Historical Developments In Children's Deep Brain Stimulation
Brain Depth Stimulation; Dystonia; Pediatrics; Adult; Basal Ganglion; Central Nervous System; Child; Clinical Feature; Clinical Outcome; Clinical Study; Degenerative Disease; Dystonia/su [surgery]; Dystonic Disorder/su [surgery]; Globus Pallidus; Human; Medical History; Myoclonus; Myoclonus Dystonia/su [surgery]; Nerve Cell Network; Nerve Conduction; Neuromodulation; Palliative Therapy; Priority Journal; Review; Side Effect; Subthalamic Nucleus; Surgery; Symptom; Thalamus; Thalamus Nucleus
Background Heterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies. Methods and Results Following lesional surgery to basal ganglia (BG) and thalamus, deep brain stimulation (DBS) is a more conservative and adjustable intervention to and validated for internal segment of the globus pallidus (GPi), highly efficient in treating isolated "primary" dystonia and associated symptoms such as subcortical myoclonus. The role of DBS in acquired, neurometabolic and degenerative disorders with dystonia deserves further exploration to confirm as an efficient and lasting therapy. However, the pathobiological background with distribution of the sequellae over the central nervous system and related clinical features, will limit DBS efficacy in these conditions. Cumulative arguments propose DBS in severe life threatening dystonic conditions called status dystonicus as first line therapy, irrespective of the underlying cause. There are no currently available validated selection criteria for DBS in pediatric dystonia. Concurrent targets such as subthalamic nucleus (STN) and several motor nuclei of the thalamus are under exploration and only little information is available in children. DBS programming in paediatric population was adopted from experience in adults. The choice of neuromodulatory DBS parameters could influence not only the initial therapeutic outcome of dystonic symptoms but also its maintenance over time and potentially the occurrence of DBS related side effects. Conclusion DBS allows efficient symptomatic treatment of severe dystonia in children and advances pathophysiological knowledge about local and distributed abnormal neural activity over the motor cortical-subcortical networks in dystonia and other movement disorders. Copyright © 2016 The Authors
Cif L; Coubes P
European Journal Of Paediatric Neurology
2017
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10.1016/j.ejpn.2016.08.010
End Of Life Care In Paediatric Neurodegenerative Disease: A Regional Experience
Degenerative Disease; Terminal Care; Case Study; Child; Clinical Article; Controlled Study; England; Family; Hospital; Human; Myoclonus; Neurodegenerative Diseases; Neurology; Only Child; Palliative Care; Palliative Therapy; Personal Experience; Rare Disease; Seizure; Social Welfare; Symptom; Terminal Care
Objectives: To identify barriers to delivering patient and family centred end of life care to children with neurodegenerative disease, of which seizures and myoclonic epilepsy is a significant symptom. To highlight good practice in delivery of end of life care to this patient population. To highlight the unique and specific challenges of end of life care in neurodegenerative disease. Introduction: New Children and Young Persons Advanced Care Planning Guidance has been introduced in 2015. The aim is to improve collaborative working between health care services for children and to create a clear, safe and effective platform from which to provide palliative care. In view of this, consideration has been given to the experience of children and families with neurodegenerative disease in different parts of the Northwest of England. These children, like many other cohorts of children with rare or chronic disease, have unique and specific needs. Methods: This case-series describes the end of life care provided to families in different regions of the Northwest. Case notes of six patients who died between 2013 and 2015 and who received input from the Paediatric Neurology Department at Royal Manchester Children's Hospital, were reviewed. All patients had a neurodegenerative disease with seizure or myoclonus as a significant symptom. Case notes were reviewed systematically with use of a developed proforma. Results: The review showed the most effective end of life care varied by geographical region likely reflecting provision of community services. Those patients with access to dedicated and engaged community and tertiary centre teams who worked in collaboration had the most effective end of life experience. Conclusions: Children with neurodegenerative disease do have unique and specific end of life needs, however, fundamental to effective end of life care is a care multi-agency cohesive and accessible team.
Donald A; Kauffmann L; Ram D; Vassallo G
Developmental Medicine And Child Neurology
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).