Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology

We report the intraoperative results, subsequent course, and 1-year follow-up evaluation of a patient with medication-refractory craniofacial dystonia for whom we planned bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) implantation but delayed the left GPi DBS implantation because of robust intraoperative effects of right GPi DBS.

A 47-year-old patient had a 5-year history of progressively severe, bilateral craniofacial dystonia with blepharospasm (Meige's syndrome) that was refractory to medications and to botulinum toxin (A and B) injections. Blepharospasm interfered with his ability to perform his duties as a Special Forces soldier and ended his military career.

Under stereotactic guidance (magnetic resonance imaging and computed tomographic image fusion, Cosman-Roberts-Wells frame, and University of Florida surgical navigation software) and with detailed microelectrode mapping (four microelectrode passes), a DBS electrode was implanted in the right posteroventral GPi. Microelectrode recordings were taken to document electrophysiological activity of neurons in the region, and intraoperative macrostimulation was performed. The patient was followed up for 6 months with right unilateral GPi DBS, and later a left GPi DBS electrode was placed.

Although DBS for primary generalized dystonia is commonly performed by simultaneously implanting bilateral GPi electrodes, it may be reasonable in cases of refractory blepharospasm and/or craniofacial dystonia to use a staged procedure for implantation in selected patients. Additionally, the physiology, especially that encountered in the striatum, may help to elucidate the pathophysiological basis for refractory blepharospasm and Meige's syndrome. More cases will be needed to determine the significance of the results reported in this article.