Sidiropoulos C, Mestre T, Hutchison W, Moro E, Valencia A, Poon YY, Fallis M, Rughani AI, Kalia SK, Lozano AM, Lang AE - May 15, 2014
We report on the clinical efficacy of bilateral globus pallidus internus deep brain stimulation in two patients with myoclonus dystonia/essential myoclonus who lack mutations in the epsilon sarcoglycan gene. The primary outcome measures were the Burke-Fahn-Marsden Dystonia Scale motor severity and the Unified Myoclonus Rating Scale scores, and the secondary outcome measure was the 36-item Short Form Health Survey score at the last postoperative follow up. Neuronal firing rates were also calculated from microelectrode recordings.