Early Surgical Treatment in a Case of Myoclonus Dystonia Syndrome. (November 2014)

• Record Type: Journal Article
• Authors: Kühn, Andrea A.; Krause, Patricia; Lauritsch, Katharina; Zentner, Christian; Brücke, Christof; Schneider, Gerd-Helge
• Is Part Of: Journal of child neurology. Volume 29:Number 11(2014:Nov.)
• Page Start: NP149
• Page End: NP150
• Publication Date: 2014-11
• Subjects: myoclonus -- dystonia -- deep brain stimulation -- quality of life -- pallidal; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073813513071
• Languages: English
• ISSNs: 0883-0738

Abstract

Myoclonus dystonia syndrome is often misdiagnosed in young children and appropriate treatment is delayed, which has a negative impact on motor development, participation, and emotional well-being. In severely affected patients, deep brain stimulation of the globus pallidus internus has been used successfully to treat both dystonia and myoclonus. Here, the authors present a case of early successful treatment of myoclonus dystonia syndrome by pallidal deep brain stimulation in a patient at the age of 17 years leading to 83% reduction in dystonia score and 89% reduction in myoclonus. The patient gained significant improvement in motor function as well as increased participation and reduced stigma.