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Early markers for cerebral palsy: Insights from the assessment of general movements

Authors:
  • University Medical Center Goettingen / Karolinska Institutet / Medical University of Graz

Abstract

Overt clinical symptoms of cerebral palsy do not emerge before a child is at least half a year old. Among the most reliable early markers for cerebral palsy are abnormal 'general movements' (GMs). Two specific abnormal GM patterns predict cerebral palsy: cramped-synchronized GMs (during preterm and term age), which lack the usual smoothness and fluent character; and limb and trunk muscles contract almost simultaneously and relax almost simultaneously. In addition, the absence of so-called fidgety movements at 3-5 months post-term age. Fidgety movements are small movements of the neck, trunk and limbs in all directions and of variable acceleration. Beside a high sensitivity (>91%) and specificity (>81%), the assessment of GMs is quick, nonintrusive and easy to acquire.
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... The 2018 review demonstrated a high sensitivity of 93% (95% confidence interval (CI) 86-96) but a low specificity for CP 59% (95% CI with the GMA (using Prechtl scoring algorithm) at 6 weeks (writhing phase). Later assessments (10-20 weeks), from the fidgety period (evaluating for the presence and quality of fidgety movements (FMs)), however, demonstrated a better sensitivity of 97% (CI 95% 93-99) and a specificity of 89% (95% CI [83][84][85][86][87][88][89][90][91][92][93] [16]. The conclusion was those later assessments in infancy with the quality of FMs as assessed by GMA had the strongest predictive value for CP. ...
... The evidence is lacking for the utilization of the GMA in these populations treated with therapeutic hypothermia. Furthermore, of possibly greater potential applicability is the inclusion of the MOS as a predictor of CP [92,93] in term and late preterm infants with NE when treated with therapeutic hypothermia. ...
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Background The General Movements Assessment is a non-invasive and cost-effective tool with demonstrated reliability for identifying infants at risk for cerebral palsy. Early detection of cerebral palsy allows for the implementation of early intervention and is associated with better functional outcomes. No review to date has summarized the utility of the General Movements Assessment to predict cerebral palsy in term and late-preterm infants diagnosed with neonatal encephalopathy. Methods We conducted a scoping review involving infants born greater than or equal to 34 weeks gestational age to identify all available evidence and delineate research gaps. We extracted data on sensitivity, specificity, and positive and negative predictive values and described the strengths and limitations of the results. We searched five databases (MEDLINE, Embase, PsychINFO, Scopus, and CINAHL) and the General Movements Trust website. Two reviewers conducted all screening and data extraction independently. The articles were categorized according to key findings, and a critical appraisal was performed. Results Only three studies, a cohort and two case series, met all of the inclusion criteria. The total number of participants was 118. None of the final eligible studies included late-preterm neonates. All three studies reported on sensitivity, specificity, and positive predictive and negative predictive values. An abnormal General Movement Assessment at 3–5 months has a high specificity (84.6–98%) for cerebral palsy with a similarly high negative predictive value (84.6–98%) when it was normal. Absent fidgety movements, in particular, are highly specific (96%) for moderate to severe cerebral palsy and carry a high negative predictive value (98%) when normal. In the time period between term and 4–5 months post-term, any cramped synchronized movements had results of 100% sensitivity and variable results for specificity, positive predictive value, and negative predictive value. Conclusions A normal General Movements Assessment at 3 months in a term high-risk infant is likely associated with a low risk for moderate/severe cerebral palsy. The finding of cramped synchronized General Movements is a strong predictor for the diagnosis of cerebral palsy by 2 years of age in the term population with neonatal encephalopathy. The deficit of high-quality research limits the applicability, and so the General Movements Assessment should not be used in isolation when assessing this population. Systematic review registration Title registration with Joanna Briggs Institute. URL: http://joannabriggswebdev.org/research/registered_titles.aspx .
... CP is complex and cyclical-it is caused by a neural lesion which causes downstream musculoskeletal effects that interact with one another and also interact with the natural processes of growth and aging. While impaired muscular development has been observed at around 15 months of age, atypical movements are noticeable as early as 3 months of age [170], potentially implying that muscle morphology is a downstream effect of altered neural activation and biomechanics, rather than a direct consequence of the neural lesion. Separating these issues in a reductive approach is difficult, but advancements in computational modelling and bioengineering approaches [171,172] create new advanced possibilities for simulating and understanding these mechanisms. ...
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Cerebral palsy (CP) is caused by a static lesion to the brain occurring in utero or up to the first 2 years of life; it often manifests as musculoskeletal impairments and movement disorders including spasticity and contractures. Variable manifestation of the pathology across individuals, coupled with differing mechanics and treatments, leads to a heterogeneous collection of clinical phenotypes that affect muscles and individuals differently. Growth of muscles in CP deviates from typical development, evident as early as 15 months of age. Muscles in CP may be reduced in volume by as much as 40%, may be shorter in length, present longer tendons, and may have fewer sarcomeres in series that are overstretched compared to typical. Macroscale and functional deficits are likely mediated by dysfunction at the cellular level, which manifests as impaired growth. Within muscle fibres, satellite cells are decreased by as much as 40–70% and the regenerative capacity of remaining satellite cells appears compromised. Impaired muscle regeneration in CP is coupled with extracellular matrix expansion and increased pro-inflammatory gene expression; resultant muscles are smaller, stiffer, and weaker than typical muscle. These differences may contribute to individuals with CP participating in less physical activity, thus decreasing opportunities for mechanical loading, commencing a vicious cycle of muscle disuse and secondary sarcopenia. This narrative review describes the effects of CP on skeletal muscles encompassing substantive changes from whole muscle function to cell-level effects and the effects of common treatments. We discuss growth and mechanics of skeletal muscles in CP and propose areas where future work is needed to understand these interactions, particularly the link between neural insult and cell-level manifestation of CP.
... La evaluación de los MGs de Prechtl es una alternativa clínica no-invasiva para la valoración postnatal del movimiento en el neonato. Esta valoración permite identificar alteraciones neuro-motrices de manera temprana, como la PC (Einspieler et al. 2012). Además, es un método de fácil aplicación y de bajo costo, que clasifica los movimientos espontáneos fisiológicos de los niños en movimientos WM y FM. ...
... The presence of persistently cramped synchronized writhing movements, followed by the absence of fidgety movements, is the strongest predictor for poor neurodevelopmental outcomes [4]. In particular, it has been reported that observation of poor repertoire pattern seems to be associated with minor neurological dysfunctions [50] and cramped-synchronized movements is highly predictive of the development of cerebral palsy [51]. Current studies indicate that the GMA is the most sensitive and specific test available to allow early detection of CP [52]. ...
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Neurodevelopmental disorders (NDD) are impairments of the growth and development of the brain and/or central nervous system. In the light of clinical findings on early diagnosis of NDD and prompted by recent advances in hardware and software technologies, several researchers tried to introduce automatic systems to analyse the baby’s movement, even in cribs. Traditional technologies for automatic baby motion analysis leverage contact sensors. Alternatively, remotely acquired video data (e.g., RGB or depth) can be used, with or without active/passive markers positioned on the body. Markerless approaches are easier to set up and maintain (without any human intervention) and they work well on non-collaborative users, making them the most suitable technologies for clinical applications involving children. On the other hand, they require complex computational strategies for extracting knowledge from data, and then, they strongly depend on advances in computer vision and machine learning, which are among the most expanding areas of research. As a consequence, also markerless video-based analysis of movements in children for NDD has been rapidly expanding but, to the best of our knowledge, there is not yet a survey paper providing a broad overview of how recent scientific developments impacted it. This paper tries to fill this gap and it lists specifically designed data acquisition tools and publicly available datasets as well. Besides, it gives a glimpse of the most promising techniques in computer vision, machine learning and pattern recognition which could be profitably exploited for children motion analysis in videos.
... Early intervention can optimize neuroplasticity, minimize complications, create suitable learning opportunities, and provide families with adequate psychological support [6]. The use of neuroimaging, Prechtl's General Movement Assessment (GMA), and the Hammersmith Infant Neurological Examination (HINE) in risky infants to identify neurodevelopmental disorders in the earliest period is recommended [7][8][9][10][11][12]. Via these assessment tools separately or together, neurodevelopmental disorders can be predicted at an early stage with high specificity and sensitivity [6]. ...
Article
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Article
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Chapter
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