Early globus pallidus internus stimulation in pediatric patients with generalized primary dystonia: long-term efficacy and safety.
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.
Mehrkens
Jan Hinnerk
JH
Department of Neurosurgery, Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany. Jan.Mehrkens@med.uni-muenchen.de.
Borggraefe
Ingo
I
Feddersen
Berend
B
Heinen
Florian
F
Bötzel
Kai
K
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