Runner’s Dystonia Successfully Treated with Unilateral Globus Pallidus Interna (GPi) Deep Brain Stimulation (DBS) (P1.043)

Abstract

Objective: To present the first case of runner’s dystonia successfully treated with unilateral pallidal DBS. Background: Runner’s dystonia is a rare form of adult-onset task-specific lower limb dystonia that typically presents in long-distance runners. Dystonic spasms initially occur only with running, but often progress to interfere with walking, resulting in career-ending disability for athletes. The small existing literature suggests treatment is challenging and may include medications, physical therapy, bracing, or botulinum toxin injections. It is rare for patients to return to pre-dystonia exercise levels. There is Class I Evidence for efficacy of bilateral GPi DBS for treatment of generalized dystonia, segmental dystonia, and adult-onset cervical dystonia that is refractory to medications and botulinum toxin injections. It is not known whether runner’s dystonia responds to GPi DBS. Methods: A 60-year-old male multi-marathoner presented with a 3-year history of involuntary right knee extension and ankle inversion with running, that progressed to affect walking. He had to stop running and could only walk with crutches or a full leg brace despite trials of multiple medications, physical therapy, and botulinum toxin injections. Due to lack of response to conservative therapy he underwent left GPi DBS. Results: Prior to DBS the patient had isolated involvement of the proximal and distal right leg, could no longer run or cycle, and was able to ambulate only with full-leg bracing or crutches. Pre-DBS Burke Fahn Marsden Dystonia Rating Scale (BFMDRS) total score was 6. Three months post- left GPi DBS he had no remaining dystonia on exam and had started to run again, which he had not done for 4 years. Although there was no visible dystonia on exam (BFMDRS = 0) the patient reported subjective improvement of 80[percnt]. Pre- and post-op videos provided. Conclusions: GPi DBS may be considered for runner’s dystonia refractory to conservative therapy.

Disclosure: Dr. Saminejad has nothing to disclose. Dr. House has received research support from Boston Scientific. Dr. Ballard had received research support from Boston Scientific. Dr. Schrock has received research support from Boston Scientific.