EEG-based sensorimotor neurofeedback for motor neurorehabilitation in children and adults: A scoping review.

Objective: Therapeutic interventions for children and young people with dystonia and dystonic/dyskinetic cerebral palsy are limited. EEG-based neurofeedback is emerging as a neurorehabilitation tool. This scoping review maps research investigating EEG-based sensorimotor neurofeedback in adults and children with neurological motor impairments, including augmentative strategies.

Pharmacological and neurosurgical management of cerebral palsy and dystonia: Clinical practice guideline update.

Dystonia, typically characterized by slow repetitive involuntary movements, stiff abnormal postures, and hypertonia, is common among individuals with cerebral palsy (CP). Dystonia can interfere with activities and have considerable impact on motor function, pain/comfort, and ease of caregiving. Although pharmacological and neurosurgical approaches are used clinically in individuals with CP and dystonia that is causing interference, evidence to support these options is limited.

Cross-frequency cortex-muscle interactions are abnormal in young people with dystonia.

Sensory processing and sensorimotor integration are abnormal in dystonia, including impaired modulation of beta-corticomuscular coherence. However, cortex-muscle interactions in either direction are rarely described, with reports limited predominantly to investigation of linear coupling, using corticomuscular coherence or Granger causality. Information-theoretic tools such as transfer entropy detect both linear and non-linear interactions between processes.

Dystonia in Childhood: How Insights from Paediatric Research Enrich the Network Theory of Dystonia.

Dystonia is now widely accepted as a network disorder, with multiple brain regions and their interconnections playing a potential role in the pathophysiology. This model reconciles what could previously have been viewed as conflicting findings regarding the neuroanatomical and neurophysiological characteristics of the disorder, but there are still significant gaps in scientific understanding of the underlying pathophysiology. One of the greatest unmet challenges is to understand the network model of dystonia in the context of the developing brain.

Transitional Care for Young People with Movement Disorders: Consensus-Based Recommendations from the MDS Task Force on Pediatrics.

Background: The International Parkinson and Movement Disorders Society (MDS) set up a working group on pediatric movement disorders (MDS Task Force on Pediatrics) to generate recommendations to guide the transition process from pediatrics to adult health care systems in patients with childhood-onset movement disorders.

Methods: To develop recommendations for transitional care for childhood onset movement disorders, we used a formal consensus development process, using a multi-round, web-based Delphi survey. The Delphi survey was based on the results of the scoping review of the literature and the results of a survey of MDS members on transition practices.

Metabolic patterns in brain 18F-fluorodeoxyglucose PET relate to aetiology in paediatric dystonia.

There 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.

Probabilistic mapping of deep brain stimulation in childhood dystonia.

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)).

Transition Services for Children and Young Adults with Movement Disorders: A Survey by the MDS Task Force on Pediatrics.

Background: There is currently very limited data related to transition services for movement disorders.

Objectives: Movement Disorders Society (MDS) Task Force on Pediatrics conducted a survey of current provision of transition for young adults with movement disorders.

Methods: The survey questionnaire was based on review of available evidence, with questions designed to capture service location, transition clinic structure, and core issues discussed.

Harnessing cognitive strategy use for functional problems and proposed underlying mechanisms in childhood-onset dystonia.

Background: There is a significant gap in knowledge about rehabilitation techniques and strategies that can help children and young people with hyperkinetic movement disorders (HMD) including dystonia to successfully perform daily activities and improve overall participation. A promising approach to support skill acquisition is the Cognitive Orientation to daily Occupational Performance (CO-OP) intervention. CO-OP uses cognitive strategies to help patients generate their own solutions to overcome self-identified problems encountered in everyday living.

Highlighting the Dystonic Phenotype Related to GNAO1.

Background: Most reported patients carrying GNAO1 mutations showed a severe phenotype characterized by early-onset epileptic encephalopathy and/or chorea.

Objective: The aim was to characterize the clinical and genetic features of patients with mild GNAO1-related phenotype with prominent movement disorders.

Methods: We included patients diagnosed with GNAO1-related movement disorders of delayed onset (>2 years).

Mental health and behaviour in children with dystonia: Anxiety, challenging behaviour and the relationship to pain and self-esteem.

Purpose: To ascertain whether young people with dystonia are more likely than the general population to have mental health and/or behavioural difficulties, and to explore factors that may contribute to these difficulties.

Method: Using a quasi-experimental design, 50 young people with dystonia aged 7-17 and their carers were recruited from the Evelina London Children's Hospital. Young people completed the Beck Youth Inventories and the Strengths and Difficulties Questionnaire.

Sensorimotor Integration in Childhood Dystonia and Dystonic Cerebral Palsy-A Developmental Perspective.

Dystonia is a disorder of sensorimotor integration, involving dysfunction within the basal ganglia, cortex, cerebellum, or their inter-connections as part of the sensorimotor network. Some forms of dystonia are also characterized by maladaptive or exaggerated plasticity. Development of the neuronal processes underlying sensorimotor integration is incompletely understood but involves activity-dependent modeling and refining of sensorimotor circuits through processes that are already taking place and which continue through infancy, childhood, and into adolescence.

Rehabilitation in childhood-onset hyperkinetic movement disorders including dystonia: Treatment change in outcomes across the ICF and feasibility of outcomes for full trial evaluation.

Background: Childhood-onset hyperkinetic movement disorders (HMD), including dystonia are notoriously difficult to treat and there are limited studies showing successful medical, surgical or non-pharmacological interventions.

Methods: This prospective study used grouped data (n = 22) from two studies of the Cognitive Orientation to daily Occupational Performance (CO-OP) Approach for patient-selected goals. Eligibility included aged 6-21 years, deep brain stimulation in place, with manual ability classification system level I-IV.

The importance of neurology and genetic testing in the patient with non-cleft velopharyngeal dysfunction.

Objective: A significant proportion of the referrals made to a speech investigation clinic in a cleft unit include patients with non-cleft velopharyngeal dysfunction (VPD). This study aims to quantify the underlying diagnoses of these patients and describe the investigative pathway and diagnostic information subsequent to presentation in our clinic.

Materials And Methods: The case notes of 136 consecutive patients with non-cleft VPD who attended our Velopharyngeal Investigation (VPI) clinic from July 2014-December 2019 were reviewed.

Targeting accuracy of robot-assisted deep brain stimulation surgery in childhood-onset dystonia: a single-center prospective cohort analysis of 45 consecutive cases.

Objective: Deep brain stimulation (DBS) is an established treatment for pediatric dystonia. The accuracy of electrode implantation is multifactorial and remains a challenge in this age group, mainly due to smaller anatomical targets in very young patients compared to adults, and also due to anatomical abnormalities frequently associated with some etiologies of dystonia. Data on the accuracy of robot-assisted DBS surgery in children are limited.

Systemic Inflammation Is Associated With Neurologic Involvement in Pediatric Inflammatory Multisystem Syndrome Associated With SARS-CoV-2.

Objective: Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a severe immune-mediated disorder. We aim to report the neurologic features of children with PIMS-TS.

Methods: We identified children presenting to a large children's hospital with PIMS-TS from March to June 2020 and performed a retrospective medical note review, identifying clinical and investigative features alongside short-term outcome of children presenting with neurologic symptoms.

EEG measures of sensorimotor processing and their development are abnormal in children with isolated dystonia and dystonic cerebral palsy.

Dystonia is a disorder of sensorimotor integration associated with abnormal oscillatory activity within the basal ganglia-thalamo-cortical networks. Event-related changes in spectral EEG activity reflect cortical processing but are sparsely investigated in relation to sensorimotor processing in dystonia. This study investigates modulation of sensorimotor cortex EEG activity in response to a proprioceptive stimulus in children with dystonia and dystonic cerebral palsy (CP).

Cognitive Strategy Training in Childhood-Onset Movement Disorders: Replication Across Therapists.

To explore preliminary effectiveness of the Cognitive Orientation to daily Occupational Performance (CO-OP) Approach in improving outcomes in childhood-onset hyperkinetic movement disorders (HMDs) including dyskinetic cerebral palsy following deep brain stimulation (DBS) across UK clinical occupational therapists. Randomized, multiple-baseline, Single Case Experimental Design N-of-1 trial with replications across participants. Five self-selected goals were identified: three goals were worked on during CO-OP and two goals were left untreated and used to assess skills transfer.

Abnormal microscale neuronal connectivity triggered by a proprioceptive stimulus in dystonia.

We investigated modulation of functional neuronal connectivity by a proprioceptive stimulus in sixteen young people with dystonia and eight controls. A robotic wrist interface delivered controlled passive wrist extension movements, the onset of which was synchronised with scalp EEG recordings. Data were segmented into epochs around the stimulus and up to 160 epochs per subject were averaged to produce a Stretch Evoked Potential (StretchEP).