Deep brain stimulation in myoclonus-dystonia syndrome

Laura Cif; Enza Maria Valente; Simone Hemm; Christine Coubes; Nathalie Vayssiere; Stéphanie Serrat; Annalisa Di Giorgio; Philippe Coubes

doi:10.1002/mds.20030

Deep brain stimulation in myoclonus-dystonia syndrome

Laura Cif, Enza Maria Valente, Simone Hemm, Christine Coubes, Nathalie Vayssiere, Stéphanie Serrat, Annalisa Di Giorgio, Philippe Coubes

IRCCS Casa Sollievo della Sofferenza

Research output: Contribution to journal › Article › peer-review

Abstract

Myoclonus-dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the ε-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS.

Original language	English
Pages (from-to)	724-727
Number of pages	4
Journal	Movement Disorders
Volume	19
Issue number	6
DOIs	https://doi.org/10.1002/mds.20030
Publication status	Published - Jun 2004

Keywords

Dystonia
Myoclonus
Neurosurgery

ASJC Scopus subject areas

Clinical Neurology
Neuroscience(all)

Access to Document

10.1002/mds.20030

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title = "Deep brain stimulation in myoclonus-dystonia syndrome",

abstract = "Myoclonus-dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the ε-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS.",

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AU - Cif, Laura

AU - Valente, Enza Maria

AU - Hemm, Simone

AU - Coubes, Christine

AU - Vayssiere, Nathalie

AU - Serrat, Stéphanie

AU - Di Giorgio, Annalisa

AU - Coubes, Philippe

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AB - Myoclonus-dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the ε-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS.

KW - Dystonia

KW - Myoclonus

KW - Neurosurgery

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