Objective: Deep brain stimulation (DBS) of the Globus Pallidus Interna (GPi) is performed for treatment of medically intractable dystonia, including primary generalized dystonia, myoclonic dystonia, and cervical dystonia.There is little experience, however, with other focal dystonias.  We present long-term follow-up of chronic pallidal DBS in blepharospasm. Blepharospasm, a focal dystonia characterized by involuntary orbicularis oculi muscle spasms, may vary in severity from a slightly bothersome condition to a disabling disorder that renders patients functionally blind.(1)

Background: We report on a case of blepharospasm who was resistant to high dose of botulinum toxin, was successfully treated with bilateral pallidal DBS and improvement was noticed within a month and effect persisted.

Method: 70-year-old man presented with difficulty to keep his eyes open for 2 years, which gets worse with bright light. He was receiving botulinum toxin injections in orbicularis oculi and initially it was helping but effect lasted for 6-8 weeks. Hence frequency of injections was increased to every 2 months and few months later, there was no other benefit with botulinum toxin. Test dose of botulinum toxin showed no response and hence it was assumed that there was development of antibodies to the toxin. As he loves traveling, this symptom bothered him significantly and hence DBS was considered as a treatment option.

Results: DBS settings are as follows: Left GPi: C+1-2-  3.85 mA, 90 microsec and 160 hertz. Right GPi: C+9-10-  6 mA, 90 microsec and 160 hertz. Within in a month, there was significant improvement in blepharospasm. Quality of life was much improved, and the patient started traveling again, which had not been possible before.

Conclusion: DBS for Meige syndrome has been previously reported with benefit and also the recent case reports of pallidal stimulation for isolated blepharospasm(2). DBS should be considered a potential therapy for medically refractory blepharospasm. Our experience is similar to other case reports of DBS for orofacial dystonia, where improvement occurred rapidly and within days. Bilateral GPi DBS may be an effective and safe treatment for select patients with primary blepharospasm who obtain disappointing results from conventional treatment.

References: 1. Ana Filipa Santos, Andreia Veiga, Luís Augusto, Rui Vaz, Maria José Rosas and Jens Volkmann. Successful Treatment of Blepharospasm by Pallidal Neurostimulation . Mov Disord Clin Pract. 2016 Jul-Aug; 3(4): 409–411. 2. Luthra NS, Mitchell KT, Volz MM, Tamir I, Starr PA, Ostrem JL. Intractable Blepharospasm Treated with Bilateral Pallidal Deep Brain Stimulation. Tremor Other Hyperkinet Mov (N Y). 2017;7:472. Published 2017 Jul 6. doi:10.7916/D8SJ1V9F

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MDS Abstracts - https://www.mdsabstracts.org/abstract/bilateral-globus-pallidum-interna-stimulation-for-blepharospasm/