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Acta Neurológica Colombiana
Print version ISSN 0120-8748On-line version ISSN 2422-4022
Abstract
TABORDA HOLGUIN, Sergio Andrés et al. Deep brain stimulation of the internal globus pallidus in myoclonus-dystonia syndrome: a case series and review of the literature. Acta Neurol Colomb. [online]. 2022, vol.38, n.3, pp.131-138. Epub Oct 22, 2022. ISSN 0120-8748. https://doi.org/10.22379/24224022418.
INTRODUCTION:
Myoclonic dystonia is a movement disorder with low prevalence, but very disabling, where refractoriness to medical treatment is frequent. Deep brain stimulation has been proposed as a therapeutic option, seeking to improve motor function, disability and quality of life in these patients.
MATERIALS AND METHODS:
We present 3 patients with a clinical diagnosis of Myoclonic-Dystonia without genetic confirmation, who underwent bilateral deep brain stimulation of the Globus Pallidus Internus.
RESULTS:
A significant improvement was evidenced in the evaluation of the unified myoclonus scale (80-90 %) and in the Burke-Fahn-Marsden dystonia scale (both in mobility and in disability). The clinical improvement was evidenced in the 3 patients, in follow-up periods that were between 6 months and 5 years after deep brain stimulation.
DISCUSSION AND CONCLUSIONS:
Findings in this Colombian case series are consistent with that reported in the literature. However, the current description provides information on the outcome of patients without genotyping undergoing deep brain stimulation, considering that the efficacy of the intervention in these types of patients without genetic confirmation has not been determined and depends on other factors.
Keywords : Dystonia; Deep brain stimulation; Globus pallidus; Quality of life (MeSH).