Charlie Fairhurst

Botulinum toxin type A injections can be an effective treatment for pain in children with hip spasms and cerebral palsy

Aim  Botulinum toxin type A (BoNT‐A) injections were used in the treatment of lower‐limb spasticity in children with cerebral palsy (CP). Anecdotal evidence suggests a reduction in pain after this treatment in children who had pain localized to a displaced hip joint. We report on our current clinical practice.

Management of drooling in children

Drooling beyond the age of 4 years is neurodevelopmentally abnormal. Chronic “sialorrhoea” is seen in children with abnormal oral sensation and/or motor control and more infrequently when there is excessive production of saliva. Salivary production from the paired glands is under autonomic parasympathetic control. Management of the problem relies on multidisciplinary teams with a focus on assessment and when appropriate conservative interventions, oral motor training, dental appliances, medical and surgical treatment programs. Medically, the focus is on modifying the neuroglandular control of saliva with the use of anticholinergic agents. The article covers these areas of background, assessment and management in detail.

Dystonia Severity Action Plan: a simple grading system for medical severity of status dystonicus and life-threatening dystonia

SIR–Dystonia is the most common movement disorder seen in childhood and may be defined as ‘a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both.’ Dystonia may arise from a range of pathologies, and impairs quality of life, participation, and function, in addition to causing pain and progressive deformity. Children and young people with dystonia may experience acute deterioration in their dyskinesia, variously termed ‘status dystonicus’ or ‘dystonic storm’, which has been broadly defined as ‘generalized, intense and potentially fatal exacerbation of muscle contractions.’ This definition is not universally used though, and the precise delineation of the point at which status dystonicus is reached is far less objective than, for example, in status epilepticus. It is important to remember that these acute episodes actually represent the severest end of a continuum of worsening dystonia. Early recognition may potentially facilitate intervention and prevent progression. In a recently reported large retrospective case series of 89 episodes of status dystonicus (58.8% occurring in children <15y) first-line pharmacological treatments of established status dystonicus (most commonly anticholinergics and tetrabenazine) were effective in ~10% of cases. Treatment in this case series frequently necessitated deep sedation in intensive care and there carried a mortality rate of 10.3%. Numerous scales exist to measure the severity of dystonia (e.g. the Burke Fahn Marsden Dystonia Rating Scale, Barry-Albright Dystonia scale). The limitation of these scales include their time-consuming nature, need for considerable training for use, a plateau affect which limits their utility in the most severely affected cases, and also concerns about reliability and smallest detectable difference, particularly in the secondary dystonias which are most commonly seen in childhood. The recently described Dyskinesia Impairment Scale holds great promise, but the length of time taken to record and score video assessments may restrict its use to the research setting. The need exists for a pragmatic criterion-referenced clinical scoring system which can be rapidly and reliably applied and which may be understood by parents and all healthcare providers, not just those professionals with an expertise in movement disorders. It is important that such a scale includes graduations up to the point at which a child or young person can be said to have entered status dystonicus, to alert clinicians to a gradual worsening of dystonia and the need for effective intervention. A common agreed nomenclature when describing exacerbations of dystonia is also necessary if clinical trials are to be developed to best guide treatment methodologies. Between our centres we have devised a simple scoring system (shown in Table I) in an effort to meet this need. The system has been devised to provide simple objective clinical criteria against which the severity of a child’s dystonia may be rated and action taken. This system has been specifically designed to be used as a tool by all health care professionals involved in the clinical care of children with dystonia, and not just the movement disorder specialist. To determine the utility of our scoring system, 10 clinical vignettes (Appendix S1; online supporting information) based on real cases managed at our centre, were provided to a mixed group of 30 health care professionals, including nursing staff, allied health professionals, and paediatricians of different grades and specialities. Each participant could score all 10 cases, with average intraclass correlation scores of 0.993 (p<0.001) indicating a high level of intrarater reliability. Furthermore, all 30 participants thought that the system would be of use during their routine clinical practice. Carers, health professionals, paediatricians, neurologists, and neurodisability consultants need to communicate effectively when managing children with worsening dystonia. This simple scale based on commonly seen clinical indicators could help plan treatment and guide where care should be delivered, for example in the ward, high-dependency, or intensive care setting. It may help focus targeted intervention and develop a picture of the child’s overall disease trajectory over time while allowing measurement of the health costs of brittle, unstable dystonia, and status dystonicus.

Speech, communication and use of augmentative communication in young people with cerebral palsy: The SH&PE population study

BACKGROUND Communication is frequently impaired in young people (YP) with bilateral cerebral palsy (CP). Important factors include motoric speech problems (dysarthria) and intellectual disability. Augmentative and Alternative Communication (AAC) techniques are often employed. The aim was to describe the speech problems in bilateral CP, factors associated with speech problems, current AAC provision and use, and to explore the views of both the parent/carer and young person about communication. METHODS A total population of children with bilateral CP (n = 346) from four consecutive years of births (1989-1992 inclusive) with onset of CP before 15 months were reassessed at age 16-18 years. Motor skills and speech were directly assessed and both parent/carer and the young person asked about communication and satisfaction with it. RESULTS Sixty had died, eight had other conditions, 243 consented and speech was assessed in 224 of whom 141 (63%) had impaired speech. Fifty-two (23% of total YP) were mainly intelligible to unfamiliar people, 22 (10%) were mostly unintelligible to unfamiliar people, 67 (30%) were mostly or wholly unintelligible even to familiar adults. However, 89% of parent/carers said that they could communicate 1:1 with their young person. Of the 128 YP who could independently complete the questions, 107 (83.6%) were happy with their communication, nine (7%) neither happy nor unhappy and 12 (9.4%) unhappy. A total of 72 of 224 (32%) were provided with one or more types of AAC but in a significant number (75% of 52 recorded) AAC was not used at home, only in school. Factors associated with speech impairment were severity of physical impairment, as measured by Gross Motor Function Scale level and manipulation in the best hand, intellectual disability and current epilepsy. CONCLUSIONS In a population representative group of YP, aged 16-18 years, with bilateral CP, 63% had impaired speech of varying severity, most had been provided with AAC but few used it at home for communication.

Cerebral palsy: the whys and hows

The descriptive term of cerebral palsy encompasses the largest group of childhood movement disorders. Severity and pattern of clinical involvement varies widely dependent on the area of the central nervous system compromised. A multidisciplinary team approach is vital for all the aspects of management to improve function and minimise disability. From a medical viewpoint, there are two pronged approaches. First a focus on developmental and clinical comorbidities such as communication, behaviour, epilepsy, feeding problems, gastro-oesophageal reflux and infections; and second on specifics of muscle tone, motor control and posture. With regards to the latter, there is an increasing number of available treatments including oral antispasticity and antidystonic medications, injectable botulinum toxin, multilevel orthopaedic and neurosurgical options and a variety of complementary and alternative therapies.

Physical and neurodevelopmental outcomes in children with single-ventricle circulation

Objective To investigate longer-term physical and neurodevelopmental outcomes of patients with hypoplastic left heart syndrome (HLHS) compared with other patients with functionally single-ventricle circulation surviving beyond the age of 10 years. Design A retrospective, observational study from a UK tertiary centre for paediatric cardiology. Results 58 patients with HLHS and 44 non-HLHS patients with single-ventricle physiology were included. Subjective reduction in exercise tolerance was reported in 72% (95% CI 61% to 84%) of patients with HLHS and 45% (31% to 60%) non-HLHS patients. Compared with non-HLHS patients, educational concerns were reported more frequently in patients with HLHS, 41% (29% to 54%) vs 23% (10% to 35%), as was a diagnosis of a behaviour disorder (autism or attention deficit hyperactivity disorder) 12% (4% to 21%) vs 0%, and referral to other specialist services 67% (55% to 79%) vs 48% (33% to 63%). Conclusions Within a group of young people with complex congenital heart disease, those with HLHS are likely to have worse physical, psychological and educational outcomes.

Fifteen-minute consultation: Modern-day art and science of managing cerebral palsy

While there remains limited intervention to address the damage to the developing brain, current multidisciplinary management of cerebral palsy (CP) needs to minimise the impact of secondary musculoskeletal complications. A focus on comorbidities to maximise function for activity and participation by supporting the child and family in their environment is required. Comprehensive clinical guidance was published by National Institute for Health and Care Excellence (NICE) earlier this year. This article aims to provide a practical clinical approach to the child and family based on:(1) art: empathy, listening and weighing up the clinical picture of the child and family in context; diagnosis, the need for support and space; and care coordination at the right time; and (2) science: the current science in CP care is rapidly expanding in terms of plasticity, pathophysiology, functional assessments and treatments.

Editorial commentary on ‘Toolbox of multi-item measures aligning with ICF core sets for children and youth with cerebral palsy’

We all know that choosing the most appropriate test from the wide variety of measures devised to assess function and interventional outcomes in children and young people with cerebral palsy can be confusing for both researchers and busy clinicians alike. Dr Schiaritis group has provided a comprehensive and robust overview of the current English Language options in line with the ICF framework. As part of this process they also, appropriately, highlight the important role of using validated measures to help us collaborate on an international perspective. It is however important that any comparative data is used to improve services and policy development rather than provide yet another level of managerial hierarchy. When used appropriately by clinicians, they can only help us all develop clear patient algorithms and pathways of care to optimise clinical health and development. In their paper the team show a clear logical progression in the identification, categorization and then linkage of validated multi itemmeasures across chronological and developmental spectrums and also across their 5 ICF and Health core sets, previously outlined. The development of any ‘toolbox’ is primarily of use in research; those of us working in a busy clinical practice are more challenged, not least from a time perspective, but also, as they recognise, in resources. Routine service provision rarely allows half an hour to focus on bimanual function, and another hour for gross motor function, but any categorisation of areas within an internationally recognised framework helps us in the process of communication within and between services. The final aspect of the study focussing on time, access, equity and finance proposed a limited core set aligned across health e was aimed at answering this problem of clinical utility. Trying to answer such complex and important