title: Early globus pallidus internus stimulation in pediatric patients with generalized primary dystonia: long-term efficacy and safety.
abstract: Primary generalized dystonia presents mainly at a young age and commonly is severely disabling. The authors report the long-term follow-up (mean, 73 months; range, 50-101 months) of 5 pediatric patients (mean age at surgery 13 years; range, 8-16 years) undergoing globus pallidus internus deep brain stimulation. Mean improvement in the Burke-Fahn-Marsden movement score was 67.4% (range, 47.0%-87.5%), 75.4% (range, 61.5%-91.7%), and 83.5% (range, 72.0%-93.3%) at 3 months, 12 months, and long-term follow-up (>36 months), respectively. Hardware problems (electrode dislocation/breakage of extension cable, and imminent perforation of extension cable) were observed in 2 patients (operative revision without sequelae). Except for mild dysarthria in 2 patients, no other therapy-related morbidity was observed. The authors found globus pallidus internus stimulation to offer a very effective and safe therapy in pediatric patients with primary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function.
authors.affiliation: Department of Neurosurgery, Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany. Jan.Mehrkens@med.uni-muenchen.de.
authors.forename: Jan Hinnerk
authors.initials: JH
authors.lastname: Mehrkens
authors.forename: Ingo
authors.initials: I
authors.lastname: Borggraefe
authors.forename: Berend
authors.initials: B
authors.lastname: Feddersen
authors.forename: Florian
authors.initials: F
authors.lastname: Heinen
authors.forename: Kai
authors.initials: K
authors.lastname: Bötzel
mesh_terms: D000293:Adolescent; D002648:Child; D046690:Deep Brain Stimulation / Q000009:adverse effects*; D020821:Dystonic Disorders / Q000503:physiopathology / Q000628:therapy*; D004567:Electrodes, Implanted; D005260:Female; D005917:Globus Pallidus / Q000502:physiology* / Q000601:surgery; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome