Quantification of head and trunk control for children with neuromotor and neuromuscular disorders

点击翻译按钮获取中文摘要

Head and trunk control (trunk control) is mandatory for effective performance of everyday functional activities such as use of vision, sitting or any upper limb activity. Children with cerebral palsy (CP), spinal muscular atrophy (SMA) and inherited neuromuscular disorders (NMD) experience impairments in trunk control which are a fundamental component of their condition.Children with CP show a direct relationship between poor trunk control (orienting the head, sitting balance) and compromised function including communication by eye gaze, use of both hands for learning, play and social development, mobility and urinary incontinence. Children with more severe CP regress in their functional abilities from age 7 years. Accurate diagnosis of trunk control can enable individualised physiotherapy, when combined with treatment as usual, to improve general motor function even in severe CP (preprint). An accurate, objective tool is needed to provide evidence encompassing multiple therapies and centres.Infants with SMA1 have similar difficulty acquiring trunk control as do children with severe CP. Using Nusinersen, these children now survive, but this drug is expensive. An accurate, objective test is needed to monitor effectiveness of this and alternative drugs.The increasing muscle weakness in children with NMD (SMA2, congenital myopathy, congenital muscular dystrophy, Duchenne muscular dystrophy) leads to deterioration in trunk control with subsequent scoliosis. Objective measurement sensitive enough to detect changes in trunk control, pre-scoliosis, would enable appropriate treatment such as physiotherapy at the optimal time. Our hypothesis is that segmental trunk control can be measured objectively and efficiently, classified to seven segmental levels. This objective measurement would promote benefits in terms of new understanding of the mechanisms of trunk control, monitoring treatment and providing evidence to inform practice for all therapy strategies that aim to improve postural control and associated motor function. All current assessments suitable for use in a clinic are validated but rely on clinical judgement that must be maintained over time and between therapists, and many cannot be used for children unable to sit by themselves. They also consider the trunk as a single unit, thus losing the accurate and detailed assessment required for specialised therapy. We anticipate this objective assessment tool could lead to interventions to enhance trunk control, and thus improvement of functional skills, for thousands of children worldwide including those with greater severity of CP.Our project will i) develop live imaging analysis technology to automate the assessment of trunk control in sitting, ii) provide a cost effective and accessible tool usable within any clinic without increasing assessment time and iii) publish an online interactive database disseminating a public standard, a reference and a training resource for measuring trunk control, increasing understanding and enhancing expertise. Assessments of trunk control of children in sitting (400 CP, 20 SMA1, 40 typically developing (TD), 40 NMD) will be recorded using 3D cameras available for the home market. This dataset will include examples of all trunk control problems, body morphology and height that the clinical tool would be required to meet. Machine learning methods will be used to train and validate the automated diagnosis. The tool will be validated against expert clinical assessment and deployed as a laptop, software and two cameras, suitable for use by clinical staff in a routine clinical environment. To prepare power calculations necessary to plan clinical trials, we will collect a longitudinal dataset of 20 children with CP and 5 infants with SMA 1 during a twelve-month period.