title: Bilateral globus pallidus internus deep brain stimulation for dyskinetic cerebral palsy supports success of cochlear implantation in a 5-year old ex-24 week preterm twin with absent cerebellar hemispheres.
abstract: BACKGROUND
  Early onset dystonia (dyskinesia) and deafness in childhood pose significant challenges for children and carers and are the cause of multiple disability. It is particularly tragic when the child cannot make use of early cochlear implantation (CI) technology to relieve deafness and improve language and communication, because severe cervical and truncal dystonia brushes off the magnetic amplifier behind the ears. Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) neuromodulation can reduce dyskinesia, thus supporting CI neuromodulation success.
  
  
  METHODS
  We describe the importance of the order of dual neuromodulation surgery for dystonia and deafness. First with bilateral GPi DBS using a rechargeable ACTIVA-RC neurostimulator followed 5 months later by unilateral CI with a Harmony (BTE) Advanced Bionics Hi Res 90 K cochlear device. This double neuromodulation was performed in series in a 12.5 kg 5 year-old ex-24 week gestation-born twin without a cerebellum.
  
  
  RESULTS
  Relief of dyskinesia enabled continuous use of the CI amplifier. Language understanding and communication improved. Dystonic storms abated. Tolerance of sitting increased with emergence of manual function. Status dystonicus ensued 10 days after ACTIVA-RC removal for infection-erosion at 3 years and 10 months. He required intensive care and DBS re-implantation 3 weeks later together with 8 months of hospital care. Today he is virtually back to the level of functioning before the DBS removal in 2012 and background medication continues to be slowly weaned.
  
  
  CONCLUSIONS
  This case illustrates that early neuromodulation with DBS for dystonic cerebral palsy followed by CI for deafness is beneficial. Both should be considered early i.e. under the age of five years. The DBS should precede the CI to maximise dystonia reduction and thus benefits from CI. This requires close working between the paediatric DBS and CI services.
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. Electronic address: jeanpierrelin@me.com.
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Lin
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Margaret
authors.initials: M
authors.lastname: Kaminska
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Sarah
authors.initials: S
authors.lastname: Perides
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Hortensia
authors.initials: H
authors.lastname: Gimeno
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Lesley
authors.initials: L
authors.lastname: Baker
authors.affiliation: Complex Motor Disorders Service, Evelina London Children's Hospital, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Lumsden
authors.affiliation: Department of Audiology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Anzell
authors.initials: A
authors.lastname: Britz
authors.affiliation: Department of Audiology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Sandra
authors.initials: S
authors.lastname: Driver
authors.affiliation: Department of Audiology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Alec
authors.initials: A
authors.lastname: Fitzgerald-O'Connor
authors.affiliation: Department of Neurosurgery, Kings' College Hospital NHS Foundation Trust, Denmark Hill, London, SE5 9RS, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Selway
mesh_terms: D001264:Athetosis / Q000534:rehabilitation*; D002531:Cerebellum / Q000002:abnormalities*; D002547:Cerebral Palsy / Q000503:physiopathology / Q000534:rehabilitation*; D002648:Child; D002675:Child, Preschool; D002819:Chorea / Q000503:physiopathology / Q000534:rehabilitation*; D019929:Cochlear Implantation / Q000295:instrumentation* / Q000534:rehabilitation; D003131:Combined Modality Therapy; D003638:Deafness / Q000503:physiopathology / Q000534:rehabilitation*; D046690:Deep Brain Stimulation / Q000295:instrumentation*; D004200:Diseases in Twins / Q000503:physiopathology / Q000534:rehabilitation*; D004421:Dystonia / Q000503:physiopathology / Q000534:rehabilitation*; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000503:physiopathology / Q000534:rehabilitation*; D008297:Male; D016896:Treatment Outcome
keywords: Childhood; Cochlear implantation; Deafness; Deep brain stimulation; Dystonic cerebral palsy; Neuromodulation