Janice Pearse

Early intervention to improve hand function in hemiplegic cerebral palsy

Children with hemiplegic cerebral palsy often have marked hand involvement with excessive thumb adduction and flexion and limited active wrist extension from infancy. Post-lesional aberrant plasticity can lead to progressive abnormalities of the developing motor system. Disturbances of somatosensory and visual function and developmental disregard contribute to difficulties with hand use. Progressive soft tissue and bony changes may occur, leading to contractures, which further limit function in a vicious cycle. Early intervention might help to break this cycle, however, the precise nature and appropriateness of the intervention must be carefully considered. Traditional approaches to the hemiplegic upper limb include medications and botulinum toxin injections to manage abnormalities of tone, and surgical interventions. Therapist input, including provision of orthoses, remains a mainstay although many therapies have not been well evaluated. There has been a recent increase in interventions for the hemiplegic upper limb, mostly aimed outside the period of infancy. These include trials of constraint-induced movement therapy (CIMT) and bimanual therapy as well as the use of virtual reality and robot-assisted therapy. In future, non-invasive brain stimulation may be combined with therapy. Interventions under investigation in the infant age group include modified CIMT and action observation therapy. A further approach which may be suited to the infant with thumb-in-palm deformity, but which requires evaluation, is the use of elastic taping. Enhanced cutaneous feedback through mechanical stimulation to the skin provided by the tape during movement has been postulated to modulate ongoing muscle activity. If effective, this would represent a low-cost, safe, widely applicable early intervention.

Effect of parent‐delivered action observation therapy on upper limb function in unilateral cerebral palsy: a randomized controlled trial

To determine whether home‐based, parent‐delivered therapy comprising action observation (AO) and repeated practice (RP) improves upper limb function more than RP alone in children with unilateral cerebral palsy (UCP).

The Tyneside Pegboard Test: development, validation, and observations in unilateral cerebral palsy

The aims of this study were twofold: first, to develop and validate a timed test of unimanual and bimanual dexterity suitable for those with disability affecting hand function; second, to explore relationships between unimanual and bimanual completion times.

Publishing protocols for trials of complex interventions before trial completion – potential pitfalls, solutions and the need for public debate

BackgroundOpen Science is ‘the movement to make scientific research, data and dissemination accessible to all levels of an inquiring society’.In the spirit of the Open Science movement, advance publication of protocols for clinical trials is now being advocated by BioMed Central, BMJ Open and others. Simultaneously, participants are becoming increasingly active in their pursuit and sharing of trial- and health- related information. Whilst access to protocols alongside published trial findings has clear benefits, advance publication of trial protocols is potentially problematic for trials of complex behavioural interventions. In this article we explain, with examples, how this could lead to unblinding, ‘contamination’ between intervention and control groups and deliberate biasing of assessment outcomes by participants. We discuss potential solutions and demonstrate the need for public debate about how this issue is best managed.ConclusionTriallists may still be underestimating participants’ interest in information. This needs to change: joint and open discussions with the public are needed to inform how we should proceed.

How does the assisting hand assessment relate to relative upper limb movement measured by actigraphy in children with hemiplegia

Objective: To report unique data about children with ALD in a UK-wide prospective study. Methods: Paediatricians notify children with progressive intellectual and neurological deterioration (PIND) via the British Paediatric Surveillance Unit Results: Between April 1997 and May 2014 the PIND study identified 74 boys with ALD. 55 had neurological symptoms, age at presentation: 2–15 years 4 months (median 6yr). Family history was positive in 27, with 13 siblings from 6 sibships; 1 boy had 2 brothers with asymptomatic ALD. There was consanguinity in 3. 46 were white, 5 other ethnicity. Presenting symptoms in 55 symptomatic ALD cases: 42 with deterioration in intellect and/or behaviour, 8 acutely (3: vomiting, 1: recurrent infections, 1: febrile encephalopathy: 1 hemi-seizures,1: collapse and 1: “acutely”),1 with an abnormal gait, 1 with “delayed development” and 3 via positive family history. 21 had visual disturbances, 11 developed seizures. 28 had CT scans: 8 showed a leukoencephalopathy, 8 were normal, 3 showed cerebral infarction and 3 non-specific abnormalities (results not available: 6). 54 had MRI scans: 47 showed a leukoencephalopathy (plus infarct in 2), 2 were normal, 1 showed an infarct (result not available: 4). VLCFAs were abnormal in 52, 53 had synacthen tests which were abnormal in 37 and normal in 3 (results not available:13). Diagnosis was confirmed by genetic studies in 20. Lorenzo’s oil was prescribed for 18. The 19 boys with asymptomatic ALD had a positive family history in 15 including 6 siblings from 3 sibships. 5 had brothers with symptomatic ALD. Conclusions: 8 cases presented acutely without neurological symptoms; some CTs, MRIs and synacthen tests were normal at presentation; some were identified via the family history and cases were increasingly diagnosed using genetic studies. Acknowledgment: Independent research funded by Department of Health (DH) Policy Research Programme [121/6443]. Views expressed not necessarily those of DH. Clinical phenotype of a novel mitochondrial disorder associated with mutations in MICU1 AM CHILDS, C LOGAN, F MUNTONI, G SZABADKAI, M DUCHEN, R PHADKE, C SEWRY, K PYSDEN, H ROPER, G CHOW, E NIKS, M KRIEK, D LEWIS-SMITH, P CHINNERY, E SHERIDAN Department of Paediatric Neurology, Leeds Children’s Hospital; Leeds Institute of Biomedical and Clinical Science, St James’s University Hospital, Leeds, UK; UCL Institute of Child Health, Dubowitz Neuromuscular Centre and Medical Research Council Centre for Neuromuscular Disease, London; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research, University College London, London; Department of Paediatrics, Birmingham Heartlands Hospital, Birmingham, UK; Department of Paediatric Neurology, Queen’s University Hospital, Nottingham; Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands; Mitochondrial Research Group, Institute of Clinical Genetics, Newcastle University

Raising Awareness of the Myth of the “Unaffected Hand” in Childhood Hemiplegia

Background and aims: The term ‘hemiplegia’ implies a unilateral deficit, yet several studies have demonstrated abnormal limb control ipsilateral to the brain lesion. This is under-recognised by clinicians and has implications for function and therapy. We documented deficits in the “unaffected” hand in children with hemiplegia using a dexterity test developed by our group for use in children with motor disorders.Methods: Participants: 33 children (4-11y) with hemiplegia (18 male; 19 left hemiplegia); 66 age, sex and handedness matched controls. Assessment: Adapted 9-hole pegboard test (2 adjacent boards; 3 peg sizes; electronically timed). Analysis: ANOVA (between-group factors group (hemiplegia/control), age (4-5, 6-7, 8-11), sex, dominant hand) performed separately for each peg size.Results: Some hemiplegic children with radiologically confirmed unilateral lesions had prolonged pegboard completion times whereas others scored similarly to controls (Figure: filled symbols - hemiplegia). For all peg sizes, main effects of group (p< 0.001) and age (p< 0.001) were significant but sex and dominant hand were not. There was a significant interaction between group and age (p< 0.005), with smaller difference in group means in the oldest age-group. This deserves exploration with a longitudinal study.FigureConclusion: The “unaffected” hand in children with hemiplegia is less dextrous than the dominant hand of controls. Therapy to improve function of the more-affected hand should be designed to achieve optimal outcomes bilaterally.

Current therapeutic management of perinatal stroke with a focus on the upper limb: a cross sectional survey of UK physiotherapists and occupational therapists

Abstract Aim: To determine current UK pediatric physiotherapist (PT) and occupational therapist (OT) management of perinatal stroke. Design: Web-based cross-sectional survey. Methods: Participants were members of the Association of Paediatric Chartered Physiotherapists and Occupational Therapists specialist section: children young people and families working with infants. Items covered prioritization of referrals, assessments, therapy approaches aimed at the upper limb, and parental support. Results: 179 therapists responded. 87.2% of PTs and 63.0% of OTs managed infants with perinatal stroke. Infants with clinical signs of motor dysfunction at referral were prioritized for early initial assessment. The most frequently used assessments were the Alberta Infant Motor Scale (AIMS) and Bayley Scales of Infant Development (BSID). Of PTs and OTs, 41.9 and 40.0% used no standardized assessments. Frequently used therapy interventions were Bobath/Neurodevelopmental Therapy (NDT), positioning aids and passive movements. 88.1% of therapists would choose a bilateral rather than unilateral (affected side) therapy approach for infants with perinatal stroke aged up to 6 months. Of PTs and OTs, 56.9 and 57.1% provided psychological support to families. Conclusions: Assessment and provision of therapy services following perinatal stroke is variable. Increased use of standardized assessments and centralized data collection regarding service provision for high-risk infants is recommended.

Promoting optimal bimanual performance in cerebral palsy

Bimanual performance is the term used to describe how we spontaneously use our two hands in activity. Klevberg et al. examined the development of bimanual performance in a Norwegian population of young children with cerebral palsy (CP). The paper adds to the existing evidence in children with unilateral CP and presents new findings for children with bilateral CP. The latter has only been possible due to the development of the Both Hands Assessment (BoHA). This is a group of children for whom the evidence for the appropriateness and efficacy of interventions is sparse. It is therefore exciting to read that the authors plan to evaluate the role of intensive training on the development of bimanual performance in children with bilateral CP. It is to be applauded that intensive interventions are considered part of current practice for children with unilateral CP in Norway. Sadly, this is not necessarily the case even in other well-resourced countries, despite increasing evidence for the effectiveness of intensive, repeated practice, goal-directed therapy. The reasons why it is not widely achieved are multi-factorial and include: how therapy services are delivered, staffing levels, training, and the availability of resources. Realistically, and in line with the Norwegian model, therapists will need to support parents and education staff to deliver therapy for children to receive an effective dose. It must be challenging for many parents to know what to do: to find sufficient varied activities which are motivating to the child; require active movement at the ‘just right’ level of challenge; and to sustain intervention over time to achieve a high intensity dose. Therapists have a key role in supporting parents to deliver a tailored therapy plan using a combination of modelling, motivational encouragement, and the supply of ideas and advice. It is important that good parent–child relationships are maintained. The required frequency of support may vary by child and family characteristics; actual support may be influenced by geographical constraints and caseload pressures. Therapists could explore using a combination of methods to achieve the required level of support; face-to-face appointments could be supplemented with telephone and text message contact and other technologies such as Skype or FaceTime. Klevberg et al. also identify related and yet unanswered questions such as the accuracy of the Mini-Manual Ability Classification System in very young children, the sensitivity of the BoHA to detect change, and the need to look similarly at the development of hand use in much younger children with unilateral and bilateral CP. This paper makes a very important point regarding the need to develop early interventions which facilitate the long-term development of optimal bimanual performance in these children. The authors conclude that early interventions are needed to promote the early development of bimanual performance. Assessments measuring bimanual performance in infants offer not just a measure of performance to evaluate therapy and guide interventions, but also an opportunity to teach parents how to promote performance in a developmentally appropriate way. Beginning early with parent-delivered therapy may not only help children achieve optimal bimanual performance sooner, but may also equip parents with the knowledge and skills which they need to continue to promote bimanual performance throughout childhood.

Early therapy in perinatal stroke: pilot feasibility trial

BackgroundStructural and functional alterations in white matter architecture are common described in children born preterm (PT) and associated to behavioral implications. Still, how critical gestat ...Introduction: The Gross Motor Function Classification System (GMFCS), the Manual Ability Classification System (MACS) and the Communication Function Classification System (CFCS) have become important tools to describe motor function in children with cerebral palsy (CP). The aim of this study was to describe the relationship between activities of daily living, motor function, and communication in children with CP. Patients and method: Ninety-one children with hemiplegic CP, (age 8y 2.4y; 79% left hemiplegic CP) and between the years 2000–2015 who were accepted to therapy in the Department of Occupational Therapy in Hacettepe University were included in the study. GMFCS, MACS, CFCS, Pediatric Evaluation Disability Inventory (PEDI), and Functional Independence Measure for Children (WeeFIM) was evaluated by the child’s therapist. Results: There was a good overall correlation between gross motor function and manual ability (r=0.754), gross motor function and communication (r=0.649), and manual ability and communication (r=0.779, p<0.001). In addition, there was a similar relationship between these three classification and assessment of daily living activities (r= 0.414 to r= 0.716 for WeeFIM; r=0.354–0.766 for PEDI; p<0.001). Conclusion: When evaluating children with CP, all three approaches must be assessed before complete clinical status is determined. In this context, the GMFCS, MACS, and CFCS seem to be well informed about daily living activities, and population-based studies seem to be very useful for health records and clinical practice for children with hemiplegic CP.

Fifteen-minute consultation: Management of the upper limb in unilateral cerebral palsy

Children with unilateral cerebral palsy (UCP) have complex health, education and social care needs. Delayed gross motor milestones are the most common presenting feature, and much of the early management focuses on gross motor skills and lower limb management. In later childhood, adolescence and adulthood, upper limb function has significant impact on activity, participation and independence. There is clear pathophysiological rationale and emerging clinical evidence that earlier intervention to improve upper limb function is beneficial. Whereas most children with UCP are managed in secondary care, it is recommended that the assessment and delivery of specialist intervention for the upper limb occurs at a regional centre.