title: Bilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study.
abstract: BACKGROUND
  Cerebral palsy (CP) with dystonia-choreoathetosis is a common cause of disability in children and in adults, and responds poorly to medical treatment. Bilateral pallidal deep brain stimulation (BP-DBS) of the globus pallidus internus (GPi) is an effective treatment for primary dystonia, but the effect of this reversible surgical procedure on dystonia-choreoathetosis CP, which is a subtype of secondary dystonia, is unknown. Our aim was to test the effectiveness of BP-DBS in adults with dystonia-choreoathetosis CP.
  
  
  METHODS
  We did a multicentre prospective pilot study of BP-DBS in 13 adults with dystonia-choreoathetosis CP who had no cognitive impairment, little spasticity, and only slight abnormalities of the basal ganglia on MRI. The primary endpoint was change in the severity of dystonia-choreoathetosis after 1 year of neurostimulation, as assessed with the Burke-Fahn-Marsden dystonia rating scale. The accuracy of surgical targeting to the GPi was assessed masked to the results of neurostimulation. Analysis was by intention to treat.
  
  
  RESULTS
  The mean Burke-Fahn-Marsden dystonia rating scale movement score improved from 44.2 (SD 21.1) before surgery to 34.7 (21.9) at 1 year post-operatively (p=0.009; mean improvement 24.4 [21.1]%, 95% CI 11.6-37.1). Functional disability, pain, and mental health-related quality of life were significantly improved. There was no worsening of cognition or mood. Adverse events were related to stimulation (arrest of the stimulator in one patient, and an adjustment to the current intensity in four patients). The optimum therapeutic target was the posterolateroventral region of the GPi. Little improvement was seen when the neurostimulation diffused to adjacent structures (mainly to the globus pallidus externus [GPe]).
  
  
  CONCLUSIONS
  Bilateral pallidal neurostimulation could be an effective treatment option for patients with dystonia-choreoathetosis CP. However, given the heterogeneity of motor outcomes and the small sample size, results should be interpreted with caution. The optimum placement of the leads seemed to be a crucial, but not exclusive, factor that could affect a good outcome.
  
  
  BACKGROUND
  National PHRC; Cerebral Palsy Foundation: Fondation Motrice/APETREIMC; French INSERM Dystonia National Network; Medtronic.
authors: AP-HP, Groupe hospitalier Pitié-Salpêtrière, Fédération de Neurologie, Paris, F-75013, France. marie.vidailhet@psl.aphp.fr
authors: Marie
authors: M
authors: Vidailhet
authors: Jerome
authors: J
authors: Yelnik
authors: Christelle
authors: C
authors: Lagrange
authors: Valerie
authors: V
authors: Fraix
authors: David
authors: D
authors: Grabli
authors: Stephane
authors: S
authors: Thobois
authors: Pierre
authors: P
authors: Burbaud
authors: Marie-Laure
authors: ML
authors: Welter
authors: Jin
authors: J
authors: Xie-Brustolin
authors: Maria-Clara Coelho
authors: MC
authors: Braga
authors: Claire
authors: C
authors: Ardouin
authors: Virginie
authors: V
authors: Czernecki
authors: Hélène
authors: H
authors: Klinger
authors: Stephan
authors: S
authors: Chabardes
authors: Eric
authors: E
authors: Seigneuret
authors: Patrick
authors: P
authors: Mertens
authors: Emmanuel
authors: E
authors: Cuny
authors: Soledad
authors: S
authors: Navarro
authors: Philippe
authors: P
authors: Cornu
authors: Alim-Louis
authors: AL
authors: Benabid
authors: Jean-Francois
authors: JF
authors: Le Bas
authors: Didier
authors: D
authors: Dormont
authors: Marc
authors: M
authors: Hermier
authors: Kathy
authors: K
authors: Dujardin
authors: Serge
authors: S
authors: Blond
authors: Pierre
authors: P
authors: Krystkowiak
authors: Alain
authors: A
authors: Destée
authors: Eric
authors: E
authors: Bardinet
authors: Yves
authors: Y
authors: Agid
authors: Paul
authors: P
authors: Krack
authors: Emmanuel
authors: E
authors: Broussolle
authors: Pierre
authors: P
authors: Pollak
mesh_terms: D000328:Adult; D001264:Athetosis / Q000150:complications / Q000628:therapy*; D001479:Basal Ganglia / Q000473:pathology; D002547:Cerebral Palsy / Q000150:complications / Q000628:therapy*; D002819:Chorea / Q000150:complications / Q000628:therapy*; D046690:Deep Brain Stimulation / Q000379:methods*; D004185:Disability Evaluation; D004421:Dystonia / Q000150:complications / Q000628:therapy*; D005260:Female; D007839:Functional Laterality; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010865:Pilot Projects; D011446:Prospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D055815:Young Adult