Objective: This report aims to demonstrate the benefit of simultaneous, bilateral globus pallidus internus (GPi) and “rescue” subthalamic nucleus (STN) deep brain stimulation (DBS) in one patient with medically refractory craniocervical dystonia (CCD).

Background: Bilateral GPi DBS and STN DBS have been independently reported as effective treatments for medically refractory CCD. While multiple studies have documented successful outcomes in CCD at both stimulation targets, some patients have a suboptimal response despite optimal lead positioning and extensive programming trials. Dual stimulation at rescue targets has been explored in Parkinson’s disease and Essential Tremor, but there is a dearth of data on outcomes for trials of dual DBS in CCD.

Method: We describe one patient with intractable long-standing CCD characterized by disabling blepharospasm, dysarthria, and dysphagia unresponsive to Botulinum toxin and oral medications including Sinemet, Artane, Baclofen, and Tetrabenazine. Bilateral GPi DBS yielded mild, not clinically meaningful improvement in dysphagia and cervical dystonia despite frequent programming visits over 2 years. Additional bilateral ‘rescue’ STN DBS was implanted 24 months later to provide additional symptomatic benefit.

Results: Toronto Western Spasmodic Torticollis Rating Scale Severity (TWSTRS-S) scores were 22 at baseline and 25 after bilateral GPi DBS (43 months post-op). Worsening TWSTRS-S was attributed to disease progression due to patient reported subjective worsening of dysphagia and blepharospasm with GPi DBS OFF. TWSTRS-S score improved to 20 seven months after implantation of bilateral STN leads. Burke Fahn Marsden dystonia rating scale movement (BFMDRS-M) scores were 31 at baseline and 30.5 after bilateral GPi surgery; BFMDRS-M scores for blepharospasm, dysarthria/dysphagia, and jaw dystonia improved with a total score decrease to 13 seven months after implantation of bilateral STN leads.

Conclusion: This case supports the utilization of ‘rescue’ STN DBS for medically refractory CCD where pallidal stimulation does not provide meaningful symptomatic benefit in CCD patients; longer follow up is needed.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/dual-globus-pallidus-internus-and-subthalamic-nucleus-deep-brain-stimulation-for-medically-refractory-craniocervical-dystonia-a-case-report/