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‐positiv...

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Bibliographic Details
Published in:Movement disorders 2005-09, Vol.20 (9), p.1194-1200
Main Authors: Zorzi, Giovanna, Marras, Carlo, Nardocci, Nardo, Franzini, Angelo, Chiapparini, Luisa, Maccagnano, Elio, Angelini, Lucia, Caldiroli, Dario, Broggi, Giovanni
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age of Onset
Antiparkinson Agents - therapeutic use
Biological and medical sciences
Child
Child, Preschool
childhood dystonia
Deep Brain Stimulation - instrumentation
Diseases of striated muscles. Neuromuscular diseases
Dystonia - drug therapy
Dystonia - physiopathology
Dystonia - therapy
Female
globus pallidus
Globus Pallidus - physiopathology
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Infant
Levodopa - therapeutic use
Male
Medical sciences
Nervous system (semeiology, syndromes)
Neurology
status dystonicus
stimulation
Surveys and Questionnaires
Videotape Recording
Walking
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Summary: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. © 2005 Movement Disorder Society
ISSN:0885-3185
1531-8257
DOI:10.1002/mds.20510