Early surgical treatment in a case of myoclonus dystonia syndrome.

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.

• Authors: Andrea A Kühn, Patricia Krause, Katharina Lauritsch, Christian Zentner, Christof Brücke, Gerd-Helge Schneider
• Journal: Journal of child neurology (J Child Neurol) Vol. 29 Issue 11 Pg. NP149-50 (Nov 2014) ISSN: 1708-8283 [Electronic] United States
• PMID: 24453142 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © The Author(s) 2014.

Topics

• Adolescent
• Deep Brain Stimulation
• Dystonic Disorders (physiopathology, surgery, therapy)
• Globus Pallidus (physiopathology, surgery)
• Humans
• Male
• Severity of Illness Index
• Treatment Outcome