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Deep brain stimulation in myoclonus–dystonia syndrome

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

Publisher
John Wiley and Sons
Year
2004
Tongue
English
Weight
65 KB
Volume
19
Category
Article
ISSN
0885-3185

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✦ Synopsis

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. © 2004 Movement Disorder Society

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