title: Stimulation of the globus pallidus internus for childhood-onset dystonia.
abstract: We report the results of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in 12 patients with childhood-onset generalized dystonia refractory to medication, including 3 patients with status dystonicus. There were 8 patients who had DYT1-negative primary dystonia, 1 had DYT1-positive dystonia, and 3 had symptomatic dystonia. Stimulation was effective in all but 1 patient. Dystonic postures and movements of the axis and limbs responded to DBS to a greater extent than oromandibular dystonia and fixed dystonic postures. These findings provide further evidence that pallidal stimulation is an effective treatment for intractable childhood-onset dystonia, including status dystonicus, and together with previous findings, suggest that it should be considered the treatment of choice for these conditions.
authors.affiliation: Department of Child Neurology, Istituto Nazionale Neurologico Carlo Besta, Milano, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Zorzi
authors.forename: Carlo
authors.initials: C
authors.lastname: Marras
authors.forename: Nardo
authors.initials: N
authors.lastname: Nardocci
authors.forename: Angelo
authors.initials: A
authors.lastname: Franzini
authors.forename: Luisa
authors.initials: L
authors.lastname: Chiapparini
authors.forename: Elio
authors.initials: E
authors.lastname: Maccagnano
authors.forename: Lucia
authors.initials: L
authors.lastname: Angelini
authors.forename: Dario
authors.initials: D
authors.lastname: Caldiroli
authors.forename: Giovanni
authors.initials: G
authors.lastname: Broggi
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000978:Antiparkinson Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D046690:Deep Brain Stimulation / Q000295:instrumentation*; D004421:Dystonia / Q000188:drug therapy / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D005917:Globus Pallidus / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007980:Levodopa / Q000627:therapeutic use; D008297:Male; D011795:Surveys and Questionnaires; D014743:Videotape Recording; D016138:Walking
chemical_list: D000978:Antiparkinson Agents; D007980:Levodopa