Motor Developmental Outcomes Research Articles

PRO133 Validating Vignettes for a Rare Disease Using Clinician Interviews to Evaluate the Impact on Health-Related Quality of Life in Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency in France

In the absence of utilities from clinical trials, health related quality of life (HRQoL) can be estimated using health utilities derived through vignette studies and discrete choice experiments (DCE). This enables treatment evaluation in cost-utility analysis for HTA assessments. Vignettes should have face and content validity; obtaining input into the design from caregivers/parents of aromatic l-amino acid decarboxylase (AADC) deficiency patients, and clinicians treating these patients is, thus, critical. The study objective was to validate the vignettes and DCE attributes already developed and validated for the UK– this time specifically for France. These will be used in a subsequent study to estimate patient HRQoL in France. Initial vignettes were developed based on a literature review, interactions with clinicians, as well as parents via interviews, advisory boards and anonymised surveys. To capture improvements on therapy, input into key DCE attributes were also obtained. The health states were bedridden, head control, sitting unsupported, standing with assistance, and walking with assistance aligned to major motor developmental milestones and outcomes in the clinical trials. The vignettes and DCE attributes were translated including full reconciliation. In a second step, the French translation as well as the content of the vignettes were validated using 4 French clinicians’ input. The translation process resulted in minor amends to the vignette wordings for each health state. The French clinicians supported the health state descriptions with 6 suggested changes including the addition of ‘tiredness’ as a symptom to two health states and minor revisions to the language translation. While this project supported the face and content validity of the vignettes and attributes previously developed for the UK, it also showed that it is important to validate with local experts the language when planning to derive utilities for a cost-effectiveness model of an AADC deficiency treatment in a specific country.

Early Childhood General Anesthesia and Neurodevelopmental Outcomes in the Avon Longitudinal Study of Parents and Children Birth Cohort.

Most common anesthetic agents have been implicated in causing neurodegeneration in the developing animal brain, leading to warnings regarding their use in children. The hypothesis of this study was that exposure to general anesthesia and surgery before 4 yr would associate with adverse neurodevelopmental outcomes at age 7 to 16 yr. This cohort study comprised 13,433 children enrolled in the Avon Longitudinal Study of Parents and Children, a prospective, population-based birth cohort born between 1991 and 1993 in southwest England. Children were grouped by none, single, or multiple exposures to general anesthesia and surgery by 4 yr. Motor, cognitive, linguistic, educational, social, and behavioral developmental outcomes were evaluated at 7 to 16 yr using school examination results, validated parent/teacher questionnaires, or clinic assessments. Continuous outcomes were z-scored. P-value thresholds were corrected using false discovery rate procedures. This study compared 46 neurodevelopmental outcomes in 13,433 children: 8.3% (1,110) exposed singly and 1.6% (212) exposed multiply to general anesthesia and surgery. Of these, the following reached predefined levels of statistical significance (corrected P < 0.00652): dynamic balance scores were 0.3 SD (95% CI, 0.1, 0.5; P < 0.001) lower in multiply exposed children; manual dexterity performance was 0.1 SD (95% CI, 0.0, 0.2; P = 0.006) lower in singly and 0.3 SD (95% CI, 0.1, 0.4; P < 0.001) lower in multiply exposed children; and social communication scores were 0.1 SD (95% CI, 0.0, 0.2; P = 0.001) and 0.4 SD (95% CI, 0.3, 0.5; P < 0.001) lower in singly and multiply exposed children, respectively. General anesthesia and surgery were not associated with impairments in the remaining neurodevelopmental measures including: general cognitive ability; attention; working memory; reading, spelling, verbal comprehension and expression; behavioral difficulties; or national English, mathematics, and science assessments (all ≤0.1 SD; corrected P ≥ 0.00652). Early childhood general anesthesia and surgery were not associated with a global picture of clinically and statistically significant neurodegenerative effects, providing reassurance about the neurotoxic potential of general anesthesia. Exposure to anesthesia and surgery was associated with significantly lower motor and social linguistic performance.

The degree of prematurity affects functional brain activity in preterm born children at school-age: An EEG study

Prematurely born children are at higher risk for long-term adverse motor and cognitive outcomes. The aim of this paper was to compare quantitative measures derived from electroencephalography (EEG) between extremely (EP) and very prematurely (VP) born children at 9–10 years of age.Fifty-five children born <32 weeks' of gestation underwent EEG at 9–10 years of age and were assessed for motor development and cognitive outcome. Relative frequency power and functional connectivity, as measured by the Phase Lag Index (PLI), were calculated for all frequency bands. Per subject, power spectrum and functional connectivity results were averaged over all channels and pairwise PLI values to explore differences in global frequency power and functional connectivity between EP and VP children. Brain networks were constructed for the upper alpha frequency band using the Minimum Spanning Tree method and were compared between EP and VP children. In addition, the relationships between upper alpha quantitative EEG results and cognitive and motor outcomes were investigated.Relative power and functional connectivity were significantly higher in VP than EP children in the upper alpha frequency band, and VP children had more integrated networks. A strong positive correlation was found between relative upper alpha power and motor outcome whilst controlling for gestational age, age during EEG recording, and gender (ρ = 0.493, p = 0.004).These results suggest that 9–10 years after birth, the effects of the degree of prematurity can be observed in terms of alterations in functional brain activity and that motor deficits are associated with decreases in relative upper alpha power.

Individual longitudinal neurodevelopmental trajectories of children treated with hypothermia for perinatal asphyxia from 3 months to 5 years of age

BackgroundHypothermia for perinatal asphyxia is a common treatment to decrease morbidity. This study aims to describe a) individual longitudinal neurodevelopmental trajectories over 5 years in children with perinatal asphyxia treated with hypothermia and b) the correlation between movement quality at 3 months and motor developmental outcomes at 5 years of age. MethodsIn this longitudinal cohort study, 18 children (12 male) were assessed at 3 (t1), 6 (t2), 12 (t3), and 24 (t4) months, and at the age of 5 (t5) years, with standardized norm-referenced tests. ResultsSix children showed abnormal movement quality assessed with General Movements (t1) and all showed severe neurodevelopmental disabilities at t5. The 12 children without severe disabilities, showed a significant normalization of z-scores over the five assessment points (linear mixed model analysis). At t5, four of these children scored mildly delayed motor or cognitive development. Conclusion and implicationsChildren without anomalies on the MRI before hospital discharge and normal movement quality at 3 months of age showed normal neurodevelopment at the age of 5, however, individual motor trajectories showed variability over time. Presents of abnormal GMs tend to detect CP and developmental problems, advocating a developmental surveillance to determine need for early intervention.

Long-Term Effects of Intrauterine Exposure to Antidepressants on Physical, Neurodevelopmental, and Psychiatric Outcomes: A Systematic Review.

Reviews on child outcomes following in utero antidepressant exposure have focused on short-term outcomes. However, several recent individual studies reported on adverse physical, neurodevelopmental, and psychiatric outcomes beyond infancy and early childhood. The objective of this systematic review was to establish the long-term effects of prenatal antidepressant exposure on physical, neurodevelopmental, and psychiatric outcomes in individuals aged 4 years and older. Embase, MEDLINE Ovid, Web of Science, Cochrane Central, and Google Scholar were systematically searched for all relevant articles, written in English and published prior to November 8, 2018, using terms describing antidepressants, pregnancy, and developmental outcomes. All original research articles on long-term outcomes of prenatal antidepressant exposure were eligible for inclusion. After screening and removal of duplicates, a total of 34 studies were identified. Included articles were qualitatively analyzed to determine inconsistency, indirectness, imprecision, and study bias. The identified studies demonstrated statistically significant associations between prenatal antidepressant exposure and a range of physical, neurodevelopmental, and psychiatric outcomes. Yet, the risk of confounding by indication was high. When controlling for confounders, 5 studies investigating physical outcomes (asthma, cancer, body mass index [BMI], epilepsy) found no association except conflicting outcomes for BMI. Eighteen studies examining neurodevelopmental outcomes (cognition, behavior, IQ, motor development, speech, language, and scholastic outcomes) found no consistent associations with antidepressant exposure after taking confounders into account. Eleven studies investigated psychiatric outcomes. After adjusting for confounders, prenatal antidepressant exposure was associated with affective disorders but not with childhood psychiatric outcomes (eg, autism spectrum disorders, attention-deficit/hyperactivity disorder). Reported associations between in utero exposure to antidepressants and physical, neurodevelopmental, and psychiatric outcomes, in large part, seem to be driven by the underlying maternal disorder. When limiting confounding by indication, prenatal exposure to antidepressants was significantly associated only with offspring BMI and affective disorders.

Protocol for the trial to establish a causal linkage between mycotoxin exposure and child stunting: a cluster randomized trial

BackgroundThe number of stunted children has fallen globally but continues to increase in Africa. Stunting is estimated to contribute to 14–17% of child deaths under 5 years of age and is a risk factor for poor cognitive and motor development and educational outcomes. Inadequate dietary intake and disease are thought to be the immediate causes of undernutrition and stunting. However, improving infant diets through complementary feeding interventions has been shown to only modestly reduce stunting. Multiple observational studies demonstrate a dose response relationship between fetal and post-natal aflatoxin exposure and reduced linear growth.MethodsThis community-based cluster randomized trial will measure the effect of a reduced aflatoxin diet on length-for-age Z scores at 18 months in central Tanzania. All 52 health facilities in the Kongwa District of Dodoma Region were randomized into two groups. Starting at 6 months of age, participants in the intervention group receive a low-aflatoxin pre-blended porridge flour containing maize and groundnut (ratio 4:1 respectively) and low-aflatoxin groundnut flour, whereas in the control group the same porridge mix and groundnut flour are promoted through education but acquired by the household. Both groups will receive the same infant and young child feeding education and a thermos flask. A total of 3120 infants between 6 weeks and 3 months of age will be recruited into the study over 1 year. Data will be collected four times – at recruitment and when the infants are 6, 12 and 18 months of age. In a cohort of 600 infants, additional data will be collected at 9 and 15 months of age. The primary outcome is length-for-age at 18 months. Secondary outcomes include the Z scores for weight-for-age, middle upper arm circumference and head circumference, and the blood biomarker aflatoxin-albumin in the full sample, with the urine biomarker aflatoxin M1 analyzed in the cohort only.DiscussionBetter understanding the etiology of childhood stunting can lead to more appropriate interventions and policies to further reduce linear growth faltering and meet the Sustainable Development Goals.Trial registrationNCT03940547, (April 24, 2019).

Gross motor developmental dysfunctional outcomes in infantile and toddler pediatric intensive care unit survivors

BackgroundIncreasing studies have focused on motor function/dysfunction in PICU survivors; however, most studies have focused on adults and older children. This study investigated gross motor developmental function outcomes in infantile and toddler pediatric intensive care unit (PICU) survivors and the factors associated with gross motor developmental functions.MethodsThis observational study was conducted in the PICU of the First Hospital of Jilin University between January 2019 and March 2019. Thirty-five eligible patients were divided into the dysfunctional (n = 24) or non-dysfunctional (n = 11) group according to the results of the Peabody Developmental Motor Scales, Second Edition (PDMS-2). Baseline gross motor function for all participants before PICU admission was measured via the Age and Stages Questionnaires, Third Edition (ASQ-3). The PDMS-2 was used to evaluate gross motor development function before PICU discharge.ResultsThe gross motor developmental dysfunction incidence was 68.6%. Linear correlation analysis showed that the gross motor quotient (GMQ) was positively correlated with the pediatric critical illness score (PCIS, r = 0.621, P < 0.001), and negatively correlated with length of PICU stay (r = − 0.556, P = 0.001), days sedated (r = − 0.602, P < 0.001), days on invasive mechanical ventilation (IMV; r = − 0.686, P < 0.001), and days on continuous renal replacement therapy (CRRT; r = − 0.538, P = 0.001). Linear regression analysis showed that IMV days (β = − 0.736, P = 0.001), sepsis (β = − 18.111, P = 0.003) and PCIS (β = 0.550, P = 0.021) were independent risk factors for gross motor developmental dysfunction.ConclusionsGross motor developmental dysfunction in infantile and toddler PICU survivors is more common and may be exacerbated by experiences associated with longer IMV days and increasing illness severity combined with sepsis.Trial registrationThe trial ‘Early rehabilitation intervention for critically ill children’ has been registered at http://www.chictr.org.cn/showproj.aspx?proj=23132. Registration number: ChiCTR1800020196.

Genetic variation in dopamine neurotransmission and motor development of infants born extremely-low-birthweight.

To determine if genetic variation associated with decreased dopamine neurotransmission predicts a decrease in motor development in a convenience cohort study of infants born extremely-low-birthweight (ELBW). Four hundred and ninety-eight infants born ELBW had genome-wide genotyping and a neurodevelopmental evaluation at 18 to 22months of age, corrected for preterm birth. A polygenic risk score (PRS) was created to combine into one predictor variable the hypothesized influences on motor development of alleles at seven independent single nucleotide polymorphisms previously associated with relative decreases in both dopamine neurotransmission and motor learning, by summing the number of alleles present in each infant (range=0-14). The motor development outcome was the Psychomotor Development Index (PDI) of the Bayley Scales of Infant Development, Second Edition. The linear regression models were adjusted for seven clinical and four genetic ancestry covariates. The mean PRS of infants with cerebral palsy (CP) was compared to those without CP. PRS was inversely related to PDI (p=0.011). Each 1-point increase in PRS resulted in an average decrease in PDI of 1.37 points. Patients with CP did not have a greater mean PRS than those without (p=0.67), both with and without adjustment for covariates. Genetic variation that favors a decrease in dopamine neurotransmission predisposes to a decrease in motor development in infants born ELBW, but not to the diagnosis of CP. Genetic variation in dopamine neurotransmission was associated with a decrease in motor development in infants born at an extremely-low-birthweight. It does not predispose to the diagnosis of cerebral palsy.

Effects of recombinant human growth hormone treatment on growth, body composition, and safety in infants or toddlers with Prader-Willi syndrome: a randomized, active-controlled trial

BackgroundPrader-Willi syndrome (PWS) is a rare complex genetic disorder and is characterized by short stature, muscular hypotonia, abnormal body composition, psychomotor retardation, and hyperphagia. Recombinant human growth hormone (rhGH) treatment improves the symptoms in children with PWS, and early treatment results in more favorable outcomes. However, systematic studies in infants and toddlers under 2 years of age are lacking. This multicenter, randomized, active-controlled, parallel-group, open-label, Phase III study aimed to evaluate the safety of rhGH (Eutropin, LG Chem, Ltd.) and its efficacy on growth, body composition, and motor and cognitive development in infants and toddlers with PWS compared with a comparator treatment (Genotropin, Pfizer, Inc.). Eligible Korean infants or toddlers with PWS were randomly assigned to receive Eutropin or comparator (both 0.24 mg/kg/week, 6 times/week) for 1 year. Height standard deviation score (SDS), body composition, and motor and cognitive development were measured.ResultsThirty-four subjects (less than 24 months old) were randomized into either the Eutropin (N = 17) group or the comparator (N = 17) group. After 52 weeks of rhGH treatment, height SDS and lean body mass increased significantly from baseline in both groups: the mean height SDS change (SD) was 0.75 (0.59) in the Eutropin group and 0.95 (0.66) in the comparator group, and the mean lean body mass change (SD) was 2377.79 (536.25) g in the Eutropin group and 2607.10 (641.36) g in the comparator group. In addition, percent body fat decreased significantly: the mean (SD) change from baseline was − 8.12% (9.86%) in the Eutropin group and − 7.48% (10.26%) in the comparator group. Motor and cognitive developments were also improved in both groups after the 1-year treatment. The incidence of adverse events was similar between the groups.ConclusionsrhGH treatment for 52 weeks in infants and toddlers with PWS improved growth, body composition, and motor and cognitive development, and efficacy and safety outcomes of Eutropin were comparable to those of Genotropin. Hence, Eutropin is expected to provide safe and clinically meaningful improvements in pediatric patients with PWS.Trial registrationThe study was registered at ClinicalTrials.gov (identifier: NCT02204163) on July 30, 2014.URL: https://clinicaltrials.gov/ct2/show/NCT02204163?term=NCT02204163&rank=1

Motor developmental outcomes in 2 babies with very severe osteogenesis imperfecta (type II)

Motor developmental outcomes in 2 babies with very severe osteogenesis imperfecta (type II)

Resting-State fMRI Networks in Children with Tuberous Sclerosis Complex.

There are no published studies examining resting state networks (RSNs) and their relationship with neurodevelopmental metrics in tuberous sclerosis complex (TSC). We aimed to identify major resting-state functional magnetic resonance imaging (rs-fMRI) networks in infants with TSC and correlate network analyses with neurodevelopmental assessments, autism diagnosis, and seizure history. Rs-fMRI data from 34 infants with TSC, sedated with propofol during the scan, were analyzed to identify auditory, motor, and visual RSNs. We examined the correlations between auditory, motor, and visual RSNs at approximately 11.5 months, neurodevelopmental outcome at approximately 18.5 months, and diagnosis of autism spectrum disorders at approximately 36 months of age. RSNs were obtained in 76.5% (26/34) of infants. We observed significant negative correlations between auditory RSN and auditory comprehension test scores (p = .038; r = -.435), as well as significant positive correlations between motor RSN and gross motor skills test scores (p = .023; r = .564). Significant positive correlations between motor RSNs and gross motor skills (p = .012; r = .754) were observed in TSC infants without autism, but not in TSC infants with autism, which could suggest altered motor processing. There were no significant differences in RSNs according to seizure history. Negative correlation between auditory RSN, as well as positive correlation between motor RSN and developmental outcome measures might reflect different brain mechanisms and, when identified, may be helpful in predicting later function. A larger study of TSC patients with a healthy control group is needed before auditory and motor RSNs could be considered as neurodevelopmental outcome biomarkers.

Motor Proficiency and Generalized Self-Efficacy Toward Physical Activity in Children With Intestinal Failure.

Survival rates of children with intestinal failure have increased; however, associated co-morbidities may affect long-term motor developmental outcomes. This study evaluates motor proficiency and generalized self-efficacy toward physical activity (PA) in children ages 6 to 12 years with intestinal failure. This is an observational, cross-sectional study of children followed in a multidisciplinary intestinal rehabilitation program. Motor proficiency was assessed using the Bruininks-Oseretsky Test of Motor Proficiency-2 Short Form (BOT-2 SF) and the Scales of Independent Behavior (parent-proxy report). Children completed the Children's Self-Perceptions of Adequacy in and Predilection for Physical Activity (CSAPPA) and a PA questionnaire. Relevant demographic and medical variables were correlated with assessment results. Participants include 30 children (18 males), median age 7 years (interquartile range [IQR] 6-9) with gestational age 35 weeks (IQR 32-39) and birth weight 2.13 kg (IQR 1.68-2.77). Thirteen (43%) were dependent on parenteral nutrition. Fifteen (50%) scored below average on the BOT-2 SF. Lower BOT-2 SF scores were significantly associated with lower CSAPPA scores (r = 0.480, P = 0.01), with a common barrier to PA being the presence of a central line or enterostomy tube. Gestational age, height z scores, length of hospital admissions, and number of septic events were all significantly correlated with lower scores in motor proficiency. Number of septic events and total parenteral nutrition days were significant predictors of lower BOT-2 SF scores, when adjusting for birth weight. Multiple medical variables related to intestinal failure may affect motor proficiency and PA self-efficacy. Developmental follow-up is important to optimize motor skill development and promote PA participation.

IMOVE: Intensive Mobility training with Variability and Error compared to conventional rehabilitation for young children with cerebral palsy: the protocol for a single blind randomized controlled trial

BackgroundCerebral palsy (CP) is the most common cause of physical disability in children. The best opportunity to maximize lifelong independence is early in motor development when there is the most potential for neuroplastic change, but how best to optimize motor ability during this narrow window remains unknown. We have systematically developed and pilot-tested a novel intervention that incorporates overlapping principles of neurorehabilitation and infant motor learning in a context that promotes upright mobility skill and postural control development. The treatment, called iMOVE therapy, was designed to allow young children with CP to self-initiate motor learning experiences similar to their typically developing peers. This manuscript describes the protocol for a subsequent clinical trial to test the efficacy of iMOVE therapy compared to conventional therapy on gross motor development and other secondary outcomes in young children with CP.MethodsThe study is a single-blind randomized controlled trial. Forty-two participants with CP or suspected CP between the ages of 1–3 years will be randomized to receive either the iMOVE or conventional therapy group. Distinguishing characteristics of each group are detailed. Repeated measures of gross motor function will be collected throughout the 12–24 week intervention phase and at three follow-up points over one year post therapy. Secondary outcomes include measures of postural control, physical activity, participation and caregiver satisfaction.DiscussionThis clinical trial will add to a small, but growing, body of literature on early interventions to optimize the development of motor control in young children with CP. The information learned will inform clinical practice of early treatment strategies and may contribute to improving the trajectory of motor development and reducing lifelong physical disability in individuals with CP.Trial registrationClinicalTrials.gov identifier NCT02340026. Registered January 16, 2015.

Clinical and neuropsychological outcomes for children with phenylketonuria in Upper Egypt; a single-center study over 5 years.

BackgroundPhenylketonuria (PKU) is considered to be a rare inborn error of metabolism but one of the commonest causes of mental retardation if untreated.ObjectivesThe present study was done to characterize the clinical patterns of PKU and analyze various neuropsychiatric outcomes in PKU children in Sohag Province, Egypt.Patients and methodsA prospective cohort study was conducted on 113 PKU patients, diagnosed during the period from 2012 to 2017, at the Pediatric Neurology Clinic of Sohag University Hospital, Upper Egypt. One hundred and ten cases were diagnosed based on clinical suspicion combined with laboratory confirmation by measuring their plasma phenylalanine levels using amino acid analyzer, while 3 cases were detected during neonatal screening. With the exception of the 3 cases detected during neonatal screening, all patients were clinically diagnosed and treated late. Psychometric evaluations of PKU patients were done using intelligence quotient (IQ, Stanford– Binet V), Childhood Autism Rating Scale, and Children’s Attention and Adjustment Survey. Dietetic management was applied. The results of neuroimaging (computed tomography or magnetic resonance imaging of the brain) and electroencephalography were included when available.ResultsThe overall results showed that 15.9% had hyperphenylalaninemia, 35.4% had mild to moderate PKU, and classic PKU was diagnosed in 48.7%. Global developmental delay (54.9%) and delayed language (29.2%) were the most frequent presentations. Moderately impaired or delayed overall IQ was present in 77%. While, 83.2% had moderately impaired or delayed verbal IQ, autism was diagnosed in 50.4%. Super average and average probability of attention-deficit hyperactivity disorder was diagnosed in 88.5%. Abnormal neuroimaging (white matter abnormalities and brain atrophy) was the most important significant predictor for poor language and motor developmental outcome (P<0.05).ConclusionPKU children had variable neuropsychological outcomes, mainly attention-deficit hyperactivity disorder and impaired verbal IQ, both of which were not related to the initial phenylalanine levels or to duration of dietary therapy, but were significantly related to early dietary intervention.

Comparison of writhing movements assessments in terms of predictive validity of neurological outcome between high-risk preterm and full-term infants

Objective To compare the predictive validity of writhing movements assessments of neurological outcome between high-risk preterm and full-term infants. Methods High-risk preterm and full-term infants who accepted the general movements(GMs) assessments from July 2013 to April 2014 and took part in follow-up regularly for 1 year in the Newborn Pediatrics Department of the Fifth Central Hospital of Tianjin were confirmed as the participants.GMs recordings during writhing movements period (at least once) and fidgety movements period (at least once) were collected and assessed.Neurological outcome was confirmed by Peabody Developmental Motor Scale-2(PDMS-2) when the patients were 1 year old.Then the predictive validities of writhing movements assessments of neurological outcome between high-risk preterm and full-term infants were calculated and compared. Results There was no significant difference in the detection rate of writhing movements between preterm and full-term infant groups(χ2=1.592, P=0.207). There was no significant difference in the detection rate of fidgety movements between preterm and full-term infant groups(χ2=1.605, P=0.205). The sensitivity was 92.9%, the specificity was 90.0%, and the negative predictive value was 97.8% in the stage of writhing movement to the motor development outcome in preterm infant group; the sensitivity was 85.7%, the specificity was 94.0%, and the negative predictive value was 95.9% in the stage of fidgety movement to the motor development outcome in preterm infant group; there was a good consistency between the assessment of writhing movement and neurological outcome confirmed by PDMS-2(Kappa=0.703, P<0.01). The specificity was 71.0%, the positive predictive value was 55.6% in the stage of writhing movement to the motor development outcome in full-term infant group; there was a worse consistency between the assessment of writhing movement and neurological outcome confirmed by PDMS-2(Kappa=0.555, P<0.01). Both the sensitivity and the specificity were 75.0% in the stage of writhing movement to the cerebral palsy in preterm infant group; there was a poor consistency between the assessment of writhing movement and neurological outcome confirmed by PDMS-2(Kappa=0.311, P<0.05). The specificity was 85.4%, the positive predictive value was 22.2% in the stage of writhing movement to the cerebral palsy in full-term infant group; there was still a poor consistency between the assessment of writhing movement and neurological outcome confirmed by PDMS-2(Kappa=0.319, P<0.05). Both the sensitivity and the negative predictive value were 100.0% in the stage of fidgety movement to the cerebral palsy in both preterm and full-term infant groups. Conclusions The predictive validity of writhing movements assessments to the motor development outcome in preterm infant group is higher than in full-term infant group, and it can be used as a tool for early and accurate prediction of neural development outcome of brain injured premature infants.The predictive validity of writhing movements assessments of the cerebral palsy is poor.Both the sensitivity and the negative predictive value were high in the stage of fidgety movement to the cerebral palsy in both preterm and full-term infant groups, and it may be used to predict the cerebral palsy earlier. Key words: Writhing movements; Infant, preterm; Brain injury; Motor development outcome; Predictive validity

Spontaneous movements of preterm infants is associated with outcome of gross motor development

AimsWe conducted a longitudinal cohort study to analyze the relationship between outcome of gross motor development in preterm infants and factors that might affect their development. MethodsPreterm infants with a birth weight of <1500 g were recruited. We measured spontaneous antigravity limbs movements by 3D motion capture system at 3 months corrected age. Gross motor developmental outcomes at 6 and 12 months corrected age were evaluated using the Alberta Infant Motor Scale (AIMS). Statistical analysis was carried out by canonical correlation analysis. ResultsEighteen preterm infants were included. In the 6 months corrected age analysis, spontaneous movement had a major effect on Prone and Sitting at 6 months corrected age of AIMS. In the 12 months corrected age analysis, spontaneous movement had a major effect on Sitting and Standing at 12 months corrected age of AIMS. ConclusionsIn preterm infants, better antigravity spontaneous movements at 3 months corrected age were significantly correlated with better gross motor development at 6 or 12 months corrected age.

Perinatal stroke - from symptoms to follow-up

We aimed to analyze patient characteristics of term neonates with the diagnosis of stroke between 2006 and 2017 at the 3rd level Neonatal Intensive Care Unit of the Szent János Hospital. We conducted a retrospective and prospective analysis including 18 newborns with stroke. Presentation, imaging methods, etiology and clinical context were discussed. All patients had a follow-up at 2 years of age or later. Subject of the study - In the past 10 years 17 term born and one premature neonate born at 36 weeks of age were diagnosed with stroke in our unit. All patients were born at good condition generally with high Apgar scores (9±1). Cesarean section was performed in 4 cases. With an estimated incidence of one in 1600-4000 births, the incidence of perinatal stroke in our unit was found to be the same as mentioned in the international databases. Regarding imaging method, cranial ultrasound scan do not visualise arterial ischaemic stroke therefore head MRI is recommended. Neurological symptoms of the patients presented in the first two days of life. Etiology included thrombophilia (4/18), infection (4/18), vascular malformation (2/18), moderate asphyxia (2/18) and pre-eclampsia (2/18). Middle cerebral artery was involved in 50% while the anterior cerebral artery was affected in 33%. The stroke occured in the left hemisphaerium in 44%, in the right side in 39% and was bilateral in 17%. In two cases the stroke was diagnosed in utero. Early childhood developmental support resulted in average or above average gross and fine motor development and cognitive outcome. Presenting neurological symptoms typically occur in the first few days after birth when perinatal stroke need to be considered among the broad spectrum of neonatal illnesses. Normal developmental outcome can be achieved even in cases of extensive brain damage with early childhood developmental support. Severely impaired development was observed in the cases of in utero stroke. Inherited prothrombotic disorders may have implications for subsequent pregnancies of the mother.

Application of movement recognition technology in assessing spontaneous general movements in preterm infants

Preterm birth is a major factor which induces neurological and motor impairments, particularly cerebral palsy, in high-risk infants. Early identification of potential neurodevelopmental impairments provides the opportunity to improve neurodevelopmental outcomes in preterm infants through early rehabilitation interventions. Clinically, the general movement assessment is a pivotal tool to predict neurodevelopmental outcomes, especially motor developmental outcomes, in high-risk infants. Movement recognition can continuously capture relevant limb movements and perform objective and quantitative assessment using computerized approaches. Various methods of recording and analyzing spontaneous general movements for infants at a risk of cerebral palsy have been extensively explored. This article summarizes the general movement assessment method and reviews the translational research on using movement recognition technology for the assessment of spontaneous general movements of preterm infants.

Long Term Motor Developmental Outcomes of Children Following Intestinal Transplantation

Long Term Motor Developmental Outcomes of Children Following Intestinal Transplantation

Validation of an MRI Brain Injury and Growth Scoring System in Very Preterm Infants Scanned at 29- to 35-Week Postmenstrual Age.

The diagnostic and prognostic potential of brain MR imaging before term-equivalent age is limited until valid MR imaging scoring systems are available. This study aimed to validate an MR imaging scoring system of brain injury and impaired growth for use at 29 to 35 weeks postmenstrual age in infants born at <31 weeks gestational age. Eighty-three infants in a prospective cohort study underwent early 3T MR imaging between 29 and 35 weeks' postmenstrual age (mean, 32+2 ± 1+3 weeks; 49 males, born at median gestation of 28+4 weeks; range, 23+6-30+6 weeks; mean birthweight, 1068 ± 312 g). Seventy-seven infants had a second MR scan at term-equivalent age (mean, 40+6 ± 1+3 weeks). Structural images were scored using a modified scoring system which generated WM, cortical gray matter, deep gray matter, cerebellar, and global scores. Outcome at 12-months corrected age (mean, 12 months 4 days ± 1+2 weeks) consisted of the Bayley Scales of Infant and Toddler Development, 3rd ed. (Bayley III), and the Neuro-Sensory Motor Developmental Assessment. Early MR imaging global, WM, and deep gray matter scores were negatively associated with Bayley III motor (regression coefficient for global score β = -1.31; 95% CI, -2.39 to -0.23; P = .02), cognitive (β = -1.52; 95% CI, -2.39 to -0.65; P < .01) and the Neuro-Sensory Motor Developmental Assessment outcomes (β = -1.73; 95% CI, -3.19 to -0.28; P = .02). Early MR imaging cerebellar scores were negatively associated with the Neuro-Sensory Motor Developmental Assessment (β = -5.99; 95% CI, -11.82 to -0.16; P = .04). Results were reconfirmed at term-equivalent-age MR imaging. This clinically accessible MR imaging scoring system is valid for use at 29 to 35 weeks postmenstrual age in infants born very preterm. It enables identification of infants at risk of adverse outcomes before the current standard of term-equivalent age.