title: [Treatment of early-onset generalized dystonia by chronic bilateral stimulation of the internal globus pallidus. Apropos of a case].
abstract: Dystonia musculorum deformans is an inherited severe disease, with a wide clinical polymorphism. The most severe clinical forms with early onset carry a high risk of life-threatening complications. In the absence of any efficient medical treatment, bilateral pallidotomy has previously been reported to be of value in the management of this disease. We report the first clinical case of a severe early-onset generalized dystonia dramatically improved by a bilateral stimulation of the internal globus pallidus. In November 1996, we proposed this neurosurgical procedure for a 8-year-old girl, who had suffered since the age of 3 from severe generalized dystonia, and who progressively became totally dependent and bedridden. She had been under sedation and permanent controlled respiratory assistance for the last two months. The etiology of the disease remained unknown (the DYT1 mutation was absent). Under general anesthesia, we bilaterally implanted a four-contacts electrode in the internal globus pallidus, using the Leksell's stereotactic frame and a 1.5 tesla MRI control. A dramatic improvement was noted 6 weeks later and led us to connect the two electrodes to neurostimulators inserted under the abdominal skin.
authors.affiliation: Service de Neurochirurgie, Centre Gui-de-Chauliac, CHU, Montpellier. p-coubes@chu-montpellier.fr
authors.forename: P
authors.initials: P
authors.lastname: Coubes
authors.forename: B
authors.initials: B
authors.lastname: Echenne
authors.forename: A
authors.initials: A
authors.lastname: Roubertie
authors.forename: N
authors.initials: N
authors.lastname: Vayssière
authors.forename: S
authors.initials: S
authors.lastname: Tuffery
authors.forename: V
authors.initials: V
authors.lastname: Humbertclaude
authors.forename: G
authors.initials: G
authors.lastname: Cambonie
authors.forename: M
authors.initials: M
authors.lastname: Claustres
authors.forename: P
authors.initials: P
authors.lastname: Frerebeau
mesh_terms: D017668:Age of Onset; D002648:Child; D004422:Dystonia Musculorum Deformans / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D004599:Electric Stimulation Therapy / Q000295:instrumentation / Q000379:methods; D004567:Electrodes, Implanted; D005260:Female; D005917:Globus Pallidus / Q000503:physiopathology; D006801:Humans; D013238:Stereotaxic Techniques
vernacular_title: Traitement de la dystonie généralisée à début précoce par stimulation chronique bilatérale des globus pallidus internes. A propos d'un cas.