German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM)

Koy, Anne; Weinsheimer, M.; Pauls, K. Amande M.; Kühn, Andrea A.; Krause, Patricia; Huebl, Julius; Schneider, Gerd‐Helge; Deuschl, Günther; Erasmi, R.; Falk, Daniela; Krauss, Joachim K.; Lütjens, G.; Schnitzler, Alfons; Wojtecki, Lars; Vesper, Jan; Korinthenberg, Rudolf; Coenen, Volker A.; Visser‐Vandewalle, Veerle; Hellmich, Martin; Timmermann, Lars

doi:10.1016/j.ejpn.2016.05.023

Cited by 32 publications

(33 citation statements)

References 43 publications

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“…However, more research is needed. Two multicenter registries for pediatric DBS with uniform data elements and outcomes have already been established . Results from these registries will further contribute to understanding of the role of DBS in children.…”

Section: Discussionmentioning

confidence: 99%

“…Two multicenter registries for pediatric DBS with uniform data elements and outcomes have already been established. 39,40 Results from these registries will further contribute to understanding of the role of DBS in children. Further, the first prospective, multicenter trial with planned doubleblind crossover in children and young people with cerebral palsy is underway.…”

Section: Strength and Limitationsmentioning

confidence: 99%

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Deep brain stimulation for pediatric dystonia: a meta‐analysis with individual participant data

Elkaim

Alotaibi

Sigal

et al. 2018

Develop Med Child Neuro

107

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Aim We performed a meta‐analysis with individual participant data of deep brain stimulation (DBS) for dystonia in children and young people. Method Three databases (PubMed, Embase, and Web of Science) were queried from January 1999 to August 2017 with no language restrictions to identify case studies and cohort studies reporting on pediatric patients (age ≤21y) with dystonia. The primary outcomes were changes in Burke‐Fahn‐Marsden (BFM) or Barry‐Albright Dystonia Scale scores. A mixed‐effects regression was used to identify associations between clinical covariates and outcomes. Results Of 2509 citations reviewed, 72 articles (321 children) were eligible. At last follow‐up (median 12mo, 25th centile=9.0; 75th centile=32.2), 277 (86.3%) patients showed improvement in dystonia, while 66.1 percent showed clinically significant (>20%) BFM Dystonia Rating Scale‐motor improvement. On multivariable hierarchical regression, older age at dystonia onset, inherited dystonia without nervous system pathology and idiopathic dystonia (vs inherited with nervous system pathology or acquired dystonia), and truncal involvement indicated a better outcome (p<0.05). Interpretation The data suggest that DBS is effective and should be considered in selected children with inherited or idiopathic dystonia. What this paper adds Deep brain stimulation is effective in selected children with inherited or idiopathic dystonia.

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Section: Discussionmentioning

confidence: 99%

Section: Strength and Limitationsmentioning

confidence: 99%

Deep brain stimulation for pediatric dystonia: a meta‐analysis with individual participant data

Elkaim

Alotaibi

Sigal

et al. 2018

Develop Med Child Neuro

107

View full text Add to dashboard Cite

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“…Collaborative networks are required using common definitions and meaningful outcome measures to develop adequate dystonia stratification for therapeutic choice and individual patient prognosis (88, 89) within a framework of the International Classification of Function (90) with measures that are meaningful to children with dystonia and their families (66–68). New work is differentiating the relative impact of dystonia on function compared to choreoathetosis and this reveals that dystonia is the most important factor associated with neurodisability (91).…”

Section: Developing Coherent Networked Research Strategies For Diagnmentioning

confidence: 99%

Recognizing the Common Origins of Dystonia and the Development of Human Movement: A Manifesto of Unmet Needs in Isolated Childhood Dystonias

Lin

Nardocci

2016

Front. Neurol.

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Dystonia in childhood may be severely disabling and often unremitting and unrecognized. Considered a rare disorder, dystonic symptoms in childhood are pervasive in many conditions including disorders of developmental delay, cerebral palsy (CP), autism, neurometabolic, neuroinflammatory, and neurogenetic disorders. Collectively, there is a need to recognize the role of early postures and movements which characterize phases of normal fetal, infant, and child development as a backdrop to the many facets of dystonia in early childhood neurological disorders and to be aware of the developmental context of dystonic symptoms. The role of cocontraction is explored throughout infancy, childhood, young adulthood, and in the elderly. Under-recognition of pervasive dystonic disorders of childhood, including within CP is reviewed. Original descriptions of CP by Gowers are reviewed and contemporary physiological demonstrations are used to illustrate support for an interpretation of the tonic labyrinthine response as a manifestation of dystonia. Early recognition and molecular diagnosis of childhood dystonia where possible are desirable for appropriate clinical stratification and future precision medicine and functional neurosurgery where appropriate. A developmental neurobiological perspective could also be useful in exploring new clinical strategies for adult-onset dystonia disorders focusing on environmental and molecular interactions and systems behaviors.

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“…Pallidal DBS is a technique of proven effectiveness and safety for the treatment of dystonia in adults 11,20,21,41,43 and children. 5,8,15,17,18,33,35,40 It has also been used to treat lifethreatening conditions such as status dystonicus. 3,10 Our previous experience with surgery for the treatment of dystonia was described in an earlier publication by Valldeoriola et al 41 In order to offer this treatment to pediatric patients in our country, we have created a Pediatric Multidisciplinary Movement Disorders Unit and have initiated a DBS Program.…”

Section: Discussionmentioning

confidence: 99%

Frameless robot-assisted pallidal deep brain stimulation surgery in pediatric patients with movement disorders: precision and short-term clinical results

Candela

Vanegas

Darling

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEThe purpose of this study was to verify the safety and accuracy of the Neuromate stereotactic robot for use in deep brain stimulation (DBS) electrode implantation for the treatment of hyperkinetic movement disorders in childhood and describe the authors’ initial clinical results.METHODSA prospective evaluation of pediatric patients with dystonia and other hyperkinetic movement disorders was carried out during the 1st year after the start-up of a pediatric DBS unit in Barcelona. Electrodes were implanted bilaterally in the globus pallidus internus (GPi) using the Neuromate robot without the stereotactic frame. The authors calculated the distances between the electrodes and their respective planned trajectories, merging the postoperative CT with the preoperative plan using VoXim software. Clinical outcome was monitored using validated scales for dystonia and myoclonus preoperatively and at 1 month and 6 months postoperatively and by means of a quality-of-life questionnaire for children, administered before surgery and at 6 months’ follow-up. We also recorded complications derived from the implantation technique, “hardware,” and stimulation.RESULTSSix patients aged 7 to 16 years and diagnosed with isolated dystonia (DYT1 negative) (3 patients), choreo-dystonia related to PDE2A mutation (1 patient), or myoclonus-dystonia syndrome SGCE mutations (2 patients) were evaluated during a period of 6 to 19 months. The average accuracy in the placement of the electrodes was 1.24 mm at the target point. At the 6-month follow-up, patients showed an improvement in the motor (65%) and functional (48%) components of the Burke-Fahn-Marsden Dystonia Rating Scale. Patients with myoclonus and SGCE mutations also showed an improvement in action myoclonus (95%–100%) and in functional tests (50%–75%) according to the Unified Motor-Rating Scale. The Neuro-QOL score revealed inconsistent results, with improvement in motor function and social relationships but worsening in anxiety, cognitive function, and pain. The only surgical complication was medial displacement of the first electrode, which limited intensity of stimulation in the lower contacts, in one case.CONCLUSIONSThe Neuromate stereotactic robot is an accurate and safe tool for the placement of GPi electrodes in children with hyperkinetic movement disorders.

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German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM)

Cited by 32 publications

References 43 publications

Deep brain stimulation for pediatric dystonia: a meta‐analysis with individual participant data

Deep brain stimulation for pediatric dystonia: a meta‐analysis with individual participant data

Recognizing the Common Origins of Dystonia and the Development of Human Movement: A Manifesto of Unmet Needs in Isolated Childhood Dystonias

Frameless robot-assisted pallidal deep brain stimulation surgery in pediatric patients with movement disorders: precision and short-term clinical results

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