Hammersmith Infant Neurological Examination Research Articles

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.

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.

Application of the Hammersmith Infant Neurological Examination in the developmental follow-up of high-risk infants.

To investigate the independent influences affecting the global score of the Hammersmith Infant Neurological Examination (HINE) in the early life of high-risk infants and to provide evidence for early effective screening and for evaluating interventions. We conducted a prospective cohort study of 258 high-risk infants assessed by the HINE and Gesell Developmental Diagnosis Schedule at 3, 6, 9, and 12 months corrected age. A multiple linear regression model was developed to investigate independent influences on HINE global score at 3 months corrected age. The accuracy of the HINE global score was analysed by calculating the discriminant, concurrent, and predictive validities according to ages. There were nine independent influences affecting the HINE global score at 3 months corrected age in high-risk infants. The discriminant, concurrent, and predictive validities of the HINE for gross motor developmental delays at 12 months corrected age were all statistically significant (p < 0.05). Different neonatal clinical settings are related to the HINE global score of high-risk infants early in life. The HINE can be used for longitudinal monitoring of neurological development in the first year of life in a typical Chinese clinical setting and the findings at all four ages tested relate to neuromotor outcomes at 12 months corrected age.

Therapeutic hypothermia after perinatal asphyxia in Vietnam: medium-term outcomes at 18 months – a prospective cohort study

AimTo determine neurodevelopmental outcome at 18 months after therapeutic hypothermia for hypoxic-ischaemic encephalopathy (HIE) infants in Vietnam, a low-middle-income country.MethodProspective cohort study investigating outcomes at 18 months in severely asphyxiated...

Reliability, Knowledge Translation, and Implementability of the Spanish Version of the Hammersmith Infant Neurological Examination

Purpose. This study aimed to: (a) translate and cross-culturally adapt the Hammersmith Infant Neurological Examination (HINE) into Spanish; (b) evaluate its intra- and inter-examiner reliability; (c) support a knowledge translation and tool implementation program in early intervention; and (d) evaluate its reliability and implementation for professionals one year after receiving training. Materials and methods. The translation followed the World Health Organization’s recommendations. Reliability was assessed in 25 infants aged between 3 and 15 months with identifiable risks of cerebral palsy (CP). The implementation was also evaluated by analyzing the reliability of professionals without previous experience of the tool by using a pre-survey and a follow-up survey one year after training. The survey covered aspects related to the use of early detection tools of CP and the use of HINE, including attitudes, opinions, and perceptions. Results. An excellent intra- and inter-examiner agreement was obtained for the total score of the HINE intra-class correlation coefficient (ICC = 0.98 in both indices). One year after training, the professionals also showed excellent reliability values (ICC = 0.99), as well as an increase in sensitization and skills in evidence-based practices for the early detection of “high risk” of CP. Conclusions. The Spanish version of HINE is a reliable measure for the neurological evaluation of “high risk” of CP and can be administered after standardized training and without costs to acquire the evaluation. This allows its accessible and widespread implementation in the clinical context.

Neurological assessment tool for screening infants during the first year after birth: The Brief-Hammersmith Infant Neurological Examination.

To develop a short version of the original Hammersmith Infant Neurological Examination (HINE) to be used as a screening tool (Brief-HINE) and to establish if the short examination maintains good accuracy and predictive power for detecting infants with cerebral palsy (CP). Eleven items were selected from the original HINE ('visual response'; 'trunk posture'; 'movement quantity'; 'movement quality'; 'scarf sign'; 'hip adductor angles'; 'popliteal angle'; 'pull to sit'; 'lateral tilting'; 'forward parachute reaction'; 'tendon reflexes') identifying those items previously found to be more predictive of CP in both low- and high-risk infants. In order to establish the sensitivity of the new module, the selected items were applied to existing data, previously obtained using the full HINE at 3, 6, 9, and 12 months, in 228 infants with typical development at 2 years and in 82 infants who developed CP. Brief-HINE scores showed good sensitivity and specificity, at each age of assessment, for detecting infants with CP. At 3 months, a score of less than 22 was associated with CP with a sensitivity of 0.88 and a specificity of 0.92; at 6, 9, and 12 months, the cut-off scores were less than 25 (sensitivity 0.93; specificity 0.87), less than 27 (sensitivity 0.95; specificity 0.81), and less than 27 (sensitivity 1; specificity 0.86) respectively. The presence of more than one warning sign, or items that are not optimal for the age of assessment, imply the need for a full examination reassessment. These findings support the validity of the Brief-HINE as a routine screening method and the possibility of its use in clinical practice.

Comparison of neurological assessment and general movements of infants at risk at different time points

Background: Brain development is a constant interaction between genetic, biological, and environmental factors. Its maturation process is extremely sensitive, and if it does not occur in sync, brain damage can occur in the immature brain, triggering a set of permanent movement and posture disorders. Thus, early diagnosis of developmental changes is essential for intervention to begin as soon as possible, optimizing results, improving functional skills, and reducing the damage to the future life of these babies, and serve as a basis for the actions of professional teams dedicated to stimulation and monitoring of child development. Objectives: To compare the neurological assessment and general movements of infants at risk at 40, 52, and 64 weeks of gestational age (GA). Methods: Infants at risk born in a reference maternal and child hospital were assessed for their neuromotor behavior using the "Hammersmith Neonatal Neurological Examination (HNNE)", "Hammersmith Infant Neurological Examination (HINE)" and "General Movements Assessment (GMA)" scales at 40, 52, and 64 weeks gestational age. Data will be correlated and analyzed descriptively. Results: 33 infants at risk were assessed at 40, 52 and 64 weeks, i.e., up to six months of corrected age. Male gender (75.8%), a cesarean delivery (51.5%), and white race (60.6%) predominated. The Median Apgar score was nine, mean of five prenatal visits, and a mean GA of 35.8 weeks. Prematurity and congenital syphilis were the main diagnoses found. 30.3% of the patients underwent neuroimaging exams, especially brain ultrasounds, with normal results. There was a predominance of "altered evaluation" in HNNE and HINE at the three evaluative moments for term babies, especially for babies with congenital syphilis, and "adequate" for premature babies. In the GMA evaluation and regarding the presence of fidgety, there was a higher frequency of the "suboptimal" classification and most presented fidgety. In the longitudinal comparison between the three moments assessed using the HNNE/HINE scales, it was found that there was a significant difference between the first and the third evaluative moment (p = 0.029), where babies improved their classification. There were positive associations of results between the HNNE/HINE scales with scores in different variables. Conclusion: Infants at risk had altered scores on neurological assessments, indicating developmental delay. Children at risk or with delayed neurological development may present restrictions in activities and participation with compromised quality of life and even school learning. Therefore, it is extremely important to include standardized scales during neonatal screening in the routine of the Neonatal Intermediate Care Unit (NICU), as they are highly reliable and low-cost, so that early identification of neurological changes can be enabled, and thus intervene early, enhancing development.

Translation and measurement properties of the Portuguese-Brazil version of the Hammersmith Infant Neurological Examination (HINE-Br).

The current study aimed to translate the Hammersmith Infant Neurological Examination (HINE) into Brazilian Portuguese and analyze the reliability of the translated version for a population of Brazilian infants. This was a methodological study, approved by the Ethics Committee, carried out between June 2020 and May 2021. HINE is a standardized clinical neurological examination used for the early detection of cerebral palsy. The quantitative section, "neurological examination", contains 26 items scored from 0 to 3 points, divided into five categories: cranial nerve function, posture, movements, muscle tone and reflexes, and reactions. The HINE translation followed four steps: translation, synthesis, back-translation, and evaluation by an expert committee. To verify the reliability of the HINE-Br (Portuguese-Brazil version) two independent examiners evaluated 43 infants, between 3 and 22 months of age. Internal consistency was verified by Cronbach's Alpha coefficient and interrater reliability by the intraclass correlation coefficient (ICC). The translated version was similar to the original version and a few semantic and idiomatic adjustments were necessary. Appropriate internal consistency (Alpha=0.91) was found for the 26 items of the HINE-Br, as well as strong interrater reliability for the total score (ICC2.1=0.95), and also for the five categories (ICC2.1=0.83-0.95). The HINE-Br presents adequate rates of internal consistency and interrater reliability, and can be used for the evaluation of children at risk for cerebral palsy, between 3 and 24 months of age, by pediatricians and pediatric physical therapists.

A Call for Early Detection of Cerebral Palsy.

Cerebral palsy (CP) is the most common physical disability across the lifespan, but historically, CP has not been diagnosed before the age of 2 years. Barriers to early diagnosis ranged from lack of available biomarkers, absence of curative treatments, perceived stigma associated with a lifelong diagnosis, and a desire to rule out other diagnoses first. Most importantly, the fundamental question that remained was whether children would benefit from earlier detection and intervention given the paucity of research. However, evidence-based guidelines published in 2017 demonstrated that the General Movements Assessment, the Hammersmith Infant Neurological Examination, and neuroimaging can be combined with other elements such as a clinical history and standardized motor assessments to provide the highest predictive value for diagnosing CP as early as age 3 months in high-risk newborns. Implementation of these guidelines has been successful in decreasing the age at CP diagnosis, particularly in high-risk infant follow-up clinics with expertise in performing these assessments. Early detection of CP allows for clinical and research opportunities investigating earlier interventions during a critical period of neuroplasticity, with the goal of improving developmental trajectories for children and their families. New guidelines and research are now being developed with a focus on early, targeted interventions that continue to be studied, along with global detection initiatives.

Hammersmith Infant Neurological Examination Subscores Are Predictive of Cerebral Palsy

The Hammersmith Infant Neurological Examination (HINE) is a standardized assessment that identifies early signs of cerebral palsy (CP). In practice, the clinician performs this assessment in its entirety, yielding a global score. This study aimed to investigate the individual HINE subscores and "asymmetries" as predictive indicators of CP. In this retrospective nested case-control study, a pediatric neurologist performed the HINE on a cohort of three- to four-month-old former neonatal intensive care unit infants. The infants' neurodevelopmental outcomes were determined by chart review when they were aged two to three years. We performed univariate and multivariable logistic regression analyses to yield the accuracy of the global HINE score, HINE subscores, and "asymmetries" in classifying infants with and without CP. Of the 108 infants on whom HINE was performed, 50 were either discharged due to normal developmental progress or were lost to follow-up. Of the remaining 58 subjects, 17 had CP and 41 did not. Receiver operator characteristic (ROC) curves of univariate models yielded the following area under the curve (AUC) scores: global HINE score (AUC=0.75), "reflexes and reactions" (AUC=0.80), "cranial nerve function" (AUC=0.76), "asymmetries" (AUC=0.75), and "movements" (AUC=0.71). The ROC for our multivariable model (AUC=0.91) surpassed the global HINE score's predictive value for CP. The weighted combination of HINE subscores and "asymmetries" outperforms the global HINE score in predicting CP. These findings suggest the need for revisiting HINE, but further validation with a larger dataset is required.

KONJENİTAL SANTRAL HİPOVENTİLASYON SENDROMU VE SEREBRAL PALSİLİ OLGUDA FİZYOTERAPİ VE REHABİLİTASYON PROGRAMININ ETKİSİNİN İNCELENMESİ: BİR OLGU SUNUMU

Congenital Central Hypoventilation Syndrome is a rare genetic disorder that presents from birth and prevents automatic control of respiration. Our aim in this study was to examine the effectiveness of a 6-month physical therapy program applied to a patient with Congenital Central Hypoventilation Syndrome and cerebral palsy. A 6-month-old patient who was diagnosed with Congenital Central Hypoventilation Syndrome and subsequently developed Cerebral Palsy due to asphyxia was included in the study. The case was enrolled in a physical therapy program for 2 days a week for 6 months in the home environment where they lived. The case was evaluated before and after treatment. In our case, the Modified Ashworth Scale, the Alberta Infant Motor Scale, the Hammersmith Infant Neurological Examination, Test of Sensory Functions in Infants, and the Face, Legs, Activity, Cry, Consol ability scale were used. In our case, pre-treatment and post-treatment scores were as follows, respectively: Alberta Infant Motor Scale score 3-8, Hammersmith Infant Neurological Examination score 6-17, Test of Sensory Functions in Infants score 2-9, and Face, Legs, Activity, Cry, Consol ability scale score 8-3. In our case, there was a significant improvement in Modified Ashworth Scale scores before and after treatment. Additionally, at the conclusion of the study, it was noted that the heightened pain and sensitivity resulting from the patient's extended stay in the intensive care unit reduced with the treatment. We believe that the administered physiotherapy and rehabilitation program offered support to the patient in achieving neuro-motor and sensory integration, underscoring the essential role of physiotherapy in rare diseases.

Cerebral Palsy – Early Diagnosis and Intervention Trial: protocol for the prospective multicentre CP-EDIT study with focus on diagnosis, prognostic factors, and intervention

BackgroundEarly diagnosis of cerebral palsy (CP) is important to enable intervention at a time when neuroplasticity is at its highest. Current mean age at diagnosis is 13 months in Denmark. Recent research has documented that an early-diagnosis set-up can lower diagnostic age in high-risk infants. The aim of the current study is to lower diagnostic age of CP regardless of neonatal risk factors. Additionally, we want to investigate if an early intervention program added to standard care is superior to standard care alone.MethodsThe current multicentre study CP-EDIT (Early Diagnosis and Intervention Trial) with the GO-PLAY intervention included (Goal Oriented ParentaL supported home ActivitY program), aims at testing the feasibility of an early diagnosis set-up and the GO-PLAY early intervention. CP-EDIT is a prospective cohort study, consecutively assessing approximately 500 infants at risk of CP. We will systematically collect data at inclusion (age 3–11 months) and follow a subset of participants (n = 300) with CP or at high risk of CP until the age of two years. The GO-PLAY early intervention will be tested in 80 infants with CP or high risk of CP.Focus is on eight areas related to implementation and perspectives of the families: early cerebral magnetic resonance imaging (MRI), early genetic testing, implementation of the General Movements Assessment method, analysis of the GO-PLAY early intervention, parental perspective of early intervention and early diagnosis, early prediction of CP, and comparative analysis of the Hand Assessment for Infants, Hammersmith Infant Neurological Examination, MRI, and the General Movements method.DiscussionEarly screening for CP is increasingly possible and an interim diagnosis of “high risk of CP” is recommended but not currently used in clinical care in Denmark. Additionally, there is a need to accelerate identification in mild or ambiguous cases to facilitate appropriate therapy early. Most studies on early diagnosis focus on identifying CP in infants below five months corrected age. Little is known about early diagnosis in the 50% of all CP cases that are discernible later in infancy. The current study aims at improving care of patients with CP even before they have an established diagnosis.Trial registrationClinicalTrials.gov ID 22013292 (reg. date 31/MAR/2023) for the CP-EDIT cohort and ID 22041835 (reg. date 31/MAR/2023) for the GO-PLAY trial.

Managing mothers' and fathers' uncertainty during their journey through early neurodevelopmental follow-up for their high-risk infants-A qualitative account.

Early diagnosis of cerebral palsy is possible by 5 months corrected age for 'at-risk' infants, using diagnostic tools such as the Hammersmith Infant Neurological Examination (HINE), Prechtl's General Movements Assessment (GMA) and magnetic resonance imaging (MRI). This is an uncertain and stressful time for parents where provision of appropriate information and support is essential. To explore parents' views and experiences in relation to the new early neurodevelopmental follow-up of 'at-risk' infants. Thirteen in-depth one-to-one qualitative interviews were conducted by the primary researcher, with eight parents (six mothers and two fathers) of 'at-risk' infants eligible for a follow-up clinic where the GMA and HINE were performed at 12-week corrected age. Interviews used a pre-piloted topic guide and took place before and after the clinic. Interviews were audio-recorded, transcribed verbatim and analysed using inductive coding and thematic analysis using the framework approach. Seven themes were identified: (1) attempting to manage uncertainty, (2) taking priority, (3) trusting professionals, (4) independence in the parent role, (5) feeling understood, (6) patterns of care and (7) individuality. Parents reported experiencing uncertainty about their current situation and future. Adequate preparation for and timing of information are vital. When uncertainty is poorly managed, parents' wellbeing suffers. Individual parents' perspectives and infants' developmental trajectories differ, and information should be tailored specifically for this. A parent's understanding of the journey through neurodevelopmental care for their high risk infants is initially very limited. Implementing a counselling service for parents to access psychological support and digital reminder system for clinic appointments, as well as providing more tailored information through trusted professionals, could all improve future parents' experiences.

Protocol for a randomized controlled trial to evaluate a year-long (NICU-to-home) evidence-based, high dose physical therapy intervention in infants at risk of neuromotor delay.

Developmental disabilities and neuromotor delay adversely affect long-term neuromuscular function and quality of life. Current evidence suggests that early therapeutic intervention reduces the severity of motor delay by harnessing neuroplastic potential during infancy. To date, most early therapeutic intervention trials are of limited duration and do not begin soon after birth and thus do not take full advantage of early neuroplasticity. The Corbett Ryan-Northwestern-Shirley Ryan AbilityLab-Lurie Children's Infant Early Detection, Intervention and Prevention Project (Project Corbett Ryan) is a multi-site longitudinal randomized controlled trial to evaluate the efficacy of an evidence-based physical therapy intervention initiated in the neonatal intensive care unit (NICU) and continuing to 12 months of age (corrected when applicable). The study integrates five key principles: active learning, environmental enrichment, caregiver engagement, a strengths-based approach, and high dosage (ClinicalTrials.gov identifier NCT05568264). We will recruit 192 infants at risk for neuromotor delay who were admitted to the NICU. Infants will be randomized to either a standard-of-care group or an intervention group; infants in both groups will have access to standard-of-care services. The intervention is initiated in the NICU and continues in the infant's home until 12 months of age. Participants will receive twice-weekly physical therapy sessions and caregiver-guided daily activities, assigned by the therapist, targeting collaboratively identified goals. We will use various standardized clinical assessments (General Movement Assessment; Bayley Scales of Infant and Toddler Development, 4th Edition (Bayley-4); Test of Infant Motor Performance; Pediatric Quality of Life Inventory Family Impact Module; Alberta Infant Motor Scale; Neurological, Sensory, Motor, Developmental Assessment; Hammersmith Infant Neurological Examination) as well as novel technology-based tools (wearable sensors, video-based pose estimation) to evaluate neuromotor status and development throughout the course of the study. The primary outcome is the Bayley-4 motor score at 12 months; we will compare scores in infants receiving the intervention vs. standard-of-care therapy.