Hammersmith Infant Neurological Examination Research Articles

Telehealth of Infants at Risk During the COVID-19 Pandemic: Physical Therapists’ and Caregiver’s Perceptions and Costs

Aim We aimed to describe telehealth used to detect infants at risk of neurodevelopmental delay and assess the assessors’ and caregivers’ perceptions and costs. Methods This was an observational study in which five physical therapists applied the General Movement Assessment, the neurological exam based on the Hammersmith Infant Neurological Examination, and the Alberta Infant Motor Scale via telehealth in 65 infants at risk of neurologic delay during the COVID-19 pandemic. The perceptions of assessors and caregivers were assessed using a questionnaire. We tabulated the family’s costs (internet access) and the therapist’s expenses (internet access and professional fees). Results In general, assessors felt comfortable and reported good quality of teleassessment. They highlighted the significant effort they and caregivers required for synchronous methods, challenges in clearly guiding caregivers on infant positioning, difficulties in adjusting the camera’s position, and unstable internet connections. Most assessors expressed a willingness to continue using telehealth. Caregivers displayed high satisfaction, and the costs were minimal. Conclusion Teleassessment during the COVID-19 pandemic was feasible, although implementing this method of health service in general clinical practice still requires further scrutiny.

Type 1 spinal muscular atrophy treated with nusinersen in Norway, a five-year follow-up

BackgroundNew treatments for 5q spinal muscular atrophy (SMA) have led to changes in the disease phenotype. Questions about long-term efficacy, however, persist. We present the results from five-year follow-up of the first ten Norwegian patients with SMA type1 treated with nusinersen. Methods– Ten patients referred to the expanded access program were included. Standardized assessments with Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND), the Hammersmith Infant Neurological Examination (HINE-2), compound muscle action potential (CMAP) examination and cerebrospinal fluid analysis of neurofilament light chain (cNfL) were performed. ResultAge at baseline ranged from three months to 11 years and eight months. Nine patients were alive and continued to receive treatment at 62 months of follow-up. CHOP INTEND scores increased significantly up to 38 months. Any further increase from 38 to 50 months was not statistically significant, and scores remained almost unchanged from 50 to 62 months. HINE-2 scores increased but the difference from baseline never reached statistical significance.The youngest patients showed the best motor outcome. The changes in CMAP scores were not statistically significant. cNfL values were significantly reduced after 18 months compared with baseline; the largest difference occurred between baseline and 6 months. There was a significant negative correlation between log cNfL and CHOP INTEND (p = 0.042). Bulbar and respiratory function did not improve during the observation period. ConclusionOur findings support previously reported results on efficacy and safety of nusinersen. All patients have shown improvement in motor function. The need of respiratory and nutritional support did not improve.

Randomized Comparison Trial of Rehabilitation Very Early for Infants with Congenital Hemiplegia

To compare efficacy of constraint-induced movement therapy (Baby-CIMT) with bimanual therapy (Baby-BIM) in infants at high risk of unilateral cerebral palsy (UCP). Single-blind, randomized-comparison-trial that had the following inclusion criteria: (i) asymmetric brain lesion (ii) absent fidgety General Movements, (iii) Hammersmith Infant Neurological Examination below cerebral palsy cut-points, (iv) entry at 3 to 9 months corrected age (CA), and (v) >3 point difference between hands on Hand Assessment Infants (HAI). Infants were randomized to Baby-CIMT or Baby-BIM, which comprised 6 to 9 months of home-based intervention. Daily dose varied from 20 to 40-minutes according to age (total 70-89.2 hours). Primary outcome measure was the HAI post intervention, with secondary outcomes Mini-Assisting Hand Assessment and Bayley III cognition at 24-months CA. 96 infants (51 male, 52 right hemiplegia) born median at 37-weeks' gestation were randomized to Baby-CIMT (n=46) or Baby-BIM (n=50) and commenced intervention at a mean 6.5 (SD 1.6) months CA. There were no between group differences immediately post-intervention on HAI (mean difference [MD] 0.98 HAI units, 95% confidence interval [CI] 0.94-2.91; p=0.31). Both groups demonstrated significant clinically important improvements from baseline to post-intervention (Baby-BIM MD 3.48, 95%CI 2.09-4.87; Baby-CIMT MD 4.42, 95% CI 3.07-5.77). At 24 months, 64 infants were diagnosed with UCP (35 Baby-CIMT, 29 Baby-BIM). Infants who entered the study between 3 and 6-months CA had greater change in HAI Both Hands Sum Score compared with those who entered at ≥6-months CA (MD 7.17, 95% CI 2.93, 11.41, p=0.001). Baby-CIMT was not superior to Baby-BIM, and both interventions improved hand development. Infants commencing intervention at <6 months CA had greater improvements in hand function.

Effect of Virtual Reality on Trunk Control in Children with Developmental Delays

Background: Developmental delays affect motor function and trunk control in children, impacting their overall development and daily activities. Virtual reality (VR) interventions have the potential to enhance motor abilities through engaging, personalized therapies.Objective: This study aimed to determine the effectiveness of virtual reality-based neurodevelopmental treatment with trunk control exercises (VR-NDT-TCE) in improving trunk control and cognitive functions in children with developmental delays.Methods: A single-blinded randomized controlled trial was conducted with 22 children aged 2-8 years diagnosed with developmental delays. Participants were randomly assigned to Group A (NDT-TCE) or Group B (VR-NDT-TCE) for a six-week intervention, three times per week. Trunk control was measured using the Trunk Control Measurement Scale (TCMS), and cognition was assessed through the Hammersmith Infant Neurological Examination (HINE) Performa. Data analysis was performed using SPSS version 27 with paired t-tests and Mann-Whitney tests.Results: Significant improvements were observed in trunk control (TCMS score: 20.18 ± 2.40 in Group A vs. 24.18 ± 4.67 in Group B, p = 0.017). Group B showed notable gains in cognitive parameters, including conscious state (p &lt; 0.001), emotional state (p = 0.012), and social orientation (p = 0.031).Conclusion: VR-NDT-TCE significantly improved trunk control and cognitive outcomes, offering an effective intervention for children with developmental delays.

The Small Step Early Intervention Program for Infants at High Risk of Cerebral Palsy: A Single-Subject Research Design Study.

Background/Objectives: Early interventions for infants at high risk of cerebral palsy (CP) are recommended, but limited evidence exists. Our objective was, therefore, to evaluate the effects of the family-centered and interprofessional Small Step early intervention program on motor development in infants at high risk of CP (ClinicalTrials.gov: NCT03264339). Methods: A single-subject research design was employed to investigate participant characteristics (motor dysfunction severity measured using the Hammersmith Infant Neurological Examination (HINE) and Alberta Infant Motor Scale (AIMS) at three months of corrected age (3mCA) related to intervention response. The repeated measures Peabody Developmental Motor Scales-2 fine and gross motor composite (PDMS2-FMC and -GMC) and Hand Assessment for Infants (HAI) were analyzed visually by cumulative line graphs, while the Gross Motor Function Measure-66 (GMFM-66) was plotted against reference percentiles for various Gross Motor Function Classification System (GMFCS) levels. Results: All infants (n = 12) received the Small Step program, and eight completed all five training steps. At two years of corrected age (2yCA), nine children were diagnosed with CP. The children with the lowest HINE < 25 and/or AIMS ≤ 6 at 3mCA (n = 4) showed minor improvements during the program and were classified at GMFCS V 2yCA. Children with HINE = 25-40 (n = 5) improved their fine motor skills during the program, and four children had larger GMFM-66 improvements than expected according to the reference curves but that did not always happen during the mobility training steps. Three children with HINE = 41-50 and AIMS > 7 showed the largest improvements and were not diagnosed with CP 2yCA. Conclusions: Our results indicate that the Small Step program contributed to the children's motor development, with better results for those with an initial higher HINE (>25). The specificity of training could not be confirmed.

The Impact of Parent-Based Support Programs on Neurodevelopmental Prognosis: Second-Year Results from a Newly Established Neurodevelopmental Follow-Up Unit in a Tertiary Hospital.

This study aims to assess the neurodevelopmental progress of high-risk infants 2 years post implementation of the Neurodevelopmental Follow-Up Unit (NFU) program at our hospital and explore implementation challenges for insights. Infants were assessed using the Hammersmith Infant Neurological Examination (HINE), The Alberta Infant Motor Scale (AIMS), and Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III). A multidisciplinary team provided comprehensive parent education covering neurologic cues, postural advice, and developmental instructions in accordance with the children's assessment findings. In addition, a pediatric physical therapist provided motor development training emphasizing age-appropriate milestones and functional independence, while child development specialists addressed delays identified through BSID-III assessments. A total of 121 high-risk babies were enrolled during a 2-year period. Results revealed that 9 infants exhibited suboptimal HINE scores at 3-4 months, with only 2 maintaining suboptimal scores at 12-15 months. Similarly, 2 infants with suboptimal AIMS scores at 3-4 months reached normal values at 12-15 months. Comparable improvements were observed in BSID-III scores. While no correlation between HINE and AIMS scores was found at the 3-4-month mark, a significant correlation emerged between AIMS and HINE scores at 6-9 months (r = 0.643, P < .001) and 12-15 months (r = 0.820, P < .001). Encouraging early family education alongside regular monitoring of high-risk newborns appears to have a positive impact on their motor and cognitive development. Consideration of clinical recommendations, such as tailored interventions and periodic assessments, may contribute to optimizing developmental outcomes.

Early neurodevelopmental outcomes of preterm infants with intraventricular haemorrhage and periventricular leukomalacia.

Intraventricular haemorrhage (IVH) and periventricular leukomalacia (PVL) in preterm infants are associated with an increased risk of long-term neurodevelopmental impairments (NDI) and cerebral palsy (CP). However, little is known about their impact on early neurodevelopmental outcomes despite increasing evidence highlighting the feasibility and importance of early NDI/CP diagnosis. We aimed to determine the early neurodevelopmental outcomes of preterm infants with IVH and PVL. This was a retrospective single-centre cohort study of preterm infants born at <29 weeks gestation or <1000 g birth weight who attended an Early Neurodevelopment Clinic at 3 to 4 months of corrected age. Primary outcomes of early NDI and CP/high-risk CP diagnoses based on Prechtl's General Movements Assessment and the Hammersmith Infant Neurological Examination were compared between infants without IVH and infants with mild IVH (grades I-II), severe IVH (grades III-IV), and severe brain injury (SBI; severe IVH or cystic PVL). Of 313 infants, 52.1% (n = 163), 41.2% (n = 129), 6.7% (n = 21) and 8.6% (n = 27) had no IVH, mild IVH, severe IVH and SBI, respectively. The adjusted odds of early CP/high-risk CP diagnosis were significantly higher in infants with severe IVH (aOR 6.07, 95% CI 1.50-24.50) and SBI (aOR 15.28, 95% CI 3.70-63), but not in those with mild IVH (aOR 1.24, 95% CI 0.49-3.10). However, the adjusted odds of any early NDI were similar across groups. Preterm infants with severe IVH and SBI are at increased risk of early CP/high-risk of CP diagnosis at 3 to 4 months of corrected age.

The role of Thompson score of term newborns in predicting neuromotor outcome at 1 year of age

Background: The mortality rate is approximately 20% and the frequency of neurodevelopmental sequelae in surviving newborns is approximately 30% in perinatal asphyxia; hypoxic-ischemic encephalopathy (HIE) is brain dysfunction due to lack of oxygen and blood supply to the brain. Aims and Objectives: This study was conducted to predict the pattern of neuromotor outcome as early as 3 months using Hammersmith Infant Neurological Examination (HINE) in term hypoxic babies and its relation with the Thompson score (HIE score). Materials and Methods: One-hundred twenty four term newborns with hypoxic babies were admitted to the neonatal care unit from January 2021 to August 2021. Thompson scoring was done on admission and subsequently reviewed. Discharged babies were followed up at 3 months, 6 months, and 1 year of age. A neurological examination (HINE) was done in this high-risk follow-up clinic. Maximum Thompson score was correlated with neurological score at various months. The earliest predictability of neurological examination was done at 3 months and was correlated with the neurological examination done at 12 months. Results: The correlation between the Thompson score and the 3-month score is −0.65 indicating a strong negative association between the two measurements. The correlation between 3 months and 6 months and 1-year neurological scores was moderately associated with each other. Conclusion: The maximum Thompson score was significantly higher in the mortality group compared to the discharged group. Thompson score has a significant correlation with the 3, 6, and 12-month neurological scores examined by HINE. The 3-month neurological outcome has a very significant correlation with the 1-year outcome.

Improvement in functional motor scores in patients with non-ambulatory spinal muscle atrophy during Nusinersen treatment in South Korea: a single center study

We analyzed the changes in various motor function scores over a four-year period in patients with non-ambulatory spinal muscular atrophy (SMA) during Nusinersen treatment. Patients underwent Hammersmith Infant Neurological Examination (HINE) or Hammersmith Functional Motor Scale Expanded (HFMSE) before treatment, and approximately every 4 months thereafter. Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) or Children’s Hospital of Philadelphia – Adult Test of Neuromuscular Disorders (CHOP ATEND), Revised Upper Limb Module (RULM), and Motor Function Measure (MFM) were performed based on baseline functional status. Narrative interviews were conducted to explore post-treatment physical improvement regarding activities of daily living (ADLs) and fatigue after ADLs. Based on HFMSE results, 9 patients achieved minimum clinically important differences. Average rates of change (slopes) with corresponding 95% confidence intervals for all assessment tools were in a positive direction. CHOP-INTEND showed the most prominent improvement in children and adolescents followed by HFMSE. Improvements in CHOP-ATEND were most noticeable in adults. Improvements were accompanied by changes in ADLs as observed in the narrative interviews. It is necessary to consider various functional aspects to determine the effectiveness of Nusinersen therapy. The objective assessment of the therapeutic effect of Nusinersen in non-ambulatory SMA requires consideration of functional aspects and the related ADLs.

Turkish Adaptation and Psychometric Properties of the Standardized Infant Neurodevelopmental Assessment Neurological Scale in Turkish At-Risk Infants

Background: Early identification and intervention of neurodevelopmental delays can significantly improve outcomes for infants. Therefore, having a standardized assessment tool is essential for clinicians and healthcare professionals working in this field. Objectives: The aim of this study was to assess the concurrent validity and reliability of the Turkish adaptation of the Standardized Infant Neurodevelopmental Assessment (SINDA) neurological scale. Methods: In the study, 111 infants (46 females) participated. Construct validity for the SINDA neurological scale was determined through confirmatory factor analysis, while concurrent validity was established by examining the correlation between the SINDA neurological scale and the Alberta Infant Motor Scale and the Hammersmith Infant Neurological Examination using Spearman's correlation analysis. Additionally, the test-retest reliability of the SINDA scale was examined, and the intraclass correlation coefficient (ICC) was calculated. Results: Construct validity (RMSEA = 0.050; GFI = 0.93) and concurrent validity (r = 0.19 - 0.78; p &lt; 0.05) of the SINDA neurological scale were acceptable. Confirmatory factor analysis results supported the six-factor structure of the original scale. High Cronbach’s alpha and ICC values were found (Cronbach’s α 0.74 - 0.81, ICC 0.991-0.997). Additionally, we found low to high positive correlations of SINDA with HINE and AIMS. Conclusions: The SINDA neurological scale exhibits strong psychometric qualities, making it a reliable and valid instrument for evaluating the neurodevelopmental aspects of at-risk Turkish infants. This has important implications for clinical practice, as early identification and intervention of neurodevelopmental delays can significantly improve outcomes for infants.

Stages of motor development in children with cyanotic congenital heart defects after surgical treatment

Background. The article deals with the actual problem of pediatric neurology, pediatric cardiology, and pediatric cardiac surgery, in particular, the peculiarities of neurodevelopment in children with congenital heart defects (CHD). The purpose is to conduct a study of stages of stato-motor development (SMD) in patients with cyanotic congenital heart defects after surgical treatment and to determine the influence of pathogenic factors on neurodevelopmental delay. Materials and methods. Thirty-five patients with cyanotic CHD underwent examination and surgical treatment at the Center for Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine: 20 children — with transposition of the great vessels (TGV) and 15 children — with tetralogy of Fallot (TOF). The control group consisted of 35 healthy patients whose motor development corresponded to their age. Gestational age, body weight at birth, Apgar score, saturation, and duration of artificial blood circulation during surgical treatment for CHD were considered. Assessment of motor development was carried out with the help of the following scales: Munich Functional Developmental Diagnosis (G.J. Köhler, H.D. Egelkraut) and section 2 of the Hammersmith Infant Neurological Examination. Data were processed using the software Microsoft Excel version 16.0.12527.21236. Results. The gestational age of the studied group of patients with cyanotic CHD was 38 weeks, body weight — 3,500 g, which corresponded to the control group. Patients of the first subgroup with TGV were diagnosed with lower saturation indicators (р = 0.0001), a lower 1-minute Apgar score (p = 0.028) and a longer duration of artificial blood circulation — 146.00 ± 29.03 (р = 0.043) compared to the second subgroup with TOF. SMD delay was diagnosed in 29 % of cases, namely 12 % with TGV and 17 % with TOF. The average period of sitting with delayed SMD was 8.4 ± 2.2 months (p = 0.002), walking — 18.4 ± 6.3 months (p = 0.0001). Subsequently, children with TGV continue to develop without a significant delay compared to the TOF subgroup. Conclusions. 1. Patients with cyanotic CHD are born at 38 weeks of gestation, with sufficient body weight without a significant difference depending on the type of CHD and compared to the control group of healthy children. The predominance of the male gender is typical in cyanotic CHD. However, children with transposition of the great vessels are characterized by a lower 1-minute Apgar score and lower indicators of saturation at birth compared to those with tetralogy of Fallot. 2. Based on the conducted study, a delay of SMD was found in 29 % of patients with cyanotic CHD, namely 17 % with TOF and 12 % with TGV. The average duration of sitting with delay was 8.4 ± 2.2 months and walking was 18.4 ± 6.3 months. 3. Surgical treatment was carried out according to the detected indications, for transposition of the great vessels on the first day and for tetralogy of Fallot at 8 months of life. No pathogenic influence of the duration of artificial blood circulation and aortic clamping during cardiosurgical treatment on SMD in children was found. An increase in the duration of artificial blood circulation was noted during arterial switch operation in children with TGV, but no significant difference was found in children with SMD delay. 4. Timely early surgical treatment of critical CHD — TGV on the first day after birth, a developed route for pregnant women, techniques of performing operations — ensure a better SMD of this subgroup in the future. In patients with TOF, taking into account the long period of hypoxia before the start of surgical correction, neuropsychiatric disorders of varying degrees are more often noted. The development of an individual rehabilitation program is provided for this category of children.

Early Detection and Intervention for Children with High Risk of Cerebral Palsy: A Survey of Physical Therapists and Occupational Therapists in Brazil

Aims The purpose of this study was to assess the current clinical practice of physiotherapists and occupational therapists on early detection and early intervention for children with cerebral palsy (CP) in Brazil. Methods This was a cross-sectional study. A purpose-developed electronic survey was disseminated across the country to physiotherapists and occupational therapists working with young children with or at risk of CP. Results A total of 205 anonymous respondents were included. Most participants (64.4%) agree that the diagnosis of CP can be made before 6 months of age. General Movements Assessment (26.8%) and Hammersmith Infant Neurological Examination (37.1%) were used infrequently. Infants at risk for CP receive therapy twice a week or more by 58.5% of therapists, 93.2% identified parents’ goals as the most important factor in customizing the early intervention program. The most frequent intervention strategies for this age group were active stimulation of the child (n = 182), family training (n = 161), strategies to optimize the environment (n = 143), and neurodevelopmental treatment/Bobath (n = 99). Conclusions Currently, pediatric physiotherapists and occupational therapists in Brazil do not fully incorporate best practice tools for early identification of children with CP, nor sufficient best evidence-based interventions.

Quantitative EEG features during the first day correlate to clinical outcome in perinatal asphyxia.

To assess whether computational electroencephalogram (EEG) measures during the first day of life correlate to clinical outcomes in infants with perinatal asphyxia with or without hypoxic-ischemic encephalopathy (HIE). We analyzed four-channel EEG monitoring data from 91 newborn infants after perinatal asphyxia. Altogether 42 automatically computed amplitude- and synchrony-related EEG features were extracted as 2-hourly average at very early (6 h) and early (24 h) postnatal age; they were correlated to the severity of HIE in all infants, and to four clinical outcomes available in a subcohort of 40 newborns: time to full oral feeding (nasogastric tube NGT), neonatal brain MRI, Hammersmith Infant Neurological Examination (HINE) at three months, and Griffiths Scales at two years. At 6 h, altogether 14 (33%) EEG features correlated significantly to the HIE grade ([r]= 0.39-0.61, p < 0.05), and one feature correlated to NGT ([r]= 0.50). At 24 h, altogether 13 (31%) EEG features correlated significantly to the HIE grade ([r]= 0.39-0.56), six features correlated to NGT ([r]= 0.36-0.49) and HINE ([r]= 0.39-0.61), while no features correlated to MRI or Griffiths Scales. Our results show that the automatically computed measures of early cortical activity may provide outcome biomarkers for clinical and research purposes. The early EEG background and its recovery after perinatal asphyxia reflect initial severity of encephalopathy and its clinical recovery, respectively. Computational EEG features from the early hours of life show robust correlations to HIE grades and to early clinical outcomes. Computational EEG features may have potential to be used as cortical activity biomarkers in early hours after perinatal asphyxia.

A Post-Marketing Surveillance Study of Nusinersen for Spinal Muscular Atrophy in Routine Medical Practice in China: Interim Results.

Spinal muscular atrophy (SMA) is a rare, autosomal recessive, neuromuscular disease that leads to progressive muscular weakness and atrophy. Nusinersen, an antisense oligonucleotide, was approved for SMA in China in February 2019. We report interim results from a post-marketing surveillance phase 4 study, PANDA (NCT04419233), that collects data on the safety, efficacy, and pharmacokinetics of nusinersen in children with SMA in routine clinical practice in China. Participants enrolled in PANDA will be observed for 2years following nusinersen treatment initiation. The primary endpoint is the incidence of adverse events (AEs)/serious AEs (SAEs) during the treatment period. Efficacy assessments include World Health Organization (WHO) Motor Milestones assessment, the Hammersmith Infant Neurological Examination (HINE), and ventilation support. Plasma and cerebrospinal fluid (CSF) concentrations of nusinersen are measured at each dose visit. Fifty participants were enrolled as of the January 4, 2023, data cutoff: 10 with infantile-onset (≤ 6months) and 40 with later-onset (> 6months) SMA. All 50 participants have received at least one dose of nusinersen; 6 have completed the study. AEs were experienced by 45 (90%) participants and were mostly mild/moderate; no AEs led to nusinersen discontinuation or study withdrawal. Eleven participants experienced SAEs, most commonly pneumonia (n = 9); none were considered related to study treatment. Stability or gain of WHO motor milestone was observed and mean HINE-2 scores improved in both subgroups throughout the study. No serious respiratory events occurred,and no permanent ventilation support was initiated during the study. Pre-dose nusinersen CSF concentrations increased steadily through the loading-dose period, with no accumulation in plasma after multiple doses. Nusinersen was generally well tolerated with an acceptable overall safety profile, consistent with the known safety of nusinersen. Efficacy, safety, and nusinersen exposure are consistent with prior observations. These results support continuing PANDA and evaluation of nusinersen in Chinese participants with SMA. ClinicalTrials.gov identifier, NCT04419233.

Long-Term Comparative Efficacy and Safety of Risdiplam and Nusinersen in Children with Type1 Spinal Muscular Atrophy.

Spinal muscular atrophy (SMA) is a severe genetic neuromuscular disease characterized by a loss of motor neurons and progressive muscle weakness. Children with untreated type1 SMA never sit independently and require increasing levels of ventilatory support as the disease progresses. Without intervention, and lacking ventilatory support, death typically occurs before the age of 2years. There are currently no head-to-head trials comparing available treatments in SMA. Indirect treatment comparisons are therefore needed to provide information on the relative efficacy and safety of SMA treatments for healthcare decision-making. The long-term efficacy and safety of risdiplam versus nusinersen in children with type1 SMA was evaluated using indirect treatment comparison methodology to adjust for differences between population baseline characteristics, to reduce any potential bias in the comparative analysis. An unanchored matching-adjusted indirect comparison was conducted using risdiplam data from 58 children in FIREFISH (NCT02913482) and published aggregate nusinersen data from 81 children obtained from the ENDEAR (NCT02193074) and SHINE (NCT02594124) clinical trials with at least 36months of follow-up. Children with type1 SMA treated with risdiplam had a 78% reduction in the rate of death, an 81% reduction in the rate of death or permanent ventilation, and a 57% reduction in the rate of serious adverse events compared with children treated with nusinersen. Children treated with risdiplam also had a 45% higher rate of achieving a Hammersmith Infant Neurological Examination, Module2 motor milestone response and a 186% higher rate of achieving a ≥ 4-point improvement in Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders compared with children treated with nusinersen. Long-term data supported risdiplam as a superior alternative to nusinersen in children with type1 SMA. Video abstract available for this article. Video abstract (MP4 184542 KB).

Early Neurodevelopmental Assessments for Predicting Long-Term Outcomes in Infants at High Risk of Cerebral Palsy

Studies suggest that early neurodevelopmental assessments are beneficial for identifying cerebral palsy, yet their effectiveness in practical scenarios and their ability to detect cognitive impairment are limited. To assess the effectiveness of early neurodevelopmental assessments in identifying cerebral palsy and cognitive and other neurodevelopmental impairments, including their severity, within a multidisciplinary clinic. This diagnostic study was conducted at Monash Children's Hospital, Melbourne, Australia. Participants were extremely preterm infants born at less than 28 weeks' gestation or extremely low birth weight infants less than 1000 g and term encephalopathic infants who received therapeutic hypothermia, attending the early neurodevelopmental clinic between January 2019 and July 2021. Data were analyzed from December 2023 to January 2024. Early cerebral palsy or high risk of cerebral palsy, the absence of fidgety movements, and Hammersmith Infant Neurological Examination (HINE) scores at corrected age (CA) 3 to 4 months. Early cerebral palsy or high risk of cerebral palsy diagnosis was based on absent fidgety movements, a low HINE score (<57), and medical neurological examination. The outcomes of interest were cerebral palsy, cognitive and neurodevelopmental impairments and their severity, diagnosed at 24 to 36 months' CA. A total of 116 infants (median [IQR] gestational age, 27 [25-29] weeks; 65 [56%] male) were included. Diagnosis of early cerebral palsy or high risk of cerebral palsy demonstrated a sensitivity of 92% (95% CI, 63%-99%) and specificity of 84% (95% CI, 76%-90%) for predicting cerebral palsy and 100% (95% CI, 59%-100%) sensitivity and 80% (95% CI, 72%-87%) specificity for predicting moderate to severe cerebral palsy. Additionally, the accuracy of diagnosis of early cerebral palsy or high risk of cerebral palsy was 85% (95% CI, 77%-91%) for predicting cerebral palsy and 81% (95% CI, 73%-88%) for predicting moderate to severe cerebral palsy. Similarly, the absence of fidgety movements had an 81% (95% CI, 73%-88%) accuracy in predicting cerebral palsy, and HINE scores exhibited good discriminatory power with an area under the curve of 0.88 (95% CI, 0.79-0.97) for cerebral palsy prediction. However, for cognitive impairment, the predictive accuracy was 44% (95% CI, 35%-54%) for an early cerebral palsy or high risk of cerebral palsy diagnosis and 45% (95% CI, 36%-55%) for the absence of fidgety movements. Similarly, HINE scores showed poor discriminatory power for predicting cognitive impairment, with an area under the curve of 0.62 (95% CI, 0.51-0.73). In this diagnostic study of infants at high risk for cerebral palsy or other cognitive or neurodevelopmental impairment, early neurodevelopmental assessments at 3 to 4 months' CA reliably predicted cerebral palsy and its severity at 24 to 36 months' CA, signifying its crucial role in facilitating early intervention. However, for cognitive impairment, longer-term assessments are necessary for accurate identification.

Relationship between early infant motor repertoire and neurodevelopment on the hammersmith infant neurological examination in a developmentally vulnerable First Nations cohort

AimTo implement a culturally-adapted screening program aimed to determine the ability of infant motor repertoire to predict early neurodevelopment on the Hammersmith Infant Neurological Examination (HINE) and improve Australian First Nations families' engagement with neonatal screening. MethodsA prospective cohort of 156 infants (55 % male, mean (standard deviation [SD]) gestational age 33.8 (4.6) weeks) with early life risk factors for adverse neurodevelopmental outcomes (ad-NDO) participated in a culturally-adapted screening program. Infant motor repertoire was assessed using Motor Optimality Score-revised (MOS-R), captured over two videos, 11–13+6 weeks (V1; <14 weeks) and 14–18 weeks (V2; ≥14 weeks) corrected age (CA). At 4–9 months CA neurodevelopment was assessed on the HINE and classified according to age-specific cut-off and optimality scores as; developmentally ‘on track’ or high chance of either adverse neurodevelopmental outcome (ad-NDO) or cerebral palsy (CP). ResultsFamilies were highly engaged, 139/148 (94 %) eligible infants completing MOS-R, 136/150 (91 %), HINE and 123 (83 %) both. Lower MOS-R at V2 was associated with reduced HINE scores (β = 1.73, 95 % confidence interval [CI] = 1.03–2.42) and high chance of CP (OR = 2.63, 95%CI = 1.21–5.69) or ad-NDO (OR = 1.38, 95%CI = 1.10–1.74). The MOS-R sub-category ‘observed movement patterns’ best predicted HINE, infants who score ‘4’ had mean HINE 19.4 points higher than score ‘1’ (95%CI = 12.0–26.9). Receiver-operator curve analyses determined a MOS-R cut-off of <23 was best for identifying mild to severely reduced HINE scores, with diagnostic accuracy 0.69 (sensitivity 0.86, 95%CI 0.76–0.94 and specificity 0.40, 95 % CI 0.25–0.57). A trajectory of improvement on MOS-R (≥2 point increase in MOS-R from 1st to 2nd video) significantly increased odds of scoring optimally on HINE (OR = 5.91, 95%CI 1.16–29.89) and may be a key biomarker of ‘on track’ development. InterpretationImplementation of a culturally-adapted program using evidence-based assessments demonstrates high retention. Infant motor repertoire is associated with HINE scores and the early neurodevelopmental status of developmentally vulnerable First Nations infants.

Quality Improvement Project to improve access to early screening and physiotherapy for infants at high risk of neurodevelopmental disorders

ABSTRACT Purpose and Setting Recent international guidelines for Early Diagnosis of Cerebral Palsy (CP) recommend the use of specific evidence-based tools and early therapy intervention for preterm infants at high risk of cerebral palsy. Referrals for physiotherapy and diagnoses of CP in high-risk infants discharged from the Neonatal Intensive Care Unit (NICU) at the Chelsea and Westminster NHS trust were recorded as occurring later than guidance recommends. A quality improvement project was initiated aiming to enhance identification of CP, increase provision of early physiotherapy and achieve this within the current physiotherapy staffing establishment. Methods A quality improvement project was implemented. Physiotherapists received training in using evidence-based assessment tools and early intervention. A new pathway was developed including a schedule of screening assessments and physiotherapy for high-risk infants on discharge from the NICU. This included a 14-month pilot of a new physiotherapy outpatient screening clinic. Results Following the pilot, age of referral for physiotherapy reduced from a mean of 11.84 to 4.25 months. All babies were assessed using recommended evidence-based tools including the Hammersmith Infant Neurological Examination, Hammersmith Neonatal Neurological Examination and General Movements. Previously only the Alberta Infant Motor Scales were used. Parent feedback demonstrated that the parents felt supported, valued the relationship with physiotherapists and felt confident to carry out activities suggested by physiotherapists. Conclusion and Recommendations The new physiotherapy screening clinic was effective in increasing access to early physiotherapy and evidence based diagnostic assessments and was achieved within current staffing levels. A business case is being considered to increase physiotherapy provision so intervention can be provided consistently at the planned frequency. It is suggested that other Trusts may also find this model of intervention effective.

Identifying and Evaluating Young Children with Developmental Central Hypotonia: An Overview of Systematic Reviews and Tools.

Children with developmental central hypotonia have reduced muscle tone secondary to non-progressive damage to the brain or brainstem. Children may have transient delays, mild or global functional impairments, and the lack of a clear understanding of this diagnosis makes evaluating appropriate interventions challenging. This overview aimed to systematically describe the best available evidence for tools to identify and evaluate children with developmental central hypotonia aged 2 months to 6 years. A systematic review of systematic reviews or syntheses was conducted with electronic searches in PubMed, Medline, CINAHL, Scopus, Cochrane Database of Systematic Reviews, Google Scholar, and PEDro and supplemented with hand-searching. Methodological quality and risk-of-bias were evaluated, and included reviews and tools were compared and contrasted. Three systematic reviews, an evidence-based clinical assessment algorithm, three measurement protocols, and two additional measurement tools were identified. For children aged 2 months to 2 years, the Hammersmith Infant Neurological Examination has the strongest measurement properties and contains a subset of items that may be useful for quantifying the severity of hypotonia. For children aged 2-6 years, a clinical algorithm and individual tools provide guidance. Further research is required to develop and validate all evaluative tools for children with developmental central hypotonia.

The Knowledge Translation of Early Cerebral Palsy (KiTE CP) Study: Implementing Screening Among a High-Risk Prospective Cohort of Australian Infants

ObjectiveTo describe the implementation of the international guidelines for the early diagnosis of cerebral palsy (CP) and engagement in the screening process in an Australian cohort of infants with neonatal risk factors for CP. Study designProspective cohort study of infants with neonatal risk factors recruited at <6 months CA from 11 sites in the states of Victoria, New South Wales, and Queensland, Australia. First, we implemented a multi-modal knowledge translation strategy including barrier identification, technology integration and special interest groups. Screening was implemented as follows: infants with clinical indications for neuroimaging underwent magnetic resonance imaging and/or cranial ultrasound. The General Movements Assessment (GMA) was recorded clinically or using an app (Baby Moves). Infants with absent or abnormal fidgety movements on GMA videos were offered further assessment using the Hammersmith Infant Neurological Examination (HINE). Infants with atypical findings on 2/3 assessments met criteria for high risk of CP. ResultsOf the 597 infants (56% male) recruited, 95% (n=565) received neuroimaging, 90% (n=537) had scorable GMA videos (2% unscorable/8% no video), and 25% (n=149) HINE. Overall, 19% of the cohort (n=114/597) met criteria for high risk of CP, 57% (340/597) had at least two normal assessments (of neuroimaging, GMA or HINE), and 24% (n=143/597) had insufficient assessments. ConclusionsEarly CP screening was implemented across participating sites using a multi-modal knowledge translation strategy. Although the COVID-19 pandemic affected recruitment rates, there was high engagement in the screening process. Reasons for engagement in early screening from parents and clinicians warrant further contextualization and investigation.