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OBJECTIVE: To assess seizure and developmental outcomes, their predictors, and complications in 160 children who, between 1998 and 2022, underwent surgery for lesional epilepsy with curative intent before the age of 3 years. To compare trends in epilepsy surgery in this age group before and after the year 2014. METHODS: Retrospective multicenter study. Descriptive and univariate analyses, and multivariable models for all outcomes. RESULTS: These 160 patients (76 F; 47.5%) underwent 169 surgeries (age at surgery 20.4 ± 9.4 months). At the last follow-up (77 ± 57.4 months), 121 patients (75.6%) were in Engel class I, 106 (66.2%) of whom were in Engel class Ia. Antiseizure medications were stopped in 84 patients (52.5%). Complications requiring reoperations were observed in 16 patients (10%; 9.5% of surgeries) and unexpected permanent deficits in 12 (7.5%; 7.1% of surgeries). Postoperative cognitive functions remained unchanged in 56 patients (44.4%), improved in 51 (40.5%), and worsened in 19 (15.1%). Multivariable analyses showed that the probability of achieving Engel class Ia was lower when the duration of epilepsy was longer, patients underwent preoperative video-EEG, and unexpected postoperative permanent deficits occurred. Cognitive improvement after surgery was associated with lower preoperative seizure frequency, better preoperative developmental level, and a longer postoperative follow-up. FCDII and tumors were the histopathologies carrying a higher probability of achieving seizure freedom, while polymicrogyria was associated with a lower probability of cognitive improvement. The number of patients operated on after 2014 was higher than before (61.3% vs. 38.7%), with stable outcomes. SIGNIFICANCE: Epilepsy surgery is effective and safe in infants and toddlers, although the complication rate is higher than seen in older patients. Shorter duration of epilepsy, lower seizure frequency, no need for video-EEG, tumors, and some malformations of cortical development are robust predictors of seizure and cognitive outcome that may be exploited to increase earlier referral. PLAIN LANGUAGE SUMMARY: This study analyzed the results of epilepsy surgery in 160 children who had been operated on before the age of 3 years at four Italian centers between 1998 and 2022. At the last follow-up (77 ± 57.4 months), 121 patients (75.6%) were free from disabling seizures, of which 106 (66.2%) were completely seizure-free since surgery. Major surgical complications occurred in 28 patients (17.5%), which is higher than observed with epilepsy surgery in general, but similar to hemispheric/multilobar surgery. Postoperative cognitive function remained unchanged in 56 patients (44.4%), improved in 51 (40.5%), and worsened in 19 (15.1%). Epilepsy surgery is effective and safe in infants and toddlers.
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Epilepsia , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias , Humanos , Masculino , Feminino , Lactente , Estudos Retrospectivos , Pré-Escolar , Epilepsia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Resultado do Tratamento , Procedimentos Neurocirúrgicos/efeitos adversosRESUMO
BACKGROUND: It remains a matter of debate as to what extent early intervention may facilitate long-term functional outcomes of preterm infants in the neonatal intensive care unit (NICU). We aimed to examine the effect of increasing physical therapy (PT) staff dedicated to the NICU on temporal changes (initiation, duration) of PT interventions and functional outcomes (acquisition of full oral feeding and Hammersmith Neonatal Neurological Examination). METHODS: Extremely low birth weight infants, retrospectively collected from an academic medical center, were allocated to two subgroups, either a baseline period (N = 48) without NICU-dedicated PT staff (non-dedicated group) or a quality improvement period (N = 42) with additional dedicated staff (dedicated group). RESULTS: Compared to those in the non-dedicated group, NICU infants in the dedicated group started PT earlier and had increased PT treatment for additional 14 min per day when achieving full oral feeding. The infants in the dedicated group significantly achieved full oral feeding earlier than the non-dedicated group. As for Hammersmith Neonatal Neurological Examination, there were significant differences in two items (total and tone) between the groups. CONCLUSIONS: Additional NICU-dedicated PT staff facilitated earlier intervention and increased PT treatment in terms of daily duration. Moreover, the dedication shortened the completion of full oral feeding and improved neurological development, presumably resulting in better developmental outcome.
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Recém-Nascido de Peso Extremamente Baixo ao Nascer , Unidades de Terapia Intensiva Neonatal , Humanos , Recém-Nascido , Estudos Retrospectivos , Recém-Nascido de Peso Extremamente Baixo ao Nascer/fisiologia , Masculino , Feminino , Modalidades de Fisioterapia , Desenvolvimento Infantil/fisiologia , Recém-Nascido Prematuro/crescimento & desenvolvimento , Recém-Nascido Prematuro/fisiologiaRESUMO
Dravet syndrome is a severe infantile onset developmental and epileptic encephalopathy associated with mutations in the sodium channel alpha 1 subunit gene SCN1A. Prospective data on long-term developmental and clinical outcomes are limited; this study seeks to evaluate the clinical course of Dravet syndrome over a 10-year period and identify predictors of developmental outcome. SCN1A mutation-positive Dravet syndrome patients were prospectively followed up in the UK from 2010 to 2020. Caregivers completed structured questionnaires on clinical features and disease burden; the Epilepsy & Learning Disability Quality of Life Questionnaire, the Adaptive Behavioural Assessment System-3 and the Sleep Disturbance Scale for Children. Sixty-eight of 113 caregivers (60%) returned posted questionnaires. Developmental outcome worsened at follow-up (4.45 [SD 0.65], profound cognitive impairment) compared to baseline (2.9 [SD 1.1], moderate cognitive impairment, P < 0.001), whereas epilepsy severity appeared less severe at 10-year follow-up (P = 0.042). Comorbidities were more apparent at 10-year outcome including an increase in autistic features (77% [48/62] versus 30% [17/57], χ2 = 19.9, P < 0.001), behavioural problems (81% [46/57] versus 38% [23/60], χ2 = 14.1, P < 0.001) and motor/mobility problems (80% [51/64] versus 41% [24/59], χ2 = 16.9, P < 0.001). Subgroup analysis demonstrated a more significant rise in comorbidities in younger compared to older patients. Predictors of worse long-term developmental outcome included poorer baseline language ability (P < 0.001), more severe baseline epilepsy severity (P = 0.003) and a worse SCN1A genetic score (P = 0.027). Sudden unexpected death in epilepsy had not been discussed with a medical professional in 35% (24/68) of participants. Over 90% of caregivers reported a negative impact on their own health and career opportunities. Our study identifies important predictors and potential biomarkers of developmental outcome in Dravet syndrome and emphasizes the significant caregiver burden of illness. The negative impact of epilepsy severity at baseline on long-term developmental outcomes highlights the importance of implementing early and focused therapies whilst the potential impact of newer anti-seizure medications requires further study.
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BACKGROUND: Epilepsy has a high incidence among infants during their first year of life, yet the prognosis can vary significantly. Although considerable research has been conducted on infantile spasms, studies examining infantile-onset epilepsy, excluding infantile spasms, remain limited, particularly concerning the factors influencing outcomes. Therefore, our study aims to elucidate seizure control, developmental outcomes, and prognostic factors in infants with epilepsy during their first year of life, within a single-center study in Malaysia. METHODS: We retrieved data from patients who experienced seizures before age 12 months and were followed for over two years, using electronic patient records at Hospital Raja Perempuan Zainab II in Kelantan, a state in Malaysia's east coast. We retrospectively reviewed these records and assessed clinical outcomes based on the last follow-up. RESULTS: Of 75 patients, 61 (81.3%) achieved good seizure control or remission. At the last follow-up, 24 (32%) exhibited developmental delay, whereas 19 (25.3%) displayed abnormal neuroimaging. Patients with abnormal background electroencephalographic (EEG) activity, as well as abnormal radiological findings, were more likely to experience poor seizure control and unfavorable developmental outcomes (P < 0.05). CONCLUSIONS: Our study underscores that most infants with epilepsy can achieve seizure remission. However, poor seizure control and developmental delay are associated with abnormal EEG background and characteristics, as well as neuroimaging abnormalities. The management of infantile-onset epilepsies may necessitate substantial resources and precise interventions to enhance overall outcomes.
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Epilepsia , Espasmos Infantis , Lactente , Humanos , Espasmos Infantis/complicações , Estudos Retrospectivos , Convulsões/etiologia , Convulsões/complicações , Epilepsia/epidemiologia , Prognóstico , EletroencefalografiaRESUMO
AIM: To investigate the long-term effects of early-life recurrent otitis media (OM) and subsequent behavioural problems in children at the age of 10 years. METHODS: Data from the Raine Study, a longitudinal pregnancy cohort, were used to categorise children into those with three or more episodes of OM (rOM group) and those without a history of recurrent OM in the first 3 years of life (reference group). The parent report Strengths and Difficulties Questionnaire was used to assess child behaviour at the age of 10 years. Parental questionnaires were used to report past and present diagnoses of various mental health and developmental conditions, including attention, anxiety, depression, learning, and speech-language problems. Multiple linear and logistic models were used to analyse the data and were adjusted for a fixed set of key confounding variables. RESULTS: The linear regression analysis revealed significant, independent associations between a history of recurrent OM and higher Strengths and Difficulties Questionnaire scores, including total, internalising, externalising, emotional, attention/hyperactivity and peer problems subscales. Logistic regression analyses revealed an independent increased likelihood for children in the rOM group to have a diagnosis of attention, anxiety, learning and speech-language problems. CONCLUSION: Children at 10 years of age with an early history of recurrent OM are more likely to exhibit attentional and behavioural problems when compared to children without a history of recurrent OM. These findings highlight the association between early-life recurrent OM and later behavioural problems that may require professional allied health-care interventions.
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Otite Média , Comportamento Problema , Criança , Humanos , Estudos Longitudinais , Estudos de Coortes , Otite Média/diagnóstico , Otite Média/epidemiologia , Comportamento Infantil , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Abusive head trauma (AHT) is a major cause of traumatic brain injury in infancy. This exploratory study compared standardized developmental assessment versus functional outcome assessment between 18 months and 5 years of age following AHT in infancy. METHODS: Observational cross-sectional study after surviving AHT in infancy. Seventeen children between 18 months and 5 years of age underwent clinical examination, developmental assessment using the Schedule of Growing Skills II (SGS II) and functional assessment using the Glasgow Outcome Scale-Extended Pediatric Revision (GOS-E Peds). Additional clinical information was extracted from medical records. RESULTS: Age at assessment ranged from 19 to 53 months (median 26 months). Most (n = 14) were delayed in at least 1 domain, even without neurological or visual impairment or visible cortical injury on neuroimaging, including 8 children with favourable GOS-E Peds scores. The most affected domain was hearing and language. Delay in the manipulative domain (n = 6) was associated with visual and/or neurological impairment and greater severity of delay across multiple domains. Eleven (64.7 %) had GOS-E Peds scores indicating good recovery, with positive correlation between GOS-Peds scores and number of domains delayed (r = 0.805, p < 0.05). CONCLUSION: The SGS-II detects behavioural and cognitive deficits not picked up by the GOS-E Peds. Combining both tools for assessment of AHT survivors under 5 years of age provides a comprehensive profile which addresses multiple domains of development and function, facilitating targeted intervention. Detection of developmental problems in the majority of survivors makes AHT prevention a public health priority.
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Lesões Encefálicas Traumáticas , Transtornos Cognitivos , Traumatismos Craniocerebrais , Humanos , Lactente , Criança , Pré-Escolar , Estudos Transversais , Traumatismos Craniocerebrais/epidemiologia , Traumatismos Craniocerebrais/etiologiaRESUMO
Background and Objectives: Advanced old age is a life stage with a high likelihood of age-related loss experiences. However, little is known about remaining gain experiences and their relation with perceived losses and health correlates in community-dwelling very old adults. Moreover, virtually nothing is known in this regard about the experiences of individuals in long-term care settings. First, we strived to establish the normative course of age-related gains and losses in advanced old age. Second, we examined whether such gain/loss perceptions in advanced aging moderated health correlates. Research Design and Methods: Data came from the nationally representative survey "Old Age in Germany D80+" conducted in 2020/2021. The sample comprised 10 578 individuals aged 80-106 years, including 587 individuals in long-term care. We used the multidimensional Awareness of Age-Related Change (AARC) questionnaire and moderated regression to analyze associations with late-life health and functioning correlates. Results: Levels of AARC-Gains were higher than those of AARC-Losses across most of the age range. Long-term care residents showed more AARC-Losses and fewer AARC-Gains compared with community-dwelling adults and contributed significantly to an overall negative balance of more losses than gains in those aged 90 years or older. Regarding functional health and autonomy, negative age effects were amplified by AARC-Losses, but buffered by AARC-Gains. A more positive ratio of gains-to-losses predicted better health and functioning. Discussion and Implications: Findings suggest that the loss aspect of development in very late life might have been overstated in the existing literature. Perceived gains and losses are of critical importance for the understanding of health correlates in very old age.
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Epidemiological studies show that social determinants of health are among the strongest factors associated with developmental outcomes after prenatal and perinatal brain injuries, even when controlling for the severity of the initial injury. Elevated socioeconomic status and a higher level of parental education correlate with improved neurologic function after premature birth. Conversely, children experiencing early life adversity have worse outcomes after developmental brain injuries. Animal models have provided vital insight into mechanisms perturbed by developmental brain injuries, which have indicated directions for novel therapeutics or interventions. Animal models have also been used to learn how social environments affect brain maturation through enriched environments and early adverse conditions. We recognize animal models cannot fully recapitulate human social circumstances. However, we posit that mechanistic studies combining models of developmental brain injuries and early life social environments will provide insight into pathways important for recovery. Some studies combining enriched environments with neonatal hypoxic injury models have shown improvements in developmental outcomes, but further studies are needed to understand the mechanisms underlying these improvements. By contrast, there have been more limited studies of the effects of adverse conditions on developmental brain injury extent and recovery. Uncovering the biological underpinnings for early life social experiences has translational relevance, enabling the development of novel strategies to improve outcomes through lifelong treatment. With the emergence of new technologies to analyze subtle molecular and behavioral phenotypes, here we discuss the opportunities for combining animal models of developmental brain injury with social construct models to deconvolute the complex interactions between injury, recovery, and social inequity.
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Lesões Encefálicas , Transtornos Mentais , Criança , Recém-Nascido , Animais , Feminino , Gravidez , Humanos , Determinantes Sociais da SaúdeRESUMO
BACKGROUND: Extremely preterm (EPT) infants (≤28 weeks) remain at risk for poor outcomes. Small baby protocols (SBPs) may improve outcomes, but optimal strategies are unknown. METHODS: This study evaluated whether EPT infants managed using an SBP would have better outcomes compared to a historical control (HC) group. The study compared a HC group of EPT infants 23 0/7 weeks to 28 0/7 weeks GA (2006-2007), to a similar SBP group (2007-2008). Survivors were followed until 13 years of life. The SBP emphasized antenatal steroids, delayed cord clamping, respiratory and hemodynamic minimalism, prophylactic indomethacin, early empiric caffeine, and control of sound and light. RESULTS: There were 35 HC subjects and 35 SBP subjects. The SBP group had less severe IVH-PVH (9 % vs. 40 %, risk ratio 0.7, 95 % CI 0.5-0.9, P = 0.002) mortality (17 % vs. 46 %, risk ratio 0.6, 95 % CI 0.5-0.9, P = 0.004), and acute pulmonary hemorrhage (6 % vs. 23 %, risk ratio 0.8, 95 % CI 0.7-1.0, P = 0.04). Compliance with the SBP protocol was excellent. For the SBP group in the first 72 h, no subjects received inotropes, hydrocortisone, or sodium bicarbonate. Intubation, mechanical ventilation, fluid boluses, sedation, red blood cell transfusions, and insulin use decreased. At 10-13 years, more SBP subjects had survived without NDI (51 % vs. 23 %, risk ratio = 1.6, 95 % CI = 1.1-2.4, P = 0.01). More SBP subjects also survived without NDI and with a Vineland Adaptive Behavior Composite score > 85 (44 % vs. 11 %, risk ratio = 2.0, 95 % CI = 1.2-3.2, P ≤0.001). The SBP group had less visual impairment. CONCLUSION: An SBP was associated with improved outcomes, including normal neurologic survival after 10 years.
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Lactente Extremamente Prematuro , Melhoria de Qualidade , Lactente , Recém-Nascido , Humanos , Feminino , Gravidez , Indometacina , Recém-Nascido Pequeno para a Idade Gestacional , Hemorragia CerebralRESUMO
Background/objectives: Maternal illicit drug use is associated with negative physical and developmental outcomes for their born children. We aim to find out the incidence of different developmental problems in a cohort of Chinese children born to drug-abusing mothers, compare the physical health and developmental outcomes of the subjects recruited in the Integrated Program to the Comprehensive Child Development Service (CCDS), and to study the potential factors on their associations. Methods: A retrospective longitudinal cohort study with frequent clinical assessments of the children's physical and developmental outcomes in a HKSAR's regional hospital from birth until 5 years old. 123 Children in Integrated Program were compared with 214 children in CCDS between 1 January 2008 and 28 February 2019. Cox regression analysis was performed to determine the possible factors associated with the developmental outcomes. Results: Developmental delay was detected in 129 children (38.9%). CCDS group has significantly higher incidence of cognitive delay (p = < 0.001), language delay (p = < 0.001), motor delay (p = < 0.001), social delay (p = 0.002), and global delay (p = 0.002). On Cox multivariable regression analysis, integrated program (HRadj 0.53, 95% C. I. 0.34-0.84), social support (HRadj 0.45, 95% C.I. 0.25-0.80), and maternal abstinence from drug use up to 2-year post-delivery (HRadj 0.62, 95% C.I. 0.40-0.95) were significant protective factors, while male gender (HRadj 1.73, 95% C.I. 1.18-2.54) was a significant risk factor. Conclusion: CCDS achieves early engagement of drug-abusing expectant mothers during pregnancy, and an early integrated program with multidisciplinary collaboration was an independent factor in improving the developmental outcomes of these vulnerable children.
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BACKGROUND: This study examined the contextual factors associated with psychological need satisfaction (PNS) and the predictability of the PNS components, together with the contextual factors, on the developmental outcomes of elite young athletes in the Ethiopian sports academies, and further identified differences in perception of PNS from a comparative perspective. The study used a cross-sectional survey design applying developmental and PNS theories as guiding frameworks. Samples of elite young Ethiopian athletes participated (n = 257, 47.47% were women with a mean age of 17.44 years and SD = 0.87, and 52.53% were men with a mean age of 18.25 years and SD = 1.14). RESULTS: Structural equation modeling showed that the three PNS domains, together with the five contextual factors positively predicted the three developmental outcomes measured (41-54% explained variance). Moreover, there were higher differences in PNS (0.55 ≤ Cohen's d ≥ 0.71) among young athletes classified by the sport types. DISCUSSION: As per the findings of this study, young athletes may differ in the levels of PNS they obtained depending on the type of sports enrolled in sports academies. Also, the results of this study indicated that PNS attained may be modestly influenced by some contextual factors. It also evidenced that those developmental outcomes in elite young athletes are significantly positively associated with contextual and PNS factors. Stakeholders such as young athlete coaches, parents, sports psychologists, and administrators must consider the differing implications of program type during the elite young athletes' participation in sports academics and the significant positive association between contextual factors, PNS, and developmental outcomes of elite young athletes. CONCLUSIONS: In sum, the PNS of youth athletes may differ across sports types and the talent development of elite young athletes should emphasize the individual nature of the processes. Also, it can be concluded that the PNS components than the contextual factors had higher predictions of developmental outcomes.
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The literature concerning the neurocognitive evolution of children with transfusion-transfusion syndrome (TTS) is poor beyond the first year of life. It is therefore of particular interest to trace the developmental and management pathway, from the age of 18 months to 11 years, of a boy who presented with TTS in utero. This example illustrates the benefit of early multidisciplinary support and the diagnostic issues raised with the development of the child.
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Transfusão Feto-Fetal , Feminino , Humanos , Recém-Nascido , Masculino , GravidezRESUMO
Introduction: Mother-child interactions during the first years of life have a significant impact on the emotional and cognitive development of the child. In this work, we study how a prenatal diagnosis of malformation may affect maternal representations and the quality of these early interactions. To this end, we conducted a longitudinal observational study of mother-child interactions from the gestational stage until the baby completed 12 months of age. Participants and Methods: We recruited 250 pregnant women from a local university hospital. Among them, 50 mother-infant dyads participated in all stages of the study. The study group consisted of 25 pregnant women with fetuses with some structural alteration and the control group consisted of 25 pregnant women with fetuses without structural anomalies. We collected obstetric and socio demographic data and pregnancy outcomes. Anxiety and depressive state data were collected using the COVI and Raskin Scales. We video-recorded the mother-infant interactions during several stages, including when the child was a newborn and when the child was 2, 4, 6, 9, and 12 months of age. The quality of the mother infant interactions were measured using the Coding Interactive Behavior (CIB). The interactive moments recorded on video was composed of three different activities, each one lasting appoximately 3 min, which included (1) Free Interaction, where the mother was instructed to interact "as usual" without any toy, (2) Toy Interaction, where the mother and baby played with a puppv, and (3) Song Interaction, where the mother and baby interacted while the mother sang the "Happy Birthday" song. Results: In the gestational phase, there was a significant difference between the groups with respect to anxiety and depression scores, which were significantly higher for the study group. In the postnatal phase, we found significant differences between the groups with respect to CIB scales after the child completed 6 months of age: the study group presented significantly higher values of Maternal Sensitivity at 6 months of age, of Baby Involvement at 9 and 12 months of age, and of Dyadic Reciprocity at 6, 9, and 12 months of age, while the control group presented significantly higher values of Withdrawal of the Baby at 6 months of age, and of Dyadic Negative States at 6 and 9 months of age. Conclusion: The support offered by the study favored the mother-infant bond and had a positive effect on the quality of interaction during the first year of life, despite the presence of prenatal diagnosis.
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Identification of prenatal characteristics that predict later infant development may afford opportunities for early intervention, potentially optimizing childhood development outcomes. The aim of the present study was to examine the effects of selected prenatal factors (maternal-fetal attachment, maternal adult attachment, maternal well-being, and previous perinatal loss) on later infant development. Pregnant women were recruited from two antenatal clinics at one tertiary hospital and asked to complete self-report questionnaires. The Bayley's Scales of Infant and Toddler Development were then completed one to two years after their baby's birth. Independent samples t-tests, correlational analyses, and multivariate linear regression models were conducted. Results from 40 dyads revealed that more favorable maternal-fetal attachment, more secure/less anxious maternal attachment, and higher maternal well-being predicted maternal reports of infant adaptive behavior regardless of previous perinatal loss. Infants of women without perinatal loss scored higher in external observer-rated cognitive development compared to infants of women with previous perinatal loss. While further research is required, findings indicate that a mother's well-being and her relationship with her baby during pregnancy contributes to positive perceptions of her infant's daily living skills. Supporting the parenting of women with perinatal loss is required to, in turn, promote optimal cognitive development in infants.
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Desenvolvimento Infantil , Relações Mãe-Filho , Adulto , Criança , Feminino , Humanos , Lactente , Comportamento do Lactente , Relações Mãe-Filho/psicologia , Apego ao Objeto , Gravidez , Cuidado Pré-Natal/métodosRESUMO
Hemimegalencephaly (HME) is a rare brain congenital malformation, consisting in altered neuronal migration and proliferation within one hemisphere, which is responsible for early onset drug-resistant epilepsy. Hemispherotomy is an effective treatment option for patients with HME and drug-resistant epilepsy. Surgical outcome may be variable among different surgical series, and the long-term neuropsychological trajectory has been rarely defined using a standardized neurocognitive test. We report the epileptological and neuropsychological long-term outcomes of four consecutive HME patients, operated on before the age of three years. All patients were seizure-free and drug-free, and the minimum follow-up duration was of five years. Despite the excellent post-surgical seizure outcome, the long-term developmental outcome is quite variable between patients, ranging from mild to severe intellectual disabilities. Patients showed improvement mainly in communication skills, while visuo-perceptive and coordination abilities were more impaired. Epileptological outcome seems to be improved in early treated patients; however, neuropsychological outcome in HME patients may be highly variable despite early surgery.
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INTRODUCTION: Conventional methods of neurological assessment of infants can detect nervous system damage, but also have a weakness, i.e., the inability to make predictions for neurological deficits. Prechtl's general movement assessment is a diagnostic tool for the functional assessment of young nervous system. The aim of the study was to assess the quality of spontaneous motor activity in preterm newborns as well as to determine the neurological outcome at the age of 24 months. After that, the predictive value of spontaneous motor activity for neuro-developmental outcome at the age of 24 months was determined. MATERIAL AND METHODS: The study included 160 pre-terms children, and designed as a prospective clinical study. Observation of spontaneous motor activity was performed according to the principles of Prechtl's method. RESULTS: Spontaneous motor activity was observed in three periods for each newborn: within 5 days of birth, in the period of 44-46 gestation weeks, and in the period of 50-54 gestation weeks of post-menstrual age. Neurological outcome was assessed at the age of 24 months, and was classified as: normal finding, minimal neurological dysfunction, and cerebral palsy. All preterms, who presented normal patterns of spontaneous movements in neonatal and infant periods had a normal neurological functional outcomes at the age of 24 months. Newborns with pathological patterns of movement (cramped synchronized and absence of fidgety movements) in neonate and infant periods in the final outcomes had minimal neurological dysfunction or cerebral palsy. CONCLUSIONS: Assessment of general movement in preterms is a valuable method in prediction of dysfunctions in later neurological development. Early detection of symptoms of minimal neurological deficit and cerebral palsy is of crucial importance since it enables timely inclusion of children into neuro-developmental treatment.
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Paralisia Cerebral , Lactente , Criança , Humanos , Recém-Nascido , Pré-Escolar , Paralisia Cerebral/diagnóstico , Estudos Prospectivos , Recém-Nascido Prematuro/fisiologia , Movimento/fisiologia , Atividade Motora/fisiologiaRESUMO
OBJECTIVE: To determine epilepsy and neurodevelopmental outcomes beyond 2 y of age and their putative prognostic factors in children with West syndrome (WS). METHODS: This cross-sectional study was initiated after approval from Institutional Ethics Committee. A follow-up cohort of 114 children (aged ≥ 2 y) diagnosed and treated for WS at the authors' center were assessed in-person for epilepsy and neurodevelopmental outcomes using Vineland Social Maturity Scale - Malin's adaptation for Indian children. Subsequently, age at onset, lead-time-to-treatment, etiology, and response to any of the standard therapies were analyzed as possible predictors of these outcomes. RESULTS: Of 114 children (mean age: 55 ± 32 mo, 91 boys), structural etiology was the predominant underlying etiology (79.8%) for WS. At 2 y of age, 64% had ongoing seizures. At the last follow-up, 76% had social quotient < 55, and 39% had cerebral palsy (spastic quadriparesis in 21%). An underlying structural etiology was associated with ongoing seizures [OR (95% CI) 3.5 (1.4-9); p = 0.008] at 2 y of age and poor developmental outcomes [OR (95% CI): 3.3 (1.3-8.9); p = 0.016]. Complete cessation of spasms with the standard therapy was significantly associated with better seizure control [OR (95% CI): 5.4 (2.3-13); p < 0.001] and neurodevelopmental outcome [OR (95% CI): 5.2 (1.8-14.9); p < 0.001]. CONCLUSION: The majority of children with WS have a poor neurodevelopmental outcome and epilepsy control on follow-up. The underlying etiology and response to initial standard therapy for epileptic spasms have a prognostic role in predicting the neurological outcome in these patients on follow-up.
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Epilepsia , Espasmos Infantis , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Estudos Transversais , Eletroencefalografia , Epilepsia/complicações , Epilepsia/etiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Convulsões/tratamento farmacológico , Espasmo/complicações , Espasmo/tratamento farmacológico , Espasmos Infantis/diagnóstico , Espasmos Infantis/etiologia , Adulto JovemRESUMO
Down syndrome (DS) is the most prevalent neurodevelopmental disorder, with a known genetic cause. Besides facial dysmorphologies and congenital and/or acquired medical conditions, the syndrome is characterized by intellectual disability, accelerated aging, and an increased likelihood of an early onset Alzheimer's disease in adulthood. These common patterns of DS are derived from the long-held standard in the field of DS research, that describes individuals with DS as a homogeneous group and compares phenotypic outcomes with either neurotypical controls or other neurodevelopmental disorders. This traditional view has changed, as modern research pinpoints a broad variability in both the occurrence and severity of symptoms across DS, arguing for DS heterogeneity and against a single "DS profile." Nevertheless, prenatal counseling does not often prioritize the awareness of potential within-group variations of DS, portraying only a vague picture of the developmental outcomes of children with DS to expectant parents. This mini-review provides a concise update on existent information about the heterogeneity of DS from a full-spectrum developmental perspective, within an interdisciplinary context. Knowledge on DS heterogeneity will not only enable professionals to enhance the quality of prenatal counseling, but also help parents to set targeted early interventions, to further optimize daily functions and the quality of life of their children.
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Enteroviruses (EVs) and human parechoviruses (HPeVs) are a major cause of central nervous system (CNS) infection in young infants. They have been implicated in neurodevelopmental delay, however limited data are available. The aim of this study is to describe the clinical outcome of young infants and to assess and compare the medium-term neurodevelopment following CNS infections caused by EV and HPeV. A multicentre observational ambispective study was conducted between May 2013 and March 2018. Children under 3 months of age with EV or HPeV CNS infection excluding encephalitis were included. Infants were contacted 1 year after the acute infection and their neurological development was evaluated using the Ages and Stages Questionnaire-3 (ASQ-3). If any area assessed was abnormal during the first round of tests, a second round was completed 6 to 12 months later. Forty-eight young infants with EV and HPeV CNS infection were identified: 33 (68.8%) were positive for EV and 15 (31.3%) for HPeV. At first assessment 14 out of 29 EV (48.3%) and 3 out of 15 HPeV (20%) positive cases presented some developmental concern in the ASQ-3 test. EV-positive infants showed mild and moderate alteration in all domains analyzed and HPeV-positive infants showed mild alterations only in gross and fine motor domains. Significant alterations in communication were observed in EV-positive but not in HPeV-positive infants (31 vs. 0%, p = 0.016). At second assessment 4 out of 13 EV-positive patients (30.8%) showed mild to moderate concerns in communication and gross motor function domains and 3 out of 13 (23.1%) showed significant concern in fine motor function. Although CNS infections without associated encephalitis are generally assumed to be benign our study shows that at a median age of 18 months almost half of the EV-infected infants (48.3%) and 20% of HPeV-positive infants presented some developmental concern in the ASQ-3 test. We recommend monitor the neurological development of infants during the first years of life after HPeV CNS infection and especially after EV CNS infection, even in mild cases, for an early intervention and stimulation of psychomotor development if necessary.