Eur J Paediatr Neurol
November 2024
The Publisher regrets that this article is an accidental duplication of an article that has already been published in < European Journal of Paediatric Neurology, 53C (2024) 3209, http://10.1016/j.ejpn.
View Article and Find Full Text PDFArch Dis Child Educ Pract Ed
May 2024
Dystonia is a common disorder of movement and tone, characterised by sustained or intermittent muscle contractions causing abnormal movements, postures or both. Children and young people with dystonia can experience episodes of acute worsening tone, which require prompt treatment. When most severe, dystonia may become life-threatening-a state called 'status dystonicus'.
View Article and Find Full Text PDFDev Med Child Neurol
September 2024
This commentary is on the clinical practice guideline by Fehlings et al. on pages 1133–1147 of this issue.
View Article and Find Full Text PDFParkinsonism Relat Disord
July 2023
Background: Status Dystonicus (SD) represents the most severe end of the spectrum of dystonia. We aimed to explore whether reported features of cases of SD have changed over time.
Methods: A systematic review of cases of SD reported from 2017 to 2023 and comparison of features to data extracted from 2 previous literature reviews (epochs 2012-2017 and pre-2012).
Hypertonia in childhood may arise because of a variable combination of neuronal and non-neuronal factors. Involuntary muscle contraction may be due to spasticity or dystonia, which represent disorders of the spinal reflex arch and of central motor output respectively. Whilst consensus definitions for dystonia have been established, definitions of spasticity vary, highlighting the lack of a single unifying nomenclature in the field of clinical movement science.
View Article and Find Full Text PDFArch Dis Child
September 2023
Elevated tone (hypertonia) is a common problem in children with physical disabilities. Medications intended to reduce tone often have limited efficacy, with use further limited by a significant side effect profile. Consequently, there has been growing interest in the application of Neurosurgical Interventions for the Management of Posture and Tone (NIMPTs).
View Article and Find Full Text PDFThere is a lack of imaging markers revealing the functional characteristics of different brain regions in paediatric dystonia. In this observational study, we assessed the utility of [18F]2-fluoro-2-deoxy-D-glucose (FDG)-PET in understanding dystonia pathophysiology by revealing specific resting awake brain glucose metabolism patterns in different childhood dystonia subgroups. PET scans from 267 children with dystonia being evaluated for possible deep brain stimulation surgery between September 2007 and February 2018 at Evelina London Children's Hospital (ELCH), UK, were examined.
View Article and Find Full Text PDFParkinsonism Relat Disord
December 2022
Objectives: In adults with dystonia Probabilistic Stimulation Mapping (PSM) has identified putative "sweet spots" for stimulation. We aimed to apply PSM to a cohort of Children and Young People (CYP) following DBS surgery.
Methods: Pre-operative MRI and post-operative CT images were co-registered for 52 CYP undergoing bilateral pallidal DBS (n = 31 genetic/idiopathic dystonia, and n = 21 Cerebral Palsy (CP)).
Eur J Paediatr Neurol
November 2022
Background: The COVID-19 pandemic resulted in an unprecedented societal and healthcare global crisis. Associated changes in regular healthcare provision and lifestyle through societal lockdown are likely to have affected clinical management and well-being of children/young people with neurodisability, who often require complex packages of multidisciplinary care.
Methods: We surveyed 108 families of children/young people with severe physical neurodisability and multiple comorbidities to understand how the pandemic had affected acute clinical status, routine healthcare provision, schooling and family mental and social well-being.
Aim: To evaluate clinicians' perspectives on the impact of 'lockdown' during the COVID-19 pandemic for children and young people with severe physical neurodisability and their families.
Method: Framework analysis of comments from families during a recent service review was used to code the themes discussed according to the World Health Organization International Classification of Functioning, Disability and Health (ICF) and interpreted into emergent themes to summarize the impact of lockdown (Stage 1). They were presented to a clinician focus group for discussion (consultants and physiotherapists working in a specialist motor disorders service, [Stage 2]).
Background: Intrathecal baclofen (ITB) is a useful treatment for hypertonia where non-invasive treatments have been ineffective or poorly tolerated. There is an absence of national guidance on selection criteria and a lack of literature regarding patient characteristics and treatment details for children and young people (CYP) receiving ITB therapy in the UK and Ireland. We aimed to gather patient and treatment characteristics for CYP receiving ITB in the UK and Ireland.
View Article and Find Full Text PDFEur J Paediatr Neurol
September 2020
Unlabelled: Rett syndrome (RTT) is neurodevelopmental disorder affecting approximately 1:10000-15000 live female births, commonly associated with MECP2 gene mutations. Hand stereotypies and gait disturbance, as well as spasticity and dystonia, were noted in RTT since first descriptions. This review aimed to explore the prevalence of reported movement disorders in RTT.
View Article and Find Full Text PDFAim: To establish the prevalence of dystonic pain in children and their response to deep brain stimulation (DBS).
Method: Dystonic pain was assessed in a cohort of 140 children, 71 males and 69 females, median age 11 years 11 months (range 3y-19y 1mo), undergoing DBS in our centre over a period of 10 years. The cohort was divided into aetiological dystonia groups: 1a, inherited; 1b, heredodegenerative; 2, acquired; and 3, idiopathic.
Arch Dis Child Educ Pract Ed
October 2020
Elevated tone (hypertonia) is a common problem seen in the paediatric clinic. For most children and young people, hypertonia is just one aspect of a broader disorder of movement and posture. This paper describes a clinical approach to the management of hypertonia in children, considering the contribution of high tone to the functional problems experienced by the child, the potential adverse effects of reducing tone, side effects of the intervention and the importance of setting objectives/goals for intervention which can be measured at follow-up.
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