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.