Stimulation of the globus pallidus internus for childhood-onset dystonia.
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.
Zorzi
Giovanna
G
Department of Child Neurology, Istituto Nazionale Neurologico Carlo Besta, Milano, Italy.
Marras
Carlo
C
Nardocci
Nardo
N
Franzini
Angelo
A
Chiapparini
Luisa
L
Maccagnano
Elio
E
Angelini
Lucia
L
Caldiroli
Dario
D
Broggi
Giovanni
G
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
D000978:Antiparkinson Agents; D007980:Levodopa