Unilateral Deep Brain Stimulation of Globus Pallidus internus and Medial Intermediate Ventral Nuclei of the Optic Thalamus combined in a case of Dystonia Myoclonus with good evolution after 6 years of follow-up.

Abstract

Introduction: Myoclonus dystonia syndrome (MDS) is an autosomal dominant or sporadic disorder characterized by slowly progressive myoclonic jerks and dystonia that sometimes becomes disabling. The objective of our work to demonstrate the possibility of good long-term functional results, and to analyze the respective literature on the state of the art on the subject.
Materials and methods: We present a clinical case of a 61-year-old patient with advanced and refractory symptoms, operated on in July 2014 with good evolution, according to the Burn-Fahn- Madsen and QoL scales. The examination of the bibliography was carried out through Pubmed, Latindex, Scielo and Medline, examining more than two thousand works published in four languages (English, French, Portuguese and Spanish). Results: We present a 61-year-old patient, who to date presents sustained improvement more than 6 years after his surgical intervention. Findings in the literature point to the possibility of obtaining occasional prolonged improvements, but we obtained little information on the use and results of the combined method. Conclusions: MDS has occasionally been successfully treated with combined stimulation on occasion, but over the past 10 years its use appears to have been reduced or abandoned. The publication of this case with prolonged improvement aims to relaunch the combined method as a treatment.