globus-pallidus-interna-deep-brain-stimulation-in-cranio-cervical-dystonia-meige-syndrome-patient-in-hong-kong/