Early surgical treatment in a case of myoclonus dystonia syndrome.
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.
Department of Neurology, Charité-University Medicine, Berlin, Germany andrea.kuehn@charite.de.
Andrea A
AA
Kühn
Department of Neurology, Charité-University Medicine, Berlin, Germany.
Patricia
P
Krause
Department of Neurology, Charité-University Medicine, Berlin, Germany.
Katharina
K
Lauritsch
Department of Neurology, Martha Maria Hospital, Halle/Saale, Germany.
Christian
C
Zentner
Department of Neurology, Charité-University Medicine, Berlin, Germany.
Christof
C
Brücke
Department of Neurosurgery, Charité-University Medicine, Berlin, Germany.
Gerd-Helge
GH
Schneider
D000293:Adolescent; D046690:Deep Brain Stimulation; D020821:Dystonic Disorders / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D005917:Globus Pallidus / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome
deep brain stimulation; dystonia; myoclonus; pallidal; quality of life