Predictive value of the General Movements Assessment and Standardized Infant NeuroDevelopmental Assessment in infants at high risk of neurodevelopmental disorders.

AIM: To compare the predictive values of the General Movements Assessment (GMA) and the Standardized Infant NeuroDevelopmental Assessment (SINDA) neurological scale for atypical neurodevelopmental outcome in 3-month-old at-risk infants. METHOD: A total of 109 infants (gestational age 30 weeks; range: 24-41; 52 males) attending a non-academic outpatient clinic were assessed with the GMA and the SINDA at 3 (2-4) months corrected age. The GMA pays attention to the complexity of general movements and presence of fidgety movements. Atypical neurodevelopmental outcome at 24 months corrected age (and older) implied cerebral palsy (CP) or a Bayley Mental Development Index or Bayley Psychomotor Development Index lower than 70. RESULTS: At 24 months corrected (and older) age, 16 children had an atypical outcome, including 14 children with CP. Regarding markedly reduced general movement complexity in combination with absent or sporadic fidgety movements, the GMA predicted an atypical outcome with specificity, positive, and negative predictive values greater than 0.900, and sensitivity of 0.687 (95% confidence interval [CI] = 0.460-0.915). SINDA predicted an atypical outcome with sensitivity, specificity, and negative predictive value greater than 0.900 and a positive predictive value of 0.652 (95% CI = 0.457-0.847). Regarding absent fidgety movements only or markedly reduced general movement complexity, the GMA predicted the outcome less well than both general movement criteria. INTERPRETATION: The SINDA and GMA both predict neurodevelopmental outcome well, but SINDA is easier to learn than the GMA; being a non-video-based assessment, it allows caregiver feedback during the consultation whereas the GMA usually does not.

Standardized Infant NeuroDevelopmental Assessment developmental and socio-emotional scales: reliability and predictive value in an at-risk population.

AIM: To assess the reliability and predictive validity of the developmental and socio-emotional scales of the Standardized Infant NeuroDevelopmental Assessment (SINDA). METHOD: To assess reliability, two sets of three assessors forming eight assessor-pairs independently rated the developmental and socio-emotional scales of 60 infants. To evaluate predictive validity, 223 infants (gestational age 30wks [range 23-41wks]; 117 males, 106 females) attending a non-academic outpatient clinic were assessed by different assessors with SINDA's neurological, developmental, and socio-emotional scales. Atypical neurodevelopmental outcome at a corrected age of 24 months or older implied a Bayley Mental or Psychomotor Developmental Index score of less than 70 or neurological disorder (including cerebral palsy). Behavioural and emotional disorders were classified according to the International Classification of Diseases, 10th Revision. Predictive values were calculated from SINDA (2-12mo corrected age, median 7mo) and typical versus atypical outcome, and for intellectual disability only (Mental Developmental Index <70). RESULTS: Assessors highly agreed on the developmental and socio-emotional assessments (developmental scores: Spearman's rank correlation coefficient ρ=0.972; single socio-emotional behaviour items: Cohen's κ=0.783-0.896). At 24 months or older, 65 children had atypical outcome. Atypical neurological scores predicted atypical outcome (sensitivity 83%, specificity 96%); atypical developmental scores predicted intellectual disability (sensitivity 77%, specificity 92%). Atypical emotionality and atypical self-regulation were associated with behavioural and emotional disorders. INTERPRETATION: SINDA's three scales are reliable, and have a satisfactory predictive validity for atypical developmental outcome at 24 months or older in a non-academic outpatient setting. SINDA's developmental scale has promising predictive validity for intellectual disability. SINDA's socio-emotional scale is a tool for caregiver counselling. WHAT THIS PAPER ADDS: Standardized Infant NeuroDevelopmental Assessment (SINDA)'s developmental and socio-emotional scales have excellent interrater reliability. Replication of the satisfactory validity of SINDA's neurological scale for atypical outcome.

Reliability and predictive validity of the Standardized Infant NeuroDevelopmental Assessment neurological scale.

AIM: To assess reliability and predictive validity of the neurological scale of the Standardized Infant NeuroDevelopmental Assessment (SINDA), a recently developed assessment for infants aged 6 weeks to 12 months. METHOD: To assess reliability, three assessors independently rated video-recorded neurological assessments of 24 infants twice. Item difficulty and discrimination were determined. To evaluate predictive validity, 181 infants (median gestational age 30wks [range 22-41wks]; 92 males, 89 females) attending a non-academic outpatient clinic were assessed with SINDA's neurological scale (28 dichotomized items). Atypical neurodevelopmental outcome at 24 months or older corrected age implied a Bayley Mental Developmental Index or Psychomotor Developmental Index lower than 70 or a diagnosis of cerebral palsy (CP). Predictive values were calculated from SINDA (2-12mo corrected age, median 3mo) and typical versus atypical outcome. RESULTS: Intraclass correlation coefficients of intrarater and interrater agreement of the neurological score varied between 0.923 and 0.965. Item difficulty and discrimination were satisfactory. At 24 months or older, 56 children (31%) had an atypical outcome (29 had CP). Atypical neurological scores (below 25th centile, ≤21) predicted atypical outcome and CP with sensitivities of 89% and 100%, and specificities of 94% and 81% respectively. INTERPRETATION: SINDA's neurological scale is reliable and in a non-academic outpatient setting has a satisfactory predictive validity for atypical developmental outcome, including CP, at 24 months or older. WHAT THIS PAPER ADDS: The Standardized Infant NeuroDevelopmental Assessment's neurological scale has a good to excellent reliability. The scale has promising predictive validity for cerebral palsy. The scale has promising predictive validity for other types of atypical developmental outcome.

CONFIABILIDAD Y VALIDEZ PREDICTIVA DE LA ESCALA NEUROLÓGICA DE LA EVALUACIÓN DEL NEURODESARROLLO INFANTIL ESTANDARIZADA: OBJETIVO: Evaluar la confiabilidad y la validez predictiva de la escala neurológica de la Evaluación del Neurodesarrollo Infantil Estandarizada (SINDA), una evaluación desarrollada recientemente para bebés de 6 semanas a 12 meses. MÉTODO: Para evaluar la confiabilidad, tres evaluadores evaluaron dos veces, de forma independiente, las evaluaciones neurológicas grabadas en videos de 24 recién nacidos. Se determinaron la dificultad del ítem y la discriminación. Para evaluar la validez predictiva, se evaluaron 181 neonatos (mediana de edad gestacional de 30 semanas [rango 22-41 semanas], 92 varones, 89 mujeres) que asisten a una clínica ambulatoria no académica con la escala neurológica de SINDA (28 ítems dicotomizados). El resultado del desarrollo neurológico atípico a los 24 meses o mayor edad corregida implicaba un índice de desarrollo mental o índice de desarrollo psicomotor Bayley inferior a 70 o un diagnóstico de parálisis cerebral (PC). Los valores predictivos se calcularon a partir de SINDA (edad corregida 2-12mo, mediana 3meses) y resultado típico versus a atípico. RESULTADOS: Los coeficientes de correlación intraclase de la concordancia intra e inter codificador del puntaje neurológico variaron entre 0.923 y 0.965. La dificultad del item y la discriminación fueron satisfactorias. A los 24 meses o más, 56 niños (31%) tuvieron un resultado atípico (29 tuvieron PC). Las puntuaciones neurológicas atípicas (por debajo del percentil 25, ≤21) predijeron un resultado atípico y PC con sensibilidades del 89% y del 100%, y especificidades del 94% y del 81%, respectivamente. INTERPRETACIÓN: La escala neurológica de SINDA es confiable y en un entorno ambulatorio no académico tiene una validez predictiva satisfactoria para la detección del desarrollo atípico, incluido la PC, a los 24 meses o más.

CONFIABILIDADE E VALIDADE PREDITIVA DA ESCALA NEUROLÓGICA DE AVALIAÇÃO PADRONIZADA NEURODESENVOLVIMENTAL INFANTIL: OBJETIVO: Avaliar a confiabilidade e validade preditiva da escala neurológica Avaliação Padronizada Neurodesenvolvimental Infantil (SINDA), uma avaliação desenvolvida recentemente para lactentes de 6 semanas a 12 meses de idade. MÉTODO: Para avaliar a confiabilidade, por duas vezes três avaliadores pontuaram independentemente avaliações neurológicas de 24 lactentes registradas em vídeo. Para avaliar a validade preditiva, 181 lactentes (idade gestacional mediana de 30 semanas[variação de 22 a 41 semanas]); 92 do sexo masculino; 89 do sexo feminino) que frequentavam uma clínica não acadêmica foram avaliados com a escala neurológica da SINDA (28 itens dicotomizados). O neurodesenvolvimento atípico na idade de 24 meses de idade corrigida ou mais tarde foi determinado por índice desenvolvimental mental da Bayley ou Item desenvolvimental psicomotor menor do que 70 ou diagnóstico de paralisia cerebral (PC). Os valores preditivos foram calculados para o SINDA (2-12 meses de idade corrigida, mediana de 3 m) e resultado típico versus atípico. RESULTADOS: Os coeficientes de correlação intraclasse de concordância intra ou inter-examinadores do escore neurológico variaram de 0,923 a 0,965. A dificuldade e discriminação do item foram satisfatórias. Aos 24 meses de idade ou mais, 56 crianças (31%) tiveram resultado atípico (29 tinham PC). Os escores neurológicos atípicos (abaixo do percentil 25, ≤21) foram preditivos de resultado atípico e PC com sensibilidades de 89% e 100%, e especificidades de 94% e 81%, respectivamente. INTERPRETAÇÃO: A escala neurológica SINDA é confiável e em um ambiente não acadêmico tem validade preditiva satisfatória para resultado atípico do desenvolvimento, incluindo PC, aos 24 meses de idade ou mais.

Cerebellar Bottom-of-Fissure Dysplasia-a Novel Cerebellar Gray Matter Neuroimaging Pattern.

We report on seven patients with a novel neuroimaging finding that involves exclusively the cerebellar gray matter at the bottom of several fissures of both hemispheres but spares the vermis. The abnormal fissures were predominantly located in the lower and lateral parts of the cerebellar hemispheres. The affected cerebellar cortex was hypointense on T1-weighted and hyperintense on T2-weighted and fluid attenuation inversion recovery sequences. In some patients, the involved cerebellar gray matter was mildly thickened and the affected fissures slightly widened. In three of seven patients, the neuroimaging findings were unchanged on follow-up studies up to 6 years. The seven patients had various indications for the brain magnetic resonance imaging studies, and none of them had cerebellar dysfunction. Based on the similarity of the neuroimaging pattern with the cerebral "bottom-of-sulcus dysplasia," we coined the term "cerebellar bottom-of-fissure dysplasia" to refer to this novel neuroimaging finding. The neuroimaging characteristic as well as the unchanged findings on follow-up favors a stable "developmental" (malformative) nature. The lack of cerebellar dysfunction in the affected patients suggests that cerebellar bottom-of-fissure dysplasia represents most likely an incidental finding that does not require specific diagnostic investigation but allows a reassuring attitude.

Computer-based analysis of general movements reveals stereotypies predicting cerebral palsy.

AIM: To evaluate a kinematic paradigm of automatic general movements analysis in comparison to clinical assessment in 3-month-old infants and its prediction for neurodevelopmental outcome. METHOD: Preterm infants at high risk (n=49; 26 males, 23 females) and term infants at low risk (n=18; eight males, 10 females) of developmental impairment were recruited from hospitals around Heidelberg, Germany. Kinematic analysis of general movements by magnet tracking and clinical video-based assessment of general movements were performed at 3 months of age. Neurodevelopmental outcome was evaluated at 2 years. By comparing the general movements of small samples of children with and without cerebral palsy (CP), we developed a kinematic paradigm typical for infants at risk of developing CP. We tested the validity of this paradigm as a tool to predict CP and neurodevelopmental impairment. RESULTS: Clinical assessment correctly identified almost all infants with neurodevelopmental impairment including CP, but did not predict if the infant would be affected by CP or not. The kinematic analysis, in particular the stereotypy score of arm movements, was an excellent predictor of CP, whereas stereotyped repetitive movements of the legs predicted any neurodevelopmental impairment. INTERPRETATION: The automatic assessment of the stereotypy score by magnet tracking in 3-month-old spontaneously moving infants at high risk of developmental abnormalities allowed a valid detection of infants affected and unaffected by CP.

Validation of a screening tool for attention and executive functions (EpiTrack Junior) in children and adolescents with absence epilepsy.

OBJECTIVE: Our prospective study aimed at the validation of EpiTrack Junior, a neuropsychological screening tool for attention and executive functions in children with epilepsy. METHODS: Twenty-two children with absence epilepsy aged 8-17 years underwent comprehensive neuropsychological evaluation including EpiTrack Junior and measures of intelligence, verbal and nonverbal memory, word fluency and visuoconstructive organization. Concurrent and discriminant validity of EpiTrack Junior subtests and total score as well as sensitivity and specificity of the total score were analyzed. RESULTS: EpiTrack Junior total score was impaired in 59% of participants. Concurrent validity was demonstrated in 4/6 subtests and for the total score. Discriminant validity was shown with respect to verbal and nonverbal long-term memory. Sensitivity was higher than specificity and highest for the "working memory index". CONCLUSION: EpiTrack Junior is recommended as a sensitive and time-efficient screening tool for attention and executive functions in children with epilepsy. Impaired results should be followed up with detailed evaluation including information from the parents and school as well as counseling where indicated.

Pattern and predictors of complementary and alternative medicine (CAM) use among pediatric patients with epilepsy.

Parents of pediatric patients with chronic conditions such as epilepsy increasingly opt for complementary and alternative medicine (CAM). However, data on the pattern and reasons of CAM use in childhood epilepsy are scarce. The objectives of this study were as follows: first, to characterize CAM use among pediatric patients with epilepsy by assessing its spectrum, prevalence, costs, and frequency of use; second, to evaluate the influence of CAM use on compliance and satisfaction with conventional care as well as to explore parent-child neurologist communication concerning CAM; and third, to investigate predictors of CAM use. A postal survey was administered to all parents of pediatric outpatients with epilepsy aged 6 to 12, who have received treatment at the neuropediatric outpatient clinic of the University Children's Hospital Heidelberg between 2007 and 2009. One hundred thirty-two of the 297 distributed questionnaires were suitable for inclusion in statistical analysis (44.7%). Forty-nine participants indicated that their children used CAM during the previous year (37.1%). Thirty different types of CAM were used, with homeopathy (55.1%), osteopathy (24.5%), and kinesiology (16.3%) being the most commonly named. A mean of 86€ (0€-500€) and 3h (1 h-30 h) per month was committed to CAM treatment. Only 53% of the users informed their child neurologist of the additional CAM treatment, while 85.6% of all parents wished to discuss CAM options with their child neurologist. Seventy-five percent of users considered the CAM treatment effective. Among the participants most likely to seek CAM treatment are parents whose children show a long duration of epileptic symptoms, parents who make use of CAM treatment themselves, and parents who value a holistic and natural treatment approach. A substantial portion of pediatric patients with epilepsy receive CAM treatment. The high prevalence of use and significant level of financial and time resources spent on CAM indicate the high importance of these treatment options for parents. On the other hand, communication concerning CAM with the child neurologist is largely insufficient despite the wish to speak about CAM. Complementary and alternative medicine users' high compliance with conventional treatment and high perceived effectiveness of CAM support an integrative approach to CAM for pediatric patients with epilepsy. Our study implies that in addition to open parent-child neurologist communication, active inquiry on CAM treatments is necessary to enable informed decision making by parents and to establish the suitability of CAM treatment for the patient. Reliable predictors for CAM use, which allow for improved identification of patients with a high likelihood to receive CAM treatment, are the duration of the illness, use of CAM by the parents themselves, and the desire of the parents to receive a holistic and natural treatment for their child.

Comparison of the developmental tests Bayley-III and Bayley-II in 7-month-old infants born preterm.

The study aims on comparing Bayley Scales of infant development third (Bayley-III) and Bayley second (Bayley-II) edition with special focus on patterns in the first year of life. Fifty-five premature infants (43 with low birth weight/LBW >1,499 g and 12 with very/extremely low birth weight/VLBW/ELBW <1,500 g) aged 7 months (corrected for prematurity) were assessed with the complete Bayley-III. From this assessment, Bayley-II results were retrospectively estimated. Bayley-III results were compared to the expected mean with one-sample t-tests. The mean scores of both editions were compared with the aid of paired-sample t-tests. Pearson correlations between subscales and editions were analysed. The Bayley-III cognitive score of the study group was significantly higher than the expected mean of the standardization sample. VLBW/ELBW had significantly lower motor scores than LBW in both editions. When compared to estimated Bayley-II scores, all relevant Bayley-III scores were significantly higher (all p < .01) with highest difference (ten points) between the motor scales of both editions. There were significant correlations not only between Bayley-III cognitive and language scales but also between language and motor scales. Given the strong association between motor and cognitive behaviour in early infancy, this age-specific pattern is heightening the risk of failure to identify infants at risk for both cognitive and motor delay. Therefore, assessment of infants should comprise all subscales. Since Bayley-III probably overestimates especially motor performance in young infants, when interpreting Bayley-III scores in this age, comparison groups are highly recommended until further validation of normative data are outstanding.

Quantification of the segmental kinematics of spontaneous infant movements.

This article introduces a method to capture the movements of the upper and the lower limb of infants using an electromagnetic tracking system and to reliably calculate the segmental kinematics. Analysis of the spontaneous movements of infants is important e.g. in the context of the "General Movement Analysis", which aims at the early diagnosis of motor dysfunctions. Due to special constraints regarding infant anatomy, previous approaches based on optical tracking could only gather position data of the infant' segments, whereas with this method in addition relative segment angles can be calculated. The spontaneous movements of the infant and simple calibration movements of the hand and the foot are used to calculate the joint centers and the joint axes of a multi-segmental chain model. The quality of the calibration movements is assessed at calibration time by calculating the root mean square deviation from the total least squares regression plane. The general accuracy of the recording is evaluated by the difference between recorded and estimated sensor positions and the difference between recorded and estimated sensor orientations. Movements of 20 infants between term and 3 months post term age were recorded and processed. A first application illustrates how abnormal movement patterns are manifested in the segmental kinematics. The results show that the presented method is a practicable and reliable way to record spontaneous infant movements and to calculate the segmental kinematics.

Auditory temporal resolution in children assessed by magnetoencephalography.

By means of magnetoencephalography we investigated the auditory-evoked fields (AEFs) elicited by broadband noise bursts in a gap-detection paradigm in children. AEFs of 16 healthy children (mean age 8.7 years) were recorded while they passively listened to 100-ms white-noise bursts with temporal gaps of 3, 6, 10 and 30 ms inserted after 5 or 50 ms. The peak of the earliest and largest positivity occurred at 97 ms and was evaluated by spatiotemporal source analysis. Psychophysical gap-detection thresholds were obtained for the same children. We found that the neuromagnetic gap responses corresponded to the psychoacoustic thresholds. AEFs thus provide an objective tool to assess auditory temporal resolution in children. Children's neuromagnetic response patterns differed significantly from the adult responses under the same experimental conditions.

[Family sense of coherence and resilience. A study on families with children with mental and physical disabilities]. / Kohärenz und Resilienz in Familien mit geistig und körperlich behinderten Kindern.

Drawing from concepts from family stress theory, resilience theory, Rolland's model of family adaptation to disability, and Antonovsky's salutogenetic model, this study explores the relationship between family sense of coherence and measures of family functioning, levels of stress, severity of the handicaps levels of functional impairment and demographic indicators. Participants were 37 families with children under 18 years with mental retardation and physical-neurological disorders that consulted the Department of Pediatric Neurology. With a discriminant analysis, measures contributing to the separation into subgroups of families with high, medium and low family cohesion were identified. The measures permitted a satisfactory discrimination into groups in this clinical sample. The highest discriminatory power held sociodemographic variables, severity of impairment, as well as levels of family functioning and measures of family coping, which are potentially amenable to therapeutic change. The results indicate that family coherence can be regarded as a global resource contributing to family resilience, and therapeutic implications are being discussed.

MR imaging-based volumetry in patients with early-treated phenylketonuria.

BACKGROUND AND PURPOSE: Our purpose was to specify the most severely affected brain structures in early treated phenylketonuria regarding volume loss and establish possible correlations between volume loss and plasma levels of phenylalanine (Phe). METHODS: In 31 patients with early treated phenylketonuria and in 27 healthy volunteers, we acquired volumetric MR imaging data. Serum Phe concentrations at different times were measured as well. Semiautomatic volumetric postprocessing of the cerebellum, cerebrum (supratentorial brain tissue), hippocampus, intracranial volume, lateral ventricles, nucleus caudatus, nucleus lentiformis, pons, and thalamus, as well as the two-dimensional extension of the corpus callosum, was performed using the software BRAINS2. For each separate brain structure, the relative differences between the normal and the phenylketonuria group (delta(rel)) were calculated. RESULTS: The cerebrum, corpus callosum, hippocampus, intracranial volume, and pons were significantly smaller in patients with phenylketonuria than in healthy patients. The volume of the lateral ventricles was significantly larger in patients with phenylketonuria than in healthy ones. The most severely affected structures were the pons (delta(rel) = 16%), hippocampus (delta(rel) = 14.5%), cerebrum (delta(rel) = 13%), and corpus callosum (delta(rel) = 10%). No significant differences were found for the basal ganglia, cerebellum, and thalamus. There were no significant correlations found between the volume of any of the different brain structures and the metabolic parameters. CONCLUSION: The most severely affected brain structures in early-treated patients with phenylketonuria regarding volume loss are the cerebrum, corpus callosum, hippocampus, and pons.

Cognitive development in 7- to 24-month-old extremely/very-to-moderately/late preterm and full-term born infants: The mediating role of focused attention.

The present study analyzed the links between prematurity, attention, and global cognitive performance in infancy and early childhood. At 7 months, focused attention (FA) was examined with an object examination task in 93 preterm infants (39 of them born extremely/very preterm, 54 born moderately/late preterm, and 38 infants born full-term). Global cognition was assessed at 7 and 24 months with the Bayley-II cognitive scale. Groups did not differ with respect to global cognitive performance but FA of infants born extremely/very preterm was significantly lower than in infants born moderately/late preterm. FA correlated significantly with both prematurity and cognitive performance at 7 months of age but not with global cognition in childhood. Findings point to a subtle adverse effect of prematurity on early attention and reveal evidence for the mediating role of FA on the effect of prematurity on cognition.

Phenotypic and molecular insights into CASK-related disorders in males.

BACKGROUND: Heterozygous loss-of-function mutations in the X-linked CASK gene cause progressive microcephaly with pontine and cerebellar hypoplasia (MICPCH) and severe intellectual disability (ID) in females. Different CASK mutations have also been reported in males. The associated phenotypes range from nonsyndromic ID to Ohtahara syndrome with cerebellar hypoplasia. However, the phenotypic spectrum in males has not been systematically evaluated to date. METHODS: We identified a CASK alteration in 8 novel unrelated male patients by targeted Sanger sequencing, copy number analysis (MLPA and/or FISH) and array CGH. CASK transcripts were investigated by RT-PCR followed by sequencing. Immunoblotting was used to detect CASK protein in patient-derived cells. The clinical phenotype and natural history of the 8 patients and 28 CASK-mutation positive males reported previously were reviewed and correlated with available molecular data. RESULTS: CASK alterations include one nonsense mutation, one 5-bp deletion, one mutation of the start codon, and five partial gene deletions and duplications; seven were de novo, including three somatic mosaicisms, and one was familial. In three subjects, specific mRNA junction fragments indicated in tandem duplication of CASK exons disrupting the integrity of the gene. The 5-bp deletion resulted in multiple aberrant CASK mRNAs. In fibroblasts from patients with a CASK loss-of-function mutation, no CASK protein could be detected. Individuals who are mosaic for a severe CASK mutation or carry a hypomorphic mutation still showed detectable amount of protein. CONCLUSIONS: Based on eight novel patients and all CASK-mutation positive males reported previously three phenotypic groups can be distinguished that represent a clinical continuum: (i) MICPCH with severe epileptic encephalopathy caused by hemizygous loss-of-function mutations, (ii) MICPCH associated with inactivating alterations in the mosaic state or a partly penetrant mutation, and (iii) syndromic/nonsyndromic mild to severe ID with or without nystagmus caused by CASK missense and splice mutations that leave the CASK protein intact but likely alter its function or reduce the amount of normal protein. Our findings facilitate focused testing of the CASK gene and interpreting sequence variants identified by next-generation sequencing in cases with a phenotype resembling either of the three groups.

Physical growth and neurodevelopmental outcome of nonhandicapped low-risk children born preterm.

BACKGROUND: Outcome studies on the effects of prematurity are increasingly restricted to extremely immature infants with birth weight below 1000 g or gestational age below 26 weeks. In contrast, studies comprising low-risk preterm infants are rare. AIM: To examine growth and neurodevelopmental outcome, 70 low-risk low birth weight (LBW) children without neurological impairment were followed from birth to 7 years of age. At 7 years of age, LBW children were compared to a matched control group born at term. METHODS: Postnatal growth was measured at 20 months in the LBW group and at 7 years in LBW and control children. At 20 months, the LBW group was assessed with the Griffiths Scales. At 7 years, LBW and control children were assessed with a neuropsychological test battery comprising tests for language, visual-perceptual, visual-motor, fine and gross motor abilities. RESULTS: At 7 years of age, the frequency of children with low (3rd-9th percentile) or subnormal (<3rd percentile) growth parameters was increased in the LBW group. The Mean Griffiths Developmental Quotient (DQ) of the preterm group was normal (102.3+/-8.4), and there were only two results below DQ 85. There was no difference between 49 children appropriate for gestational age and 21 small for gestational age (SGA) children. At 7 years of age, reduced mean test results in the range of -0.5 SDS were observed for language and visual-motor abilities in the preterm group. This was due to an increased frequency of LBW children with moderately (SDS -1.0 to -2.0 SDS) subnormal test results. Even for the slightly LBW group (2000 to 2499 g), poorer language abilities were confirmed. CONCLUSION: All LBW infants, including low-risk populations, should be included in a follow-up program in order to detect deficits early in life and begin treatment before school entry.

Negative reactivity in toddlers born prematurely: indirect and moderated pathways considering self-regulation, neonatal distress and parenting stress.

High negative reactivity in early childhood interferes with later academic and behavioral adjustment. Thus, investigating the origins of high negative affectivity in early childhood is of high relevance for understanding emotional morbidity after preterm birth. The present work explored (1) direct prematurity-related consequences for negative reactivity, (2) self-regulatory deficits as a mechanism indirectly relating prematurity to negative affectivity and (3) the implications of the interplay between procedural distress in the neonatal period and parenting stress for preterm children's negative reactivity. The sample was comprised of 146 preterm children (very vs. moderately to late preterm) and 86 healthy full-term children, both free of major neurological impairment. Assessment involved negative affect and parenting stress (parent-report; 12, 24 months corrected age, CA), effortful control (behavioral battery, parent report; 24 months CA) and the number of potentially distressing neonatal intensive care procedures as well as severity of illness during the neonatal period (retrospective chart review). There was no direct link from prematurity to a disposition for high negative reactivity in early childhood nor was prematurity indirectly associated with higher negative reactivity through lower levels of effortful control. The relation between neonatal pain and distress and negative affectivity depended on the level of parenting stress with low parenting stress at the end of the first year of children's life buffering the negative influence of neonatal distress. The present findings underscore the importance of complex interactions among environmental factors in processes of emotional plasticity after preterm birth thereby providing critical suggestions for follow-up care.

Cognitive development in very vs. moderately to late preterm and full-term children: can effortful control account for group differences in toddlerhood?

BACKGROUND: Preterm birth is thought to have an adverse impact on cognitive development and self-regulation. AIM: Examining the effect of very vs. moderately to late premature birth on cognitive development and effortful control, as well as evaluating whether effortful control explains the link between preterm birth and poorer cognitive development. SUBJECTS: Fifty-eight very preterm children (<32 weeks gestation or <1500 g birth weight), 88 moderately to late preterm children (≥32 weeks gestation and ≥1500 birth weight) and 86 full-term children (≥38 weeks gestation and ≥2500 g birth weight) were examined at the corrected age of 24 months. OUTCOME MEASURES: Observational and parent-report measures of effortful control as well as the Bayley Scales of Infant Development II (BSID II, Mental Scale) as a measurement of cognitive development were analyzed. RESULTS: Very preterm and moderately to late preterm children showed significantly lower cognitive performance compared to full-term children. Lower effortful control scores (on observational measures, but not on parent-reports) were merely found for very preterm children compared to full-term children. Observational measures of effortful control partially mediated the effects of very preterm birth on cognitive performance, but did not explain the effects of moderately to late preterm birth on cognitive performance. CONCLUSION: Preterm birth in general is related to poorer cognitive performance in toddlerhood. In addition, effortful control mediates the effects of very preterm birth on cognitive development. Findings suggest that different mechanisms link moderately to late premature birth to poor cognitive development.

Kinematic assessment of stereotypy in spontaneous movements in infants.

Movement variation constitutes a crucial feature of infant motor development. Reduced variation of spontaneous infant movements, i.e. stereotyped movements, may indicate severe neurological deficit at an early stage. Hitherto evaluation of movement variation has been mainly restricted to subjective assessment based on observation. This article introduces a method for quantitative assessment yielding an objective definition of stereotyped movements which may be used for the prognosis of neurological deficits such as cerebral palsy (CP). Movements of 3-month-old infants were recorded with an electromagnetic tracking system facilitating the analysis of joint angles of the upper and lower limb. A stereotypy score based on dynamic time warping has been developed describing movements which are self-similar in multiple degrees of freedom. For clinical evaluation, this measure was calculated in a group of infants at risk for neurological disorders (n=54) and a control group of typically developing children (n=21) on the basis of spontaneous movements at the age of 3 months. The stereotypy score was related to outcome at the age of 24 months in terms of CP (n=10) or no-CP (n=53). Using the stereotypy score of upper limb movements CP cases could be identified with a sensitivity of 90% and a specificity of 96%. The corresponding score of the leg movements did not allow for valid discrimination of the groups. The presented stereotypy feature is a promising candidate for a marker that may be used as a simple and noninvasive quantitative measure in the prediction of CP. The method can be adopted for the assessment of infant movement variation in research and clinical applications.