Language：English   Publishing type：Research paper (scientific journal)  

Hypoxic-ischemic encephalopathy (HIE), associated with high mortality and neurological sequelae, lacks established treatment except therapeutic hypothermia. Clinical-grade multilineage-differentiating stress-enduring (Muse) cells (CL2020) demonstrated safety and efficacy in nonclinical HIE rat models, thereby leading to an investigator-initiated clinical trial to evaluate CL2020 safety and tolerability in neonatal HIE as a single-center open-label dose-escalation study with 9 neonates with moderate-to-severe HIE who received therapeutic hypothermia. Each patient received a single intravenous injection of CL2020 cells between 5 and 14 days of age. The low-dose (3 patients) and high-dose (6 patients) groups received 1.5 × 106 and 1.5 × 107 cells/dose, respectively. The occurrence of any adverse event within 12 weeks following CL2020 administration was the primary endpoint of this trial. No significant changes in physiological signs including heart rate, blood pressure, and oxygen saturation were observed during or after administration. The only adverse event that may be related to cell administration was a mild γ-glutamyltransferase level elevation in one neonate, which spontaneously resolved without any treatment. All patients enrolled in the trial survived, and normal developmental quotients (≥ 85) in all 3 domains of the Kyoto Scale of Psychological Development 2001 were observed in 67% of the patients in this trial. CL2020 administration was demonstrated to be safe and tolerable for neonates with HIE. Considering the small number of patients, a randomized controlled confirmatory study is warranted to verify these preliminary findings and evaluate the efficacy of this therapy.

DOI： 10.1093/stcltm/szae071

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: Infants born to mothers with hypertensive disorders of pregnancy (HDP) reportedly have negative behavioral and neurodevelopmental outcomes. However, the effects of maternal HDP on infant brain growth have not been fully evaluated. We aimed to evaluate brain volumes and brain injury in preterm infants born to mothers with HDP using magnetic resonance (MR) imaging at term-equivalent age. STUDY DESIGN: In this cohort study, MR imaging was performed for 94 preterm infants born before 34 weeks of gestation at Nagoya University Hospital between 2010 and 2018. Twenty infants were born to mothers with HDP and 74 to mothers without HDP. MAIN OUTCOME MEASURES: Total brain volumes and regional volumetric alterations were assessed by voxel-based morphometry, and brain injury was evaluated using the Kidokoro global brain abnormality score. Developmental quotient was assessed at a corrected age of 1.5 years in 59 infants (HDP, n = 11; non-HDP, n = 48). RESULTS: No significant differences were observed in the gray and white matter volumes of the two groups (HDP: 175 ± 24 mL, 137 ± 13 mL, respectively; non-HDP: 172 ± 24 mL, 142 ± 13 mL, respectively). Additionally, no regional volumetric alterations were observed between the two groups after covariate adjustment (gestational age and infant sex). The total Kidokoro score and developmental quotient were similar in both groups. CONCLUSIONS: No significant differences in the global and regional brain volumes were observed. Further research is needed to confirm our findings at different time points of MR imaging and in different populations.

DOI： 10.1016/j.preghy.2021.06.003

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Repetitive sleep starts (RSS) are clusters of nonepileptic, spasm-like movements occurring during sleep onset. However, their characteristics have yet to be defined. We conducted a clinicoelectroencephalographic study of children with RSS to clarify their detailed characteristics. METHODS: To differentiate starts from epileptic spasms, we recruited children with brief "crescendo-decrescendo" muscle contractions that simultaneously involved the limbs and trunk without electroencephalogram changes, and that fulfilled the following criteria: (1) repeated occurrence (five or more) and (2) manifestation during sleep stage N1-N2. A total of nine children met these criteria. Their clinical information and video-electroencephalogram data were analyzed retrospectively. RESULTS: The background conditions observed at onset of RSS were perinatal hypoxic-ischemic encephalopathy (n = 4), West syndrome of unknown etiology (n = 1), and traumatic brain injury (n = 1). The age at onset of RSS, the number of starts in a given RSS cluster, the interval between starts, and the duration of surface electromyogram activity were between 3 and 46 months, 5 and 547, <1 and 60 s, and 0.3 and 5.4 s, respectively. None of the median value of these parameters differed between children with and without corticospinal tract injury. During the median follow-up period of 33 months, RSS disappeared spontaneously in five. CONCLUSION: This is the largest case series of RSS clarifying their clinicoelectroencephalographic characteristics reported to date. To avoid unnecessary antiepileptic therapies, clinicians should be aware of RSS and distinguish it from other disorders involving involuntary movements or seizures, especially epileptic spasms.

DOI： 10.1016/j.yebeh.2021.108075

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

The accuracy of brain age estimates from magnetic resonance (MR) images has improved with the advent of deep learning artificial intelligence (AI) models. However, most previous studies on predicting age emphasized aging from childhood to adulthood and old age, and few studies have focused on early brain development in children younger than 2 years of age. Here, we performed brain age estimates based on MR images in children younger than 2 years of age using deep learning. Our AI model, developed with one slice each of raw T1- and T2-weighted images from each subject, estimated brain age with a mean absolute error of 8.2 weeks (1.9 months). The estimates of our AI model were close to those of human specialists. The AI model also estimated the brain age of subjects with a myelination delay as significantly younger than the chronological age. These results indicate that the prediction accuracy of our AI model approached that of human specialists and that our simple method requiring less data and preprocessing facilitates a radiological assessment of brain development, such as monitoring maturational changes in myelination.

DOI： 10.1016/j.mri.2021.03.004

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Brain MRI provides important information about suspected congenital CMV infection in neonatally underdiagnosed children. This study aimed to describe MRI findings in children in whom congenital CMV infection was not suspected during the neonatal period and was proven retrospectively. METHODS: We enrolled 31 children referred to the pediatric neurology clinic with neurological symptoms who were proven to have congenital CMV infection based on dried umbilical cord samples. Upon diagnosis, MR and CT images were assessed using the van der Knaap scoring system integrated with additional variables. Two investigators independently assessed all images. RESULTS: The age at diagnosis was < 12 months in 14, 12-24 months in 11, and > 24 months in 6 patients. The initial symptom triggering clinic referral was delayed development in 22, seizure in 5, deafness in 3, and hemiplegia in 1 patient. Of the 31 children, 30 had a white matter (WM) abnormality predominant in the deep WM of the parietal lobe (n = 25). Anterior temporal lesions were observed in 21 children. Cortical lesions were observed in 7 children, suggestive of polymicrogyria. No child had cerebellar or brainstem abnormalities. Brain CT was performed in 22 of 31 children, and 11 showed punctate cerebral calcification in the periventricular and/or deep WM. CONCLUSION: Patients with congenital CMV infection with delayed neurological symptoms show a relatively uniform pattern of parietal-dominant multifocal WM lesions and anterior temporal lesions, with or without polymicrogyria.

File： Kidokoro2020_Article_MRIFindingsInChildrenWithConge.pdf

DOI： 10.1007/s00234-020-02603-9

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: MICPCH is manifested as microcephaly associated with pontocerebellar hypoplasia and global developmental delay but developmental regression has never been reported. We describe the detailed clinical history of a woman with intellectual disability and microcephaly with pontine and cerebellar hypoplasia (MICPCH) with a CASK mutation who exhibited gross motor regression after adolescence. CASE: The patient experienced severe motor and intellectual developmental delay with microcephaly from infancy. The initial diagnosis was Rett syndrome based on her clinical features, including hand stereotypes and the absence of structural abnormality on magnetic resonance imaging (MRI) performed at the age of 5 years. Although gross motor abilities developed slowly and she could walk independently, she never acquired speech or understanding of languages. After adolescence, her motor ability gradually regressed so that she was unable to stand without support and moved with a wheelchair. At the age of 31 years, because of her atypical clinical course for Rett syndrome, whole exome sequencing was performed, which revealed a de novo heterozygous c.2068 + 1G > A mutation in the CASK gene (NM_001126055). Brain MRI revealed mild pontocerebellar hypoplasia compatible with the clinical phenotype of MICPCH. DISCUSSION: This case suggests that MICPCH with a CASK mutation might cause developmental regression after adolescence and might be regarded as a neurodegenerative disorder.

DOI： 10.1016/j.braindev.2020.11.007

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

Delta brush, a well-known characteristic waveform of the human preterm electroencephalogram, represents spontaneous electrical activity. Recent experimental animal model evidence suggests that delta brushes are not only spontaneous intrinsic activity but are also evoked by external sensory stimulation or spontaneous movement. Additionally, delta brushes are likely to reflect the activity of subplate neurons, which also play an important role in early brain development and network organization. Here, evidence about delta brush in human preterm electroencephalogram is provided along with future perspectives.

File： ped.14420.pdf

DOI： 10.1111/ped.14420

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：American Society of Neuroradiology ({ASNR})  

File： ajnr.A6821.full.pdf

DOI： 10.3174/ajnr.A6821

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

File： ped.14407.pdf

DOI： 10.1111/ped.14407

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

File： 1-s2.0-S1388245720303710-main.pdf

DOI： 10.1016/j.clinph.2020.06.006

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

File： 1-s2.0-S0022347618314288-main.pdf

DOI： 10.1016/j.jpeds.2018.10.016

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BACKGROUND: Post-transplant acute limbic encephalitis (PALE) has been reported, and human herpesvirus 6 (HHV-6) is one of the causes. Reports of PALE in children are still limited. In the present study, the detailed clinico-radiological findings of PALE and the neurological outcomes of four children are reported. CASES: Four patients aged three to twelve years of 142 patients who underwent allogeneic hematopoietic stem cell transplantation developed PALE. Underlying disorders were neuroblastoma in three patients and acute lymphoblastic leukemia in one. All four patients showed disorientation, behavioral changes, emotional dysregulation, anterograde amnesia, or decreased level of consciousness between 12 and 22 days after transplantation. None had clinical seizures. In all patients, MRI showed hyperintense signals in unilateral or bilateral hippocampi, especially in the CA1 area, on diffusion-weighted imaging (DWI), with a decreased apparent diffusion coefficient. Though the hippocampi were also hyperintense on T2-weighted and fluid-attenuated inversion recovery imaging, signal intensity was higher on DWI by visual inspection. Electroencephalograms showed poorly organized posterior dominant rhythm in all patients and left occipito-centro-parietal slow waves in one without epileptiform discharges. HHV-6 DNA was positive in both cerebrospinal fluid and whole blood by polymerase chain reaction in three of the four patients. Two patients had pharmaco-resistant epilepsy and cognitive impairment three years after PALE, but the other two recovered neurologically from PALE. CONCLUSION: A DWI abnormality of the hippocampal CA1 area is a good biomarker for the diagnosis of HHV-6-positive or -negative PALE in children. Glutamatergic dysregulation and hippocampal cytotoxic edema may be involved in PALE.

DOI： 10.1016/j.braindev.2026.104514

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s00246-026-04191-6

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

DOI： 10.11251/ojjscn.58.13

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：金原出版  

DOI： 10.18888/sh.0000003745

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

Infantile epileptic spasm syndrome (IESS) is developmental epileptic encephalopathy occurring in childhood. It is often difficult to predict long-term seizure outcomes at the time of onset, particularly in patients with unknown etiology. Predicting long-term seizure outcomes from electroencephalograms (EEG) at onset may be helpful for decision-making on therapeutic strategy by clinicians and families. This study included twenty-two patients with IESS of unknown etiology with a median follow-up period of 11 years (range 5-14 years). We categorized patients diagnosed with IESS of unknown etiology into good and poor outcome groups according to the presence or absence of seizures at the time of the last follow-up. Fifteen patients were categorized into the good outcome group, and the rest into the poor outcome group. A machine learning (ML) model was developed using scale EEG data during sleep to identify patients in the poor outcome group. A self-attention autoencoder (SA-AE), which is an anomaly detection method for time-series data, was adopted because the numbers of patients in the poor and good outcome groups were unbalanced. The ML model achieved a sensitivity of $1.00\pm 0.05$, specificity of $0.88\pm 0.11$, and accuracy of $0.95\pm 0.06$. In addition, the AUROC was $0.91\pm 0.09$. Thus, the SA-AE model under development enabled highly accurate prediction of long-term prognosis in patients with IESS of unknown etiology. We confirmed that trained attention among the EEG channels could be explained from the viewpoint of pathophysiology of IESS. This study contributes to the determination of appropriate treatment for patients with IESS of unknown etiology.

DOI： 10.1109/JBHI.2025.3647091

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.braindev.2025.104479

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Language：English   Publishing type：Research paper (scientific journal)  

UNLABELLED: This study aimed to clarify the characteristics of motor impairments and their relationship with perinatal risk factors in children born extremely preterm (EP). Forty children aged 6 years born EP (n = 10) and at term (n = 30) were enrolled. Patients with cerebral palsy or intellectual disabilities were excluded from the EP group. Data on the following items were collected: one-leg standing time, two-step test, grip strength, five-times sit-to-stand test, and Developmental Coordination Disorder (DCD) questionnaire. The Gait Deviation Index (GDI), an index of gait quality, and its symmetry ratio were evaluated using three-dimensional gait analysis. The results were compared between the two groups, and their correlations with perinatal risk factors in the EP group were analyzed. The EP group exhibited shorter one-leg standing times, lower two-step test scores, lower grip strength, and lower total and general coordination scores on the DCD Questionnaire compared with the control group. Regarding gait function, the EP group had a lower GDI and a higher GDI symmetry ratio. Birth weight was positively correlated with the GDI. Body weight at 36 weeks postmenstrual age was positively correlated with the GDI and DCD Questionnaire total and general coordination scores. CONCLUSION: Children born EP exhibited potentially decreased gait quality and increased gait quality asymmetry, in addition to decreased muscle strength, decreased balance function, and increased DCD traits. Lower birth weight and the subsequent weight gain may be related to decreased gait quality and increased DCD trait in school-aged children. WHAT IS KNOWN: • Developmental coordination disorders occur in approximately 20-50% of children born extremely preterm (EP). • Decreased muscle strength has been reported in children and adults born EP. WHAT IS NEW: • Children born EP showed decreased gait quality and increased gait asymmetry using three-dimensional gait analysis in addition to decreased muscle strength, decreased balance function, and increased DCD traits. • Lower birth weight and subsequent weight gain were associated with decreased gait quality and increased DCD traits in school-aged children born EP.

DOI： 10.1007/s00431-025-06550-6

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.pedneo.2025.05.014

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児感染症学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Valproic acid (VPA)-induced Fanconi's syndrome has been reported, especially in patients with severe motor and intellectual disabilities, with carnitine deficiency identified as a possible factor. Latent renal tubular dysfunction (RTD) has also been noted in patients with epilepsy treated with VPA. We aimed to evaluate latent RTD in patients treated with and without VPA by examining urine RTD markers and their relationships with clinical variables. METHODS: Urine N-acetyl-β-D-glucosaminidase/creatinine (NAG/Cr) and β2-microglobulin/creatinine (BMG/Cr) were evaluated in 147 patients with childhood-onset epilepsy. NAG/Cr and BMG/Cr were compared between 90 patients treated with VPA (VPA group) and 57 patients treated with other anti-seizure medications (non-VPA group). Single correlations and multiple regression analyses were conducted between RTD markers and clinical variables in the VPA group. RESULTS: NAG/Cr was significantly higher in the VPA group than in the non-VPA group, whereas BMG/Cr was not significantly different. High or borderline NAG/Cr (>4.0 IU/g*Cr) was seen in 85 (94 %) patients on VPA, and high NAG/Cr (≥10.0 IU/g*Cr) was seen in 21 (23 %) on VPA. In the VPA group, NAG/Cr was significantly correlated with free carnitine (FC), VPA dose adjusted for body weight, and duration of treatment with VPA. BMG/Cr was significantly correlated with the number of antiseizure medications. Multiple regression analysis showed that NAG/Cr was significantly correlated with FC. CONCLUSIONS: Latent RTD was found in many patients on VPA, and elevated RTD markers were correlated with carnitine deficiency. Physicians should be aware of latent RTD in patients with childhood-onset epilepsy treated with VPA.

DOI： 10.1016/j.eplepsyres.2025.107611

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：診断と治療社  

DOI： 10.34433/pp.0000001746

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

BACKGROUND: Rett syndrome is a genetic neurodevelopmental disorder that predominantly affects girls. While microcephaly is a common feature, there is limited information on the detailed structural changes in the brain. This study aimed to identify regional brain volume abnormalities and explore the correlation between brain volume and clinical characteristics. METHODS: We compared the regional brain volumes of 20 female children with Rett syndrome to those of 25 healthy female children. Additionally, we assessed the correlation between regional brain volume, Clinical Severity Scores, and epilepsy status. RESULTS: Significantly smaller volumes were observed in all brain regions, including the cerebral cortex, cerebral white matter, subcortical gray matter, cerebellum, and brainstem. Within the cortical regions, volume reduction was prominent in the left precentral, right lateral occipital, left precuneus, left inferior parietal, and right medial orbitofrontal cortices. After correcting for intracranial volumes, volume reduction was more prominent in the cerebral cortices than in the cerebral white matter. Small volumes were consistently observed, regardless of age. Negative correlations were observed between the volumes of multiple regions and the Clinical Severity Scores. There were no correlations among regional brain volume, seizure control, or duration of epilepsy. CONCLUSION: The mechanism underlying the cortical-dominant volume reduction remains unclear; however, it may be caused by altered synapse development associated with methyl-CpG-binding protein 2 gene abnormalities. Characteristic impairments in visual recognition and deterioration of motor function in Rett syndrome may be associated with significant volume reduction in specific cortical regions, such as the lateral occipital cortex, precuneus, and precentral gyrus.

DOI： 10.1016/j.braindev.2025.104348

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1038/s41598-025-03395-9

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Other Link： https://www.nature.com/articles/s41598-025-03395-9

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

DOI： 10.11251/ojjscn.57.175

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s00247-025-06194-6

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Other Link： https://link.springer.com/article/10.1007/s00247-025-06194-6/fulltext.html

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児保健協会  

Language：English   Publishing type：Research paper (scientific journal)  

Gait disturbance is a common motor symptom in Angelman syndrome (AS), but its characteristics have been poorly studied quantitatively. This study aimed to analyze gait characteristics in school-age children with AS using three-dimensional gait analysis (3DGA). Patients with clinically and genetically confirmed AS and healthy children aged 6-15 years were included. For gait assessments, 3DGA was performed using an eight-camera motion analysis system and eight force plates. Gait metrics, including gait speed, step length, step width, gait variability, gait deviation index, and kinematic and kinetic data of lower extremity joints were compared between the groups. Eight children with AS and 24 healthy controls were evaluated. Seven children with AS had flat feet. While step length and gait speed were similar between groups, children with AS showed greater variability in these parameters and larger step widths. Their average gait deviation index was 74.5, indicating significant gait disturbance, and characteristic features included anterior pelvic tilt, insufficient hip extension, excessive knee flexion during early stance, and reduced ankle joint power. School-age children with AS exhibit unstable, prancing gait characterized by knee flexion in the early stance phase, quantifiable using 3DGA. These findings provide foundation for evaluating therapeutic interventions.

DOI： 10.1002/ajmg.a.63983

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

Abstract

This study aims to evaluate the impact of early steroid discontinuation on total dosage and outcomes in pediatric immunoglobulin A (IgA) vasculitis patients with uncontrolled abdominal pain. This retrospective cohort study included children younger than 16 years with newly diagnosed IgA vasculitis hospitalized for abdominal pain who received their first dose of steroids between April 1, 2013, and March 31, 2019, at 14 hospitals. Patients were divided into two groups: the standard (STD) group, which received steroid therapy for at least 8 consecutive days, and the early discontinuation attempt (EDA) group, which attempted discontinuation within 7 days. EDA was further divided into two subgroups: the early discontinuation (ED) group, which completed steroid treatment within a week, and the readministration (RA) group, which required readministration. Total steroid dosage, duration of therapy, hospital stay, and complications were compared. A total of 272 patients were analyzed: STD (n = 190) and EDA (n = 82). There were no significant differences in baseline characteristics. EDA had a shorter hospital stay (8.5 vs. 15.0 days, p &lt; 0.01), fewer total steroid days (6 vs. 17.5 days, p &lt; 0.01), and lower total steroid dosage (5.4 mg/kg vs. 15.4 mg/kg, p &lt; 0.01) compared to STD, with no significant differences in complications. Among EDA patients, 22 (27%) required steroid readministration due to symptom recurrence; however, symptoms resolved in all RA patients, with lower total steroid dosage and duration compared to STD, without prolonging hospital stay.

Conclusion: Discontinuing steroids within 7 days for abdominal pain in children with IgA vasculitis reduces total steroid dosage without increasing complications, even with occasional readministration.

Clinical trial registration: Approval no. 2019–0394.

<table> <tbody> <tr> <td align="left"> What is known:• Steroids have been reported to be effective for abdominal pain in pediatric IgA vasculitis..• Steroids should be tapered gradually to reduce the risk of symptom flare-up in pediatric IgA vasculitis..</td> </tr> <tr> <td align="left"> What is new:• Early discontinuation of steroids reduced total dosage and hospital stay without increasing complications in pediatric IgA vasculitis..</td> </tr> </tbody> </table>

DOI： 10.1007/s00431-025-06107-7

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Other Link： https://link.springer.com/article/10.1007/s00431-025-06107-7/fulltext.html

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: In Japan, selumetinib is used in pediatric patients with neurofibromatosis type 1 (NF1) and symptomatic inoperable plexiform neurofibroma (PN). However, there have been no real-world reports on Japanese patients. In this study, we reported a single-center, short-term experience with selumetinib after its approval in Japan. METHODS: We prospectively collected data from 11 pediatric NF1 patients with symptomatic, inoperable PN who were initiated on selumetinib between November 2022 and May 2023; the selumetinib was administered by the same physician. Various patient factors, tumors, dose and efficacy of selumetinib, and adverse events (AE) were investigated. RESULTS: Of 11 patients included, 7 were male, with a mean age of 14 years. The sites of symptomatic main PN included the head and neck, pelvis to lower extremities, and paraspinal lesions in five, three, and three patients, respectively. The median maximum diameter of the main PN was 91 mm, and the median follow-up duration was 19 months. All patients with pain or motor dysfunction experienced symptom improvement after treatment, and the tumors tended to shrink in 7 of the 11 patients (64%). Among the six patients with disfigurements, only one experienced improvement. Of 59 AEs observed, 58 (98%) were grades 1 and 2, and 5 patients (46%) underwent temporary selumetinib withdrawal due to AEs. One patient discontinued the drug (9%) because of rash dermatitis. CONCLUSIONS: Despite the relatively short-term results, no serious AEs were observed, and many patients benefited from selumetinib treatment. In some patients, administration was discontinued or interrupted because of the balance between benefits and AEs, and further data are needed to better understand the general safety and efficacy of selumetinib.

DOI： 10.1093/jjco/hyae184

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

DOI： 10.11251/ojjscn.57.12

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：東京医学社  

DOI： 10.24479/peri.0000001915

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

Congenital cytomegalovirus (cCMV) infection is the most common congenital infection in developed countries. Although a standard therapy has not yet been established, evidence for the management of cCMV infection has been accumulating. The first edition of the "Clinical Practice Guidelines for the Management of Congenital Cytomegalovirus Infection" was published in Japan in 2023. This summary outlines the clinical questions (CQs) in the guidelines, with reference to the Japanese Medical Information Distribution Service Manual. Overall, 20 CQs with statements regarding prenatal risk assessment, prevention and management at diagnosis (CQs 1-1-1-3), diagnosis (CQs 2-1-2-6), treatment (CQs 3-1-3-7) and follow-up requirements (CQs 4-1-4-4) have been discussed. For each statement, the levels of recommendation, evidence and consensus rates were determined. These guidelines will assist in the management of patients with cCMV infection.

DOI： 10.1097/INF.0000000000004489

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Quantitative EEG is frequently used to monitor children affected by acute encephalopathy (AE), with the expectation of providing comprehensive insights into continuous EEG monitoring. However, the potential of quantitative EEG for estimating outcomes in this context remains unclear. We sought reliable prognostic markers within the color density spectral array (CDSA) of the continuous EEG for AE-affected children undergoing therapeutic hypothermia (TH). METHODS: This retrospective study analyzed CDSA data from eight scalp electrodes of 15 AE-affected children undergoing TH. Two CDSA features were investigated-high-frequency lines (HFLs) and periodic elevation in the low frequency band (PLFB)-along with the corresponding EEG characteristics. The inter-rater reliability for CDSA was assessed by four pediatric neurologists. Outcomes were grouped into either no/mild or severe decline in motor and cognitive functions, then compared with CDSA features. RESULTS: The median EEG recording time was 114 (81-151) h per child. While at least 41 % of HFLs corresponded to typical sleep spindles, 94 % of PLFB aligned with cyclic changes in the amplitude of delta/theta waves on the raw EEG. Inter-rater reliability was higher for HFLs than for PLFB (kappa values: 0.69 vs. 0.46). HFLs were significantly more prevalent in children with no/mild decline than in children with severe decline (p = 0.017), whereas PLFB did not differ significantly (p = 0.33). CONCLUSIONS: This study provides preliminary evidence that reduced HFLs on CDSA predict unfavorable outcomes in AE-affected children undergoing TH. This suggests that maintaining high-frequency waves is critical for optimal brain function.

DOI： 10.1016/j.braindev.2024.08.002

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Antenatal corticosteroids (ACS) is a well-established treatment for women at risk of preterm birth that improves neonatal outcomes. However, several concerns have been raised regarding the potential long-term adverse effects of ACS on the offspring's developing brain. Here we investigated the association between ACS and subcortical segmental volumes in preterm infants at term-equivalent age. STUDY DESIGN: This retrospective observational study was conducted using the clinical data of preterm singleton infants born between 220/7 and 336/7 gestational weeks at Nagoya University Hospital in 2014-2020. Subcortical volumes of the bilateral thalami, caudate nuclei, putamens, pallidums, hippocampi, amygdalae, and nuclei accumbens were evaluated using an automated segmentation tool, Infant FreeSurfer, and compared between neonates exposed to a single course of ACS (n = 46) and those who were not (n = 13) by multiple linear regression analysis (covariates: postmenstrual age at magnetic resonance imaging, infant sex, and gestational age at birth). We compared each subcortical volume stratified by gestational age at birth (<28 vs. ≥28 gestational weeks). RESULTS: Multivariate analyses revealed significantly smaller volumes in the bilateral amygdalae (left, p < 0.03; right, p < 0.03) and caudate nuclei (left, p < 0.03; right, p = 0.04) in neonates with ACS. Significantly smaller volumes in these regions were observed only in neonates born at 28 weeks of gestation or later. CONCLUSIONS: ACS was associated with smaller volumes of the bilateral amygdalae and caudate nuclei at term-equivalent age. This association was observed exclusively in infants born at 28 weeks of gestation or later.

DOI： 10.1016/j.ejogrb.2024.08.034

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本臨床神経生理学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) develops along with status epilepticus and widespread subcortical white matter edema. We aimed to evaluate the epileptic foci and networks in two patients with epilepsy after AESD using simultaneous electroencephalography and functional magnetic resonance imaging (EEG-fMRI). METHODS: Statistically significant blood oxygen level-dependent (BOLD) responses related to interictal epileptiform discharges (IEDs) were analyzed using an event-related design of hemodynamic response functions with multiple peaks. RESULTS: Patient 1 developed focal seizures at age 10 years, one year after AESD onset. Positive BOLD changes were observed in the bilateral frontotemporal lobes, left parietal lobe, and left insula. BOLD changes were also observed in the subcortical structures. Patient 2 developed epileptic spasms at age two years, one month after AESD onset. Following total corpus callosotomy (CC) at age three years, the epileptic spasms resolved, and neurodevelopmental improvement was observed. Before CC, positive BOLD changes were observed bilaterally in the frontotemporal lobes. BOLD changes were also observed in the subcortical structures. After CC, the positive BOLD changes were localized in the temporal lobe ipsilateral to the IEDs, and the negative BOLD changes were mainly in the cortex and subcortical structures of the hemisphere ipsilateral to IEDs. CONCLUSION: EEG-fMRI revealed multiple epileptic foci and extensive epileptic networks, including subcortical structures in two cases with post-AESD epilepsy. CC may be effective in disconnecting the bilaterally synchronous epileptic networks of epileptic spasms after AESD, and pre-and post-operative changes in EEG-fMRI may reflect improvements in epileptic symptoms.

DOI： 10.1016/j.braindev.2024.07.003

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: This cross-sectional study compared body composition and motor function between children who were born large for gestational age (LGA) and those born appropriate for gestational age (AGA) and to investigate the association between gait quality and other variables. METHODS: Body composition was determined using a bioelectrical impedance analyzer. Motor functions were assessed using one-leg standing time, timed up-and-go test, five times sit-to-stand test, and three-dimensional gait analysis. We compared the results between two groups. We performed multiple regression analysis to evaluate the association between gait deviation index and variables of LGA, fat mass index, and motor functions (adjusted for age and sex). RESULTS: Children aged 6-12 years who were born LGA at term (n = 23) and those who were born AGA at term (n = 147) were enrolled. The LGA group had a higher fat mass index (2.9 vs. 2.2, p = 0.006) and lower gait deviation index (91.4 vs. 95.4, p = 0.011) than the AGA group. On multiple regression analysis, gait deviation index was associated with being LGA and fat mass index. CONCLUSIONS: In school-aged children who were born LGA, monitoring increased fat mass index and decreased gait deviation index could lessen the risk of metabolic syndrome and reduced gait function. IMPACT: Children aged 6-12 years who were born large for gestational age (LGA) at term showed a higher fat mass index and lower gait deviation index than those who were born appropriate for gestational age at term. No significant differences in balance function or muscle strength were observed between groups. On multiple regression analysis, gait deviation index was associated with being LGA at birth and fat mass index. In school-aged children who were born LGA, monitoring increased fat mass index and decreased gait deviation index could lessen the risk of metabolic syndrome and reduced gait function.

DOI： 10.1038/s41390-024-03211-6

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.earlhumdev.2024.106098

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Various factors contribute to the development of infection-related acute encephalopathy (AE) in children, such as infectious agents and chronic underlying disorders. We studied underlying disorders in children with AE to identify predisposing factors of AE. METHODS: We investigated underlying disorders or past histories in patients with two types of AE from the database in the Tokai area of Japan between 2009 and 2022: 204 patients with AE with reduced subcortical diffusion (AED) and 137 with clinically mild encephalopathy with a reversible splenial lesion (MERS). We compared them with 89 patients with acute disseminated encephalomyelitis (ADEM) to clarify the specific disorders in the two AE types. RESULTS: The prevalence of underlying disorders in AED (34%, 70 patients) was significantly higher than that in ADEM (12%, 11 patients) (P < 0.01). The prevalence of underlying disorders in MERS was 23% (32 patients). The underlying disorders included seizure disorders, premature birth, genetic/congenital disorders, and endocrine/renal diseases. In patients with seizure disorders in AED, five patients (18%) had Dravet syndrome and four (15%) had West syndrome, whereas none with MERS had these syndromes. Twenty-five (12%) of 204 patients with AED, three (2%) with MERS, and one (1%) with ADEM were preterm or low birth weight. CONCLUSIONS: The high prevalence of seizure disorders suggests that seizure susceptibility is an important predisposing factor in AED. Premature birth also has an impact on the development of AED. Caution is required regarding the development of AE in patients with chronic seizure disorders or premature birth.

DOI： 10.1016/j.pediatrneurol.2024.03.017

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Individuals with Dravet syndrome (DS) exhibit progressive gait disturbance. No quantitative studies have been conducted to evaluate the effectiveness of medication for gait disturbance. Therefore, the aim of this study was to evaluate the effectiveness of levodopa for pathological gait in people with DS using three-dimensional gait analysis (3DGA). METHODS: Nine individuals with DS, ages 6-20 years, participated in a crossover study of levodopa and were randomly assigned to the levodopa precedence or no levodopa precedence group. Levodopa/carbidopa hydrate was prescribed at a dose of 5 mg/kg/day (body weight <60 kg) or 300 mg/day (body weight ≥60 kg). The medication was taken for 4-6 weeks (4-week washout period). 3DGA was performed three times before the study, with and without levodopa. A mixed-effects model was used to evaluate the effectiveness of levodopa. The primary outcome was the change in the Gait Deviation Index (GDI). In addition, spatiotemporal gait parameters, 6-minute walking distance (6MD), and balance were evaluated. The correlation between the effectiveness of levodopa and age or gait performance before starting levodopa was analyzed. RESULTS: Levodopa improved the GDI by 4.2 points, (p = .029), 6MD by 52 m (p = .002), and balance test result by 4.1 mm (p = .011) in participants with DS. No severe adverse events were observed, with the exception of one participant, who exhibited fever and consequently stopped taking levodopa. Levodopa was more effective in younger participants with a higher baseline gait performance. SIGNIFICANCE: Our randomized crossover trial showed that levodopa has the potential to improve gait disturbance in people with DS.

DOI： 10.1111/epi.17888

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: GTP-cyclohydrolase 1-deficient dopa-responsive dystonia (GTPCH1-deficient DRD) typically presents in childhood with dystonic posture of the lower extremities, gait impairment, and a significant response to levodopa. We performed three-dimensional gait analysis (3DGA) to quantitatively assess the gait characteristics and changes associated with levodopa treatment in patients with GTPCH1-deficient DRD. METHODS: Three levodopa-treated patients with GTPCH1-deficient DRD underwent 3DGA twice, longitudinally. Changes were evaluated for cadence; gait speed; step length; gait deviation index; kinematic data of the pelvis, hip, knee, and ankle joints; and foot progression angle. RESULTS: Levodopa treatment increased the cadence and gait speed in one of three patients and increased the gait deviation index in two of three patients. The kinematic data for each joint exhibited different characteristics, with some improvement observed in each of the three patients. There was consistent marked improvement in the abnormal foot progression angle; one patient had excessive external rotation of one foot, another had excessive bilateral internal rotation, and the other had excessive internal rotation of one foot and excessive external rotation of the opposite foot, all of which improved. CONCLUSION: The 3DGA findings demonstrate that the gait pathology and recovery process in GTPCH1-deficient DRD vary from case to case. Changes in the foot progression angle and gait deviation index can enable the effects of treatment to be more easily evaluated.

DOI： 10.1016/j.pediatrneurol.2024.03.006

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Infants born to mothers with chorioamnionitis (CAM) are at increased risk of developing adverse neurodevelopmental disorders in later life. However, clinical magnetic resonance imaging (MRI) studies examining brain injuries and neuroanatomical alterations attributed to CAM have yielded inconsistent results. We aimed to determine whether exposure to histological CAM in utero leads to brain injuries and alterations in the neuroanatomy of preterm infants using 3.0- Tesla MRI at term-equivalent age. METHODS: A total of 58 preterm infants born before 34 weeks of gestation at Nagoya University Hospital between 2010 and 2018 were eligible for this study (CAM group, n = 21; non-CAM group, n = 37). Brain injuries and abnormalities were assessed using the Kidokoro Global Brain Abnormality Scoring system. Gray matter, white matter, and subcortical gray matter (thalamus, caudate nucleus, putamen, pallidum, hippocampus, amygdala, and nucleus accumbens) volumes were evaluated using segmentation tools (SPM12 and Infant FreeSurfer). RESULTS: The Kidokoro scores for each category and severity in the CAM group were comparable to those observed in the non-CAM group. White matter volume was significantly smaller in the CAM group after adjusting for covariates (postmenstrual age at MRI, infant sex, and gestational age) (p = 0.007), whereas gray matter volume was not significantly different. Multiple linear regression analyses revealed significantly smaller volumes in the bilateral pallidums (right, p = 0.045; left, p = 0.038) and nucleus accumbens (right, p = 0.030; left, p = 0.004) after adjusting for covariates. CONCLUSIONS: Preterm infants born to mothers with histological CAM showed smaller volumes in white matter, pallidum, and nucleus accumbens at term-equivalent age.

DOI： 10.1007/s00404-023-07064-y

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：日本レックリングハウゼン病学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)  

Delta brushes are spontaneous neural activities observed in preterm electroencephalograms (EEGs) and are thought to reflect the activities of subplate neurons in the developing brain. We investigated the haemodynamic responses associated with delta brushes in five preterm infants at two time points (at 33 or 34, and 36 weeks of postmenstrual age), using simultaneous EEG-functional near-infrared spectroscopy (NIRS). An automated detection algorithm was developed to identify the brush components of delta brushes in the bipolar EEG envelope; we placed eight EEG electrodes. An eight-channel NIRS device was placed around the head of each infant to measure changes in oxy- and deoxy-haemoglobin (Hb) concentrations. Haemodynamic grand averages were calculated for local brushes in each NIRS channel. We classified the responses into five patterns based on changes in oxy- and deoxy-Hb signals (positive in-phase/anti-phase, negative in-phase/anti-phase, and unclassified) and evaluated the relationship between the locations of NIRS measurements and those of brushes, as well as the haemodynamic response patterns and infant age at the time of recording. In all the 10 recordings, we found that positive responses (oxy-Hb increases) predominated, not only in the corresponding areas but also in remote areas. Particularly, notable responses were observed in the bilateral temporal areas. Among the positive responses, the proportion exhibiting an anti-phase pattern was 12% at 33 to 34 weeks of postmenstrual age and 54% at 36 weeks of postmenstrual age. Our unexpected finding of remarkable temporal responses to localised neuronal activity supports the hypothesis that the insula is the most strongly interconnected hub in the developing brain.

DOI： 10.1007/978-3-031-67458-7_16

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：公益社団法人 日本生体医工学会  

<p>【目的】乳児てんかん性スパズム症候群(IESS)は乳幼児の発達性てんかん性脳症である。IESSの長期てんかん発作予後を予測することは，特に原因不明のIESS患者において困難である。本研究は、IESSの特徴的な脳波であるhypsarrhythmiaから機械学習を用いて原因不明のIESSの長期発作予後を予測することを目的とした。【方法】当院で原因不明のIESSと診断、治療され5年以上追跡された18例を対象とした。フォローアップの中でてんかん治療が不要となっていた13例を予後良好群、治療の継続が必要とされていた5例を予後不良群とした。予後良好群の一部のみを学習用データとし、異常検知をMulti-heads Self-attention autoencoderを用いて行った。Hypsarrhythmiaの主な構成要素であるδ帯域およびβ帯域を抽出し、ヒルベルト変換を用いて各帯域の位相を算出し特徴量とした。学習時に最小化する誤差として再構成誤差を採用した。ランダムにデータセットを交換して10回繰り返した。【結果】予後不良群の同定において、モデルはそれぞれ感度1.00±0.00、特異度0.94±0.09、精度0.97±0.05、ROC曲線下面積は0.96±0.05であった。【結論】Self-attention autoencoderは、原因不明のIESSの発作予後予測を高い精度で可能にした。</p>

DOI： 10.11239/jsmbe.annual62.207_2

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

DOI： 10.11251/ojjscn.56.9

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：東京医学社  

DOI： 10.24479/peri.0000001304

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is a leading cause of nonhereditary neurological complications. When considering antiviral treatment, it is important to differentiate between symptomatic and asymptomatic patients. This study aimed to identify candidate plasma biomarkers for neurological complications of cCMV infection using proteomic analysis. METHODS: This study retrospectively enrolled five patients with symptomatic cCMV infection, four with asymptomatic cCMV infection with isolated sensorineural hearing loss (SNHL), and five with asymptomatic cCMV infection. The plasma samples were collected during neonatal period. The peptides were analyzed using liquid chromatography-mass spectrometry. The concentrations of differentially expressed proteins were validated using an enzyme-linked immunosorbent assay. RESULTS: A total of 456 proteins were identified and quantified. The levels of 80 proteins were significantly different between patients with and without cCMV-related symptoms including isolated SNHL. The levels of 31 proteins were significantly different between patients with and without neuroimaging abnormalities. The plasma concentrations of Fms-related receptor tyrosine kinase 4 in patients with cCMV-related symptoms were significantly higher than those in patients with asymptomatic cCMV infection. Moreover, plasma peptidylprolyl isomerase A levels were significantly higher in patients with neuroimaging abnormalities than in those without. CONCLUSIONS: Proteomic analysis of patients with cCMV infection showed that Fms-related receptor tyrosine kinase 4 and peptidylprolyl isomerase A could be novel diagnostic biomarkers for neurological complications of cCMV infection.

DOI： 10.1093/jpids/piad074

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本てんかん学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an autoimmune demyelinating disorder that often manifests after infections or vaccinations. We report two patients who developed MOGAD out of eight patients with juvenile myelomonocytic leukemia (JMML) that has never been reported. METHODS: We investigated two patients with JMML who developed MOGAD among 127 patients with leukemia from 2012 to 2021. RESULTS: Patient 1 was treated for JMML and developed fever and impaired consciousness at two years and one month of age. Magnetic resonance imaging revealed high-intensity lesions in the left frontal and left occipital white matter. The serum anti-MOG antibody test was positive, while the test was negative in the stored serum 45 days before the onset of encephalopathy. He had relapse of MOGAD after steroid therapy and plasmapheresis. Patient 2, who was treated for JMML, became apathetic and mute at three years and seven months of age. Magnetic resonance imaging revealed left frontoparietal subcortical high-intensity lesions. Anti-MOG antibody at the onset of encephalopathy was positive, while it was negative in stored serum 57 days before and 47 days after the onset. CONCLUSION: We treated two patients who developed MOGAD out of eight patients with JMML and none with MOGAD out of 119 patients with acute lymphocytic leukemia, acute myelocytic leukemia, or chronic myelocytic leukemia. The activated autoimmune process via the RAS pathway abnormality may have led to the formation of the anti-MOG antibody and the onset of MOGAD. MOGAD can occur in children with JMML, and abnormalities of the RAS pathway possibly contribute to its onset.

DOI： 10.1016/j.pediatrneurol.2023.03.002

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本整形外科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本周産期・新生児医学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本整形外科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：金原出版(株)  

Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J00639&link_issn=&doc_id=20230626020020&doc_link_id=10.18888%2Fsh.0000002610&url=https%3A%2F%2Fdoi.org%2F10.18888%2Fsh.0000002610&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: This cross-sectional observational study aimed to assess gait performance, its correlation with physical functions, and its dual-task costs in children with Down syndrome (DS), to investigate their gait adaptations. METHODS: Gait performance with or without movie-watching tasks was evaluated in 17 children with DS (age, 6-12 years) and 51 age- and sex-matched controls, using three-dimensional gait analysis. We compared participants' demographics, physical functions, and gait performance without tasks between the two groups. In the DS group, correlations between physical functions, the intelligence quotient, and gait variables were assessed. Dual-task costs for gait variables were also compared between the two groups. RESULTS: Children with DS showed poorer balance function and muscle strength and lower gait quality than the control group. In the DS group, there was a significant positive correlation between gait speed, step length, and intelligence quotient. There were no correlations between the balance function, muscle strength, intelligence quotient, and gait quality. Dual-task costs for gait speed, step length, and cadence were greater in the DS group; however, there was no significant difference in dual-task costs for gait quality between the two groups. CONCLUSION: These findings highlight the importance of providing appropriate interventions for motor functions in school-aged children with DS based on their gait performance in single- and dual-task conditions, as well as on their intelligence quotient.

DOI： 10.1016/j.braindev.2022.11.001

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: Ataxic-rigid gait is a characteristic gait pathology in patients with Rett syndrome (RTT). In the present study, we aimed to quantitatively evaluate gait pathology in patients with RTT using three-dimensional gait analysis (3DGA). METHODS: We performed 3DGA in 11 patients with RTT ranging from 5 to 18 years (median age, 9 years) and in 33 age-matched healthy female controls. We compared the results of 3DGA, including spatiotemporal gait parameters and comprehensive indices of gait kinematics, such as the Gait Deviation Index (GDI) and Gait Profile Score (GPS), between the two groups. The GPS consists of nine sub-indices called Gait Variable Scores (GVSs). Decline in GDI or elevation of GPS and GVS indicated greater abnormal gait pathology. RESULTS: The patients demonstrated significantly slower walking speed, lower step length/length of the lower extremities, lower cadence, wider step width, and higher coefficient of variation of step length than the controls. Moreover, the patients had a lower GDI and higher GPS than the controls. The patients also exhibited higher GVSs for eight out of nine gait kinematics, particularly the sagittal plane in the pelvis, hip, knee, and ankle joint; coronal plane in the pelvis and hip joint; and horizontal plane in the pelvis than the controls. CONCLUSIONS: Quantitative evaluation of gait pathology in patients with RTT is possible using 3DGA. We found that in addition to ataxic-rigid gait, abnormalities in the coronal plane of the pelvis and hip joint and the horizontal plane of the pelvis were prominent.

DOI： 10.1016/j.ejpn.2022.11.010

Open Access

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

DOI： 10.11251/ojjscn.55.64

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.3233/NPM-239001

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.3233/NPM-239005

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.isci.2022.105629

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Oxford University Press (OUP)  

Abstract

Background

Mitigation measures implemented during the coronavirus disease 2019 (COVID-19) pandemic remarkably reduced the incidence of infectious diseases among children. However, a re-emergence of respiratory syncytial virus (RSV) infection was observed in 2021 in Japan. We compared the clinical characteristics of hospitalized patients with RSV infection before and during COVID-19.

Methods

We retrospectively enrolled children aged &amp;lt;6 years who were hospitalized with RSV infection in 18 hospitals and compared their clinical characteristics before (January 2019 to April 2020, 1675 patients) and during COVID-19 (September 2020 to December 2021, 1297 patients).

Results

The mean age of patients with RSV infection was significantly higher during COVID-19 than before (17.4 vs 13.7 months, P &amp;lt; .001). Compared with before COVID-19, a 2.6-fold increase in RSV cases in the 2–5 years age group was observed from sentinel surveillance during COVID-19, whereas a 1.2-fold increase was noted in the same age group among hospitalized patients. On average for all patients, consolidation shadows obtained on radiography were less frequently observed (26.1 vs 29.6%, P = .04), and reduced respiratory assistance (42.2% vs 48.7%, P &amp;lt; .001) and hospitalization stay (5.7 vs 6.0 days, P &amp;lt; .001) was required in patients with RSV infection during COVID-19.

Conclusions

Coronavirus disease 2019 and social activity restriction caused epidemiological changes in pediatric RSV infections, and a majority of patients with RSV infection aged ≥2 years did not develop severe symptoms requiring hospitalization. The RSV symptoms during the COVID-19 outbreak were equivalent to or milder than in the previous seasons.

DOI： 10.1093/ofid/ofac562

Open Access

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PubMed

Other Link： https://academic.oup.com/ofid/article-pdf/9/11/ofac562/47043659/ofac562.pdf

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児感染症学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：東京医学社  

DOI： 10.24479/peri.0000000379

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND AND PURPOSE: West syndrome is a developmental and epileptic encephalopathy characterized by epileptic spasms, neurodevelopmental regression, and a specific EEG pattern called hypsarrhythmia. Our aim was to investigate the brain activities related to hypsarrhythmia at onset and focal epileptiform discharges in the remote period in children with West syndrome using simultaneous electroencephalography and fMRI recordings. MATERIALS AND METHODS: Fourteen children with West syndrome underwent simultaneous electroencephalography and fMRI at the onset of West syndrome. Statistically significant blood oxygen level-dependent responses related to hypsarrhythmia were analyzed using an event-related design of 4 hemodynamic response functions with peaks at 3, 5, 7, and 9 seconds after the onset of each event. Six of 14 children had focal epileptiform discharges after treatment and underwent simultaneous electroencephalography and fMRI from 12 to 25 months of age. RESULTS: At onset, positive blood oxygen level-dependent responses were seen in the brainstem (14/14 patients), thalami (13/14), basal ganglia (13/14), and hippocampi (13/14), in addition to multiple cerebral cortices. Group analysis using hemodynamic response functions with peaks at 3, 5, and 7 seconds showed positive blood oxygen level-dependent responses in the brainstem, thalamus, and hippocampus, while positive blood oxygen level-dependent responses in multiple cerebral cortices were seen using hemodynamic response functions with peaks at 5 and 7 seconds. In the remote period, 3 of 6 children had focal epileptiform discharge-related positive blood oxygen level-dependent responses in the thalamus, hippocampus, and brainstem. CONCLUSIONS: Positive blood oxygen level-dependent responses with hypsarrhythmia appeared in the brainstem, thalamus, and hippocampus on earlier hemodynamic response functions than the cerebral cortices, suggesting the propagation of epileptogenic activities from the deep brain structures to the neocortices. Activation of the hippocampus, thalamus, and brainstem was still seen in half of the patients with focal epileptiform discharges after adrenocorticotropic hormone therapy.

DOI： 10.3174/ajnr.A7646

Open Access

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本臨床神経生理学会  

Language：English   Publishing type：Research paper (scientific journal)  

CaMKIIα plays a fundamental role in learning and memory and is a key determinant of synaptic plasticity. Its kinase activity is regulated by the binding of Ca2+/CaM and by autophosphorylation that operates in an activity-dependent manner. Though many mutations in CAMK2A were linked to a variety of neurological disorders, the multiplicity of its functional substrates renders the systematic molecular phenotyping challenging. In this study, we report a new case of CAMK2A P212L, a recurrent mutation, in a patient with an intellectual disability. To quantify the effect of this mutation, we developed a FRET-based kinase phenotyping strategy and measured aberrance in Ca2+/CaM-dependent activation dynamics in vitro and in synaptically connected neurons. CaMKIIα P212L revealed a significantly facilitated Ca2+/CaM-dependent activation in vitro. Consistently, this mutant showed faster activation and more delayed inactivation in neurons. More prolonged kinase activation was also accompanied by a leftward shift in the CaMKIIα input frequency tuning curve. In keeping with this, molecular phenotyping of other reported CAMK2A de novo mutations linked to intellectual disability revealed aberrant facilitation of Ca2+/CaM-dependent activation of CaMKIIα in most cases. Finally, the pharmacological reversal of CAMK2A P212L phenotype in neurons was demonstrated using an FDA-approved NMDA receptor antagonist memantine, providing a basis for targeted therapeutics in CAMK2A-linked intellectual disability. Taken together, FRET-based kinase mutation phenotyping sheds light on the biological impact of CAMK2A mutations and provides a selective, sensitive, quantitative, and scalable strategy for gaining novel insights into the molecular etiology of intellectual disability.

DOI： 10.3389/fnmol.2022.970031

Open Access

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Recently, whole-exome sequencing (WES) has been used for genetic diagnoses of patients who remain otherwise undiagnosed. WES was performed in 177 Japanese patients with undiagnosed conditions who were referred to the Tokai regional branch of the Initiative on Rare and Undiagnosed Diseases (IRUD) (TOKAI-IRUD). This study included only patients who had not previously received genome-wide testing. Review meetings with specialists in various medical fields were held to evaluate the genetic diagnosis in each case, which was based on the guidelines of the American College of Medical Genetics and Genomics. WES identified diagnostic single-nucleotide variants in 66 patients and copy number variants (CNVs) in 11 patients. Additionally, a patient was diagnosed with Angelman syndrome with a complex clinical phenotype upon detection of a paternally derived uniparental disomy (UPD) [upd(15)pat] wherein the patient carried a homozygous DUOX2 p.E520D variant in the UPD region. Functional analysis confirmed that this DUOX2 variant was a loss-of-function missense substitution and the primary cause of congenital hypothyroidism. A significantly higher proportion of genetic diagnoses was achieved compared to previous reports (44%, 78/177 vs. 24-35%, respectively), probably due to detailed discussions and the higher rate of CNV detection.

DOI： 10.1038/s41598-022-14161-6

Open Access

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本てんかん学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Of the patients with glucose transporter 1 deficiency syndrome (GLUT1-DS), 90% have a pathologic gait. Ataxic-spastic and ataxic gaits are seen in 35% of patients each. A ketogenic diet and modified Atkins diet (MAD) are effective therapy in GLUT1-DS in terms of both the seizures and movement disorder. A three-dimensional gait analysis (3DGA) system can be used to evaluate gait quantitatively using spatiotemporal data and gait kinematics. We performed 3DGA in three ambulatory patients with GLUT1-DS to evaluate the characteristics of their gait pathology, and we compared the gait variables before and after enhancing the MAD in one patient. METHODS: After examination by pediatric neurologists and pediatric orthopedic surgeons, 3DGA was performed. We assessed walking speed, step length, step width, gait variability, Gait Deviation Index (GDI), Gait Profile Score (GPS), and Gait Variable Score (GVS). RESULTS: All three patients had a low GDI and high GPS, comprehensive indices of gait pathology. The unstable gait pattern featured a wide step width in one patient and high gait variability in two patients. In the sagittal plane, the patients had increased GVSs in the knee and ankle joints due to excessive knee flexion or extension and excessive ankle plantarflexion. In the horizontal plane, the patients had increased GVSs in the pelvis, hips, and foot due to excessive rotation during walking. After enhancing the MAD, GDI, GPS, and GVSs improved. CONCLUSIONS: 3DGA has potential for quantifying the characteristics of gait pathology and its improvement with dietary therapy in patients with GLUT1-DS.

DOI： 10.1016/j.pediatrneurol.2022.04.012

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a common type of acute encephalopathy in Japan; the condition is clinically characterized by prolonged seizures as the initial neurological symptom, followed by late seizures 4-6 days later. It is difficult to differentiate AESD from prolonged febrile seizures (PFSs). Here, we explored the use of electroencephalography to differentiate AESD from PFSs. METHODS: We studied the electroencephalograms (EEGs) of children <6 years of age diagnosed with AESD or PFSs; all EEGs were recorded within 48 h of seizure onset (i.e., before the late seizures of AESD). Two pediatric neurologists evaluated all EEGs, focusing on the basic rhythm, slowing during wakefulness/arousal by stimuli, spindles, fast waves, and slowing during sleep. RESULTS: The EEGs of 14 children with AESD and 31 children with PFSs were evaluated. Spindles were more commonly reduced or absent in children with AESD than in those with PFSs (71% vs. 31%, p = 0.021). Fast waves were also more commonly reduced or absent in children with AESD (21% vs. 0%, p = 0.030). The rates of all types of slowing did not differ between children with AESD and those with PFSs, but continuous or frequent slowing during sleep was more common in the former (50% vs. 17%, p = 0.035). CONCLUSIONS: EEG findings may usefully differentiate AESD from PFSs. Reduced or absent spindles/fast waves and continuous or frequent slowing during sleep are suggestive of AESD in children with prolonged seizures associated with fever.

DOI： 10.1016/j.braindev.2021.11.003

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本整形外科学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本整形外科学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: We aimed to investigate electroencephalography (EEG)-functional magnetic resonance imaging (fMRI) findings to elucidate the interictal epileptiform discharge (IED)-related functional alterations in deep brain structures as well as the neocortex in childhood epilepsy with centrotemporal spikes (CECTS). METHODS: Ten children with CECTS (median age; 8.2 years) referred to our hospital within a year of onset were eligible for inclusion. They underwent EEG-fMRI recording during sleep. In addition, longitudinal evaluations, including medical examinations, intelligence tests, and questionnaires about developmental disabilities, were performed. The initial evaluation was performed at the same time as the EEG-fMRI, and the second evaluation was performed over 2 years after the initial evaluation. RESULTS: Three children were unable to maintain sleep during the EEG-fMRI recording, and the remaining seven children were eligible for further assessment. All patients showed unilateral-dominant centrotemporal spikes during scans. One patient had only positive hemodynamic responses, while the others had both positive and negative hemodynamic responses. All patients showed IED-related hemodynamic responses in the bilateral neocortex. For deep brain structures, IED-related hemodynamic responses were observed in the cingulate gyrus (n=4), basal ganglia (n=3), thalamus (n=2), and default mode network (n=1). Seizure frequencies at the second evaluation were infrequent or absent, and the longitudinal results of intelligence tests and questionnaires were within normal ranges. CONCLUSIONS: We demonstrated that IEDs affect broad brain areas, including deep brain structures such as cingulate gyrus, basal ganglia, and thalamus. Deep brain structures may play an important role in the pathophysiology of CECTS.

DOI： 10.1111/ped.15001

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.3233/NPM-229002

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：(株)東京医学社  

DOI： 10.24479/j00648.2022107974

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Academic Press Inc.  

DOI： 10.1016/j.yebeh.2021.108397

Web of Science

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.earlhumdev.2021.105478

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Infants born to mothers with hypertensive disorders of pregnancy (HDP) have adverse neurodevelopmental consequences in later life. Magnetic resonance spectroscopy (MRS) is used to predict subsequent neurodevelopment in the field of perinatology. AIM: We aimed to determine whether exposure to HDP in utero leads to alterations in brain metabolites in preterm infants using multi-voxel proton MRS at term-equivalent age. STUDY DESIGN: Retrospective cohort study. SUBJECTS: A total of 103 preterm infants born before 34 weeks of gestation at Nagoya University Hospital between 2010 and 2018 were eligible. Twenty-seven infants were born to mothers with HDP (HDP group), and 76 were born to mothers without HDP (non-HDP group). OUTCOME MEASURES: The peak area ratios of N-acetylaspartate (NAA)/choline (Cho), NAA/creatine (Cr), and Cho/Cr were evaluated at 10 designated regions of interest (bilateral frontal lobes, basal ganglia, thalami, temporal lobes, and occipital lobes). RESULTS: The peak area ratios of NAA/Cho and NAA/Cr in the bilateral thalami were significantly higher in the HDP group than in the non-HDP group after adjustment for covariates (postmenstrual age at MRS assessment and infant sex). No significant differences were observed in other regions. Preeclampsia, abnormal umbilical artery blood flow, and fetal growth restrictions were significantly associated with increased NAA/Cho and NAA/Cr ratios in the thalami. CONCLUSIONS: Based on the evidence that NAA/Cho and NAA/Cr ratios constantly increase with postmenstrual age in normal brain development, exposure to maternal HDP in utero may accelerate brain maturation and increase neuronal activity in preterm infants.

DOI： 10.1016/j.earlhumdev.2021.105479

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Brush or delta brush is a well-known characteristic waveform in preterm electroencephalograms. However, the longitudinal trajectory of brushes and its association with neurodevelopment remain uncertain. METHODS: We analyzed the longitudinal incidence of brushes in 36 extremely low birth weight infants without severe brain lesions and its association with neurodevelopment and white matter abnormality. Conventional eight-channel electroencephalograms were recorded at 30, 32, 36, and 40 postmenstrual weeks (PMW). Incidence of brushes was calculated as the sum of brushes from each channel separated by active sleep and quiet sleep. A developmental delay was defined as a developmental quotient of <85 assessed at corrected age of 18 months. White matter abnormalities were evaluated with term-equivalent magnetic resonance imaging. RESULTS: The median incidence of brushes (per minute) in 36 infants at PMW 30, 32, 36, and 40 was 16.4, 20.4, 22.5, and 1.8 during active sleep and 7.5, 10.3, 11.5, and 1.7 during quiet sleep, respectively. Among the 36 infants, 14 infants were diagnosed with developmental delay. Longitudinal trajectories of the incidence of brushes were different between the normal and the delayed development groups. Brushes were observed most frequently at 36 PMW in the delayed development group. The incidence of brushes at 36 PMW was significantly correlated with the severity of white matter abnormalities and negatively correlated with the developmental quotient. CONCLUSION: The incidence of brushes at 36 PMW can be a unique predictor of early neurodevelopment in extremely low birth weight infants without severe brain lesions.

DOI： 10.1016/j.braindev.2021.07.003

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児感染症学会  

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Although previous studies have identified asymmetry in gait parameters in children with developmental coordination disorder (DCD), they have not investigated whether these findings coexist with asymmetry in gait quality, as represented by the gait deviation index (GDI). RESEARCH QUESTION: This study sought to measure gait symmetry in children with traits of DCD (DCD trait) to elucidate the characteristic gait pattern. METHODS: This study included 172 (82 girls, 90 boys) participants with and without DCD trait (age: 6-12 years), as assessed using the Japanese version of the Developmental Coordination Disorder Questionnaire (DCDQ-J), which consists of three subscales. Three-dimensional gait analysis data were obtained during the gait trials. GDI, step length, and step time data were recorded for both the right and left legs, and symmetry ratios were calculated. RESULTS: Participants in the DCD trait group (n = 30) had a significantly lower GDI score (p < 0.0001) and a higher GDI symmetry ratio (p = 0.004) than typically developing children. Furthermore, DCD trait was related to the GDI symmetry ratio (odds ratio, 1.2; p = 0.001; 95 % confidence interval, 1.062-1.289). The control during movement, which was a DCDQ-J subscale, was negatively correlated with the GDI symmetry ratio (r=-0.257, p<0.001). SIGNIFICANCE: This study's results lend support to the hypothesis that an increase in the GDI symmetry ratio is associated with the DCD trait. A quantitative assessment of gait performance is important to help identify gait asymmetry, including gait quality, in children with a DCD trait. Children with a DCD trait have a poor ability to control the interaction of lower leg movements, which may cause bilateral asymmetry in gait quality.

DOI： 10.1016/j.gaitpost.2021.05.027

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Neurofibromatosis type 1 (NF1) is a genetic multisystem disorder. Clinicians must be aware of the diverse clinical features of this disorder in order to provide optimal care for it. We have set up an NF1 in-hospital medical care network of specialists regardless of patient age, launching a multidisciplinary approach to the disease for the first time in Japan. From January 2014 to December 2020, 246 patients were enrolled in the NF1 patient list and medical records. Mean age was 26.0 years ranging from 3 months to 80 years. The number of patients was higher as age at first visit was lower. There were 107 males (41%) and 139 females. After 2011, the number of patients has increased since the year when the medical care network was started. Regarding orthopedic signs, scoliosis was present in 60 cases (26%), and bone abnormalities in the upper arm, forearm, and tibia in 8 cases (3.5%). Neurofibromas other than cutaneous neurofibromas were present in 90 cases (39%), and MPNST in 17 cases (7.4%). We launched a multidisciplinary NF1 clinic system for the first time in Japan. For patients with NF1, which is a hereditary and systemic disease associated with a high incidence of malignant tumors, it will be of great benefit when the number of such clinics in Japan and the rest of Asia is increased.

DOI： 10.1038/s41598-021-91345-6

Open Access

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本臨床神経生理学会  

<p>脳波を患者の検査として用いる場合に, 臨床研究と臨床検査の境界は明確ではない場合がある。汎用性を持って施行可能なのか, 診断や治療方針の決定などに用いられるのかが研究か診療かの指標になるだろう。本編では, 小児の脳波検査において研究からclinical toolとして確立されてきた, または確立していく可能性のある手法について解説する。amplitude-integrated EEG (aEEG) は新生児領域で広く発作検出の臨床目的で使われており, 小児のICU脳波モニタリングでも用いられている。高周波律動も, デジタル脳波の普及に伴い用いられるようになった手法で, 頭皮脳波でも評価されている。脳波を他のモダリティと組み合わせることも脳波の新しい活用法である。脳波–機能的MRI同時記録は, 脳波活動に伴う血流変化をMRIで可視化し, てんかん焦点, ネットワークの解明に用いられている。</p>

DOI： 10.11422/jscn.49.141

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本臨床神経生理学会  

脳波を患者の検査として用いる場合に,臨床研究と臨床検査の境界は明確ではない場合がある。汎用性を持って施行可能なのか,診断や治療方針の決定などに用いられるのかが研究か診療かの指標になるだろう。本編では,小児の脳波検査において研究からclinical toolとして確立されてきた,または確立していく可能性のある手法について解説する。amplitude-integrated EEG(aEEG)は新生児領域で広く発作検出の臨床目的で使われており,小児のICU脳波モニタリングでも用いられている。高周波律動も,デジタル脳波の普及に伴い用いられるようになった手法で,頭皮脳波でも評価されている。脳波を他のモダリティと組み合わせることも脳波の新しい活用法である。脳波-機能的MRI同時記録は,脳波活動に伴う血流変化をMRIで可視化し,てんかん焦点,ネットワークの解明に用いられている。(著者抄録)

DOI： 10.11422/jscn.49.141

Open Access

Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2021&ichushi_jid=J03414&link_issn=&doc_id=20210607260004&doc_link_id=%2Fff2rinsy%2F2021%2F004903%2F005%2F0141-0144%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fff2rinsy%2F2021%2F004903%2F005%2F0141-0144%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本周産期・新生児医学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Bacille de Calmette et Guérin (BCG) is a live vaccine for tuberculosis that is administered to all infants in Japan. Adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS) causes immunosuppression and may result in BCG infection after BCG vaccination. We evaluated the safety of ACTH therapy initiated shortly after BCG vaccination. METHODS: We analyzed patients with WS who received ACTH therapy between 2005 and 2018. We evaluated the interval between BCG and ACTH therapy, and the rate of BCG infection during and after ACTH therapy, by retrospective chart review. RESULTS: Seventy-nine patients were included in the analysis. Twenty-three patients received ACTH therapy prior to BCG vaccination. For the remaining 56 patients, the median interval between BCG vaccination and the start of ACTH therapy (BCG-ACTH interval) was 91.5 (range 14-280) days. The BCG-ACTH interval was shorter in patients with unknown than in those with known etiologies. It was <8 weeks in 13 patients (10 with unknown and 3 with known etiologies). The minimum BCG-ACTH interval was 14 days. Six patients with epileptic spasms received BCG vaccinations because physicians did not recognize their seizures. None of the patients developed BCG infection. CONCLUSION: No patients who received ACTH therapy after BCG, even at an interval of 8 weeks, developed BCG infection. The timing of ACTH therapy initiation should be based on the risk of BCG-related adverse events and the adverse effects of any delay.

DOI： 10.1016/j.yebeh.2021.107924

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Although the initial treatment of childhood seizures is important, treatment within an appropriate time window is often difficult in resource-limited areas. This study examined childhood seizure treatment in a rural area in Japan. METHODS: We retrospectively investigated children presenting to Nakatsugawa Municipal General Hospital emergency department between 2015 and 2018. From the hospital database, we identified children who were diagnosed with seizures, epilepsy, or acute infectious encephalitis/encephalopathy or were given benzodiazepines. We considered etiology, seizure duration, and treatment according to the specialties of the doctors providing initial care. RESULTS: We extracted 236 seizure events: 40 initially treated by pediatricians, 16 by a mobile doctor team, and 180 by other doctors. Twenty patients had continuous seizures for longer than 5 min on admission. Two were treated by pediatricians at presentation; it took 4 and 7 min after arrival to stop the seizures. Four were treated by a mobile team, and 14 by other doctors; the median response times were 11.5 (range 3-47) and 19 (range 5-60) min, respectively. All patients treated by pediatricians or mobile doctor teams received intravenous or intramuscular diazepam, whereas 50% of those treated by other doctors initially received diazepam suppositories. In three of the 20 events, establishing intravenous access was difficult. SIGNIFICANCE: In rural Japan, many children with seizures are initially treated by doctors other than pediatricians or emergency physicians, and they require a longer time to achieve seizure cessation. Non-intravenous benzodiazepine formulas, which have not yet been approved in Japan, would be helpful.

DOI： 10.1016/j.braindev.2020.08.004

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND AND PURPOSE: Bottom shuffling is a locomotion strategy that precedes independent walking in some infants. Shuffling babies are generally considered to have favorable outcomes. The aim of the present study was to reveal clinical features and neurodevelopmental outcomes of shuffling babies who visited a child developmental center. METHODS: We studied 48 shuffling babies who visited Toyota Municipal Child Development Center from April 2007 to March 2015. We excluded patients with cerebral palsy, Down syndrome, or congenital disorders. In 2018, we retrospectively reviewed the clinical charts of the enrolled children. We investigated family history, neurological findings, and the developmental outcome during the follow-up period. RESULTS: During the follow-up period, 20 children (42%) were diagnosed with ASD. Gross motor development in infancy was not different between infants with and without ASD. The rate of poor eye contact at the first visit and a delay in the first word speech were significantly higher in infants with ASD than in infants without ASD. A family history of bottom shuffling was significantly less frequent in infants with ASD (10%) than in those without (39%). CONCLUSION: Some of bottom shufflers may represent ASD during follow-up. Paying attention to social and cognitive functions in shuffling babies is important.

DOI： 10.1016/j.braindev.2020.08.007

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: To establish an objective method of [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET) that can assist in the diagnosis of glucose transporter 1 deficiency syndrome (GLUT1-DS). METHODS: FDG-PET was performed in 8 patients with a mean age of 12.5 years (range, 2-22 years) with GLUT1-DS. Their PET findings were compared with those of 45 controls with a mean age of 11.2 years (range, 2-21 years) by statistical parametric mapping (SPM12, Welcome Neurological Institute). The controls had epilepsy of unknown etiology and normal MRI findings. The age-adjusted ratios of mean radioactivities in regions of interest (ROIs) of bilateral lenticular nuclei, thalami, and the whole cerebral cortex were also measured. The sensitivities and specificities of the ratios for the differential diagnosis of GLUT1-DS were also determined. RESULTS: SPM showed significantly decreased uptake in bilateral thalami and increased uptake in bilateral lenticular nuclei in patients with GLUT1-DS. There were no areas in the cerebral cortex with significant differences between patients and controls. On ROI analysis, by setting the cut-off value of the age-adjusted lenticular nuclei/thalami radioactivity ratio to 1.54, patients with GLUT1-DS were differentiated from controls with sensitivity of 1.00 and specificity of 0.98. CONCLUSION: The age-adjusted lenticular nuclei/thalami radioactivity ratio on PET can distinguish patients with GLUT1-DS from patients with epilepsy of unknown etiology with high sensitivity and specificity. It is important to pay attention to the metabolism of the lenticular nuclei and thalami on PET for the diagnosis of GLUT1-DS.

DOI： 10.1016/j.braindev.2020.07.001

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Williams syndrome (WS) is a genetically based neurodevelopmental disorder characterized by intellectual disability and impaired visuospatial recognition. The aim of this study was to analyze the gait characteristics of WS children with impaired visuospatial recognition using a three-dimensional gait analysis (3DGA) to clarify the gait adaptation needed to compensate for it. 3DGA was performed in 8 WS children with impaired visuospatial recognition (mean age, 11.8 years) and 9 age-, sex-, height-, and weight-matched controls. Clinical data, fundamental motor tests, and gait variables while walking on a flat surface and walking up a mat were compared between the two groups, and the correlations between variables were analyzed in the WS children. WS children showed impairment of balance function without muscle weakness. In walking on a flat surface, the WS group showed reduced walking speed, short step length, increased variability of step length, increased knee flexion throughout the stance phase, increased horizontal pelvic range of motion (ROM), and a low Gait Deviation Index and a high Gait Profile Score, which are indices of gait quality. In walking up a mat, the WS group showed further reduced walking speed and decreased sagittal hip flexion and ankle dorsiflexion ROM in the swing phase. Impaired balance function was significantly correlated with increased variability of step length and decreased sagittal ankle dorsiflexion ROM in the swing phase. The detailed gait pattern of WS children with impaired visuospatial recognition was presented. These findings show that impaired visuospatial recognition and balance function contribute to gait adaptation.

DOI： 10.1007/s00221-020-05946-0

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J01232&link_issn=&doc_id=20201030160010&doc_link_id=10.11251%2Fojjscn.52.414&url=https%3A%2F%2Fdoi.org%2F10.11251%2Fojjscn.52.414&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Advances in hematopoietic stem cell transplantation have improved the survival rate of malignant diseases and congenital immunodeficiencies. It has become important to assess long-term complications in survivors. To assess neurological abnormalities in children treated by transplantation, diffusion tensor imaging was performed. METHODS: Forty children who underwent head diffusion tensor imaging before and after their first transplantation were enrolled. Patients with brain lesions on conventional MRI were excluded. Fractional anisotropy and mean diffusivity were compared between patients and 28 control subjects using tract-based spatial statistics. The Strengths and Difficulties Questionnaire was administered as a behavioral evaluation after transplantation, and diffusion tensor images of patients with and without behavioral abnormalities were compared. RESULTS: The age of patients and controls was 0 to 19 years and 0 to 16 years, respectively. The date of diffusion tensor imaging was 10 to 57 days before and 40 to 153 days after transplantation. Tract-based spatial statistics showed fractional anisotropy reduction in widespread white matter in patients before and after transplantation. Mean diffusivity was high before transplantation and normalized after transplantation. Analysis comparing before and after hematopoietic stem cell transplantation shows no difference in fractional anisotropy and a higher mean diffusivity before hematopoietic stem cell transplantation. In patients with behavioral abnormalities, low fractional anisotropy and high mean diffusivity remained after transplantation. CONCLUSIONS: Longitudinal diffusion tensor imaging showed white matter abnormalities in children without conventional MRI abnormalities, which were related to behavioral problems after transplantation. Diffusion tensor imaging is useful for behavioral assessment in children undergoing transplantation.

DOI： 10.1016/j.pediatrneurol.2020.06.008

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Clinical identification of neonatal seizures (NS) remains challenging. The International League Against Epilepsy (ILAE) Task Force on Neonatal Seizures has proposed a new classification of NS, based on the 2017 ILAE seizure classification. One of the key points of this proposed NS classification is that seizure types should be determined by the "predominant" clinical feature. However, when the definition of "predominant" is uncertain, interobserver variability may arise. METHODS: We asked 49 health professionals to classify 21 NS video-electroencephalogram (EEG) recordings using the proposed 9 seizure types. RESULTS: The degree of agreement among participants was low, and agreement was weak among experts in neonatal neurology. Among experts, the rate of agreement was <50% for 2 NS. This disagreement was related to differences in the interpretation of "predominant features." Although interobserver variability was present among users of the new NS classification, the reproducibility of the NS classification was satisfactory. CONCLUSION: Education designed to foster consistent application of the standards for NS will be important for reducing interobserver variability and expanding the use of the new NS classification.

DOI： 10.1016/j.yebeh.2020.107292

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: To investigate clinical risk factors for acute magnetic resonance imaging (MRI) abnormalities in patients with benign convulsions with mild gastroenteritis or benign infantile epilepsy. STUDY DESIGN: We investigated clinical and diffusion-weighted imaging findings in 32 patients with benign convulsions with mild gastroenteritis and 22 patients with benign infantile epilepsy who underwent MRI within seven days of seizure onset between 2010 and 2015. RESULTS: Diffusion-weighted imaging showed signal hyperintensity in the splenium of the corpus callosum in seven patients with benign convulsions with mild gastroenteritis, but no abnormalities in patients with benign infantile epilepsy. Patients with benign convulsions with mild gastroenteritis with splenial lesions showed a higher rate of rotavirus detection from feces (P = 0.006), higher serum level of C-reactive protein (P = 0.04), and shorter interval between seizure onset and MRI (P = 0.002) than patients with benign convulsions with mild gastroenteritis without splenial lesions. Multivariate analysis revealed rotavirus infection as a significant risk factor for splenial lesions on diffusion-weighted imaging in patients with benign convulsions with mild gastroenteritis (P = 0.02). CONCLUSIONS: Splenial lesions are often seen during acute period in patients with benign convulsions with mild gastroenteritis. Rotavirus infection is a risk factor for splenial lesions in patients with benign convulsions with mild gastroenteritis, suggesting the role of rotavirus to cause edema in the corpus callosum. From our observations, benign convulsions with mild gastroenteritis with a splenial lesion on diffusion-weighted imaging suggests good outcomes, and extensive evaluation of these patients may be unnecessary.

DOI： 10.1016/j.pediatrneurol.2019.05.002

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

AIM: Little is known about acute febrile status epilepticus-induced injury of extrahippocampal structures. To clarify the presence and clinical significance of acute extrahippocampal injuries, we performed diffusion-weighted imaging (DWI) in children immediately after prolonged febrile seizure (PFS). METHOD: We performed a retrospective cohort study in children younger than 6 years old who visited one of two hospitals due to PFSs between January 2013 and October 2018. PFS was defined as a febrile seizure that persisted for 15 min or longer. We collected brain DWI data within 6 h of the end of PFS. When the initial DWI detected an abnormality, a follow-up DWI was performed a few days later. RESULTS: The study population consisted of 101 patients with PFSs. DWI was performed within 6 h in 51 patients, while the remaining 50 patients did not undergo imaging because of good recovery of consciousness. Restricted cortical diffusion was evident in 9 (18%) patients on initial DWI. All of them underwent DWI within 100 min after PFS. Restricted cortical diffusion was associated with male sex, asymmetrical PFS symptoms, and a shorter duration between the end of the seizure and DWI, but was not associated with seizure duration. All cortical abnormalities had resolved on follow-up DWI of these patients within 72 h after the initial imaging, but ipsilateral hippocampal hyperintensity appeared in one patient. All 9 patients with restricted cortical diffusion were finally diagnosed with PFS and discharged without sequelae. CONCLUSIONS: Some children with PFSs exhibit transient restricted diffusion in the regional cortex on DWI performed immediately after the end of PFS. These transient diffusion changes were not associated with unfavorable epileptic sequelae or neuroimaging in the short-term.

DOI： 10.1016/j.ejpn.2020.05.004

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Febrile seizures (FSs) typically occur in infants and children between 6 and 60 months of age. Rarely, FS can occur in late childhood (late FS [LFS]; >5 years of age); however, the clinical features of LFS remain unclear. We aimed to clarify the clinical features of LFS. METHODS: We retrospectively analyzed data from patients with LFS who visited Hirakata City Hospital between January 2004 and December 2014. We defined LFS as a seizure accompanied by fever (temperature ≥38 °C) occurring after 5 years of age, without a central nervous system infection. RESULTS: A total of 505 patients (349 boys, 156 girls: 5-14 years old) were included. A history of FS before 60 months of age was observed in 319 of 460 patients (69.3%) with sufficient information about previous FS history among the 505 patients enrolled. LFS was more likely to occur in males (69.1%). Seizure duration was ≤15 min in 87.4% of cases. A family history of FS in first-degree relatives was observed in 103/327 cases (31.5%). Among LFS cases, 45% occurred at 5 years of age, and 92.1% experienced only one seizure after 5 years of age. The number of seizure episodes gradually lessened with age, decreasing drastically to 5.6% of cases older than 9 years. CONCLUSIONS: Our findings suggest that sex differences, seizure duration, and family history were similar for LFS and FS. Over 90% patients with LFS experienced no recurrence after 5 years of age. Further study is needed to verify the recurrence rate of LFS.

DOI： 10.1016/j.braindev.2020.02.009

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：(株)東京医学社  

DOI： 10.24479/j00648.2020230092

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：BMJ  

<sec><title>Background</title>3C/Ritscher-Schinzel syndrome is characterised by congenital cranio-cerebello-cardiac dysplasia, where <italic>CCDC22</italic> and <italic>WASHC5</italic> are accepted as the causative genes. In combination with the retromer or retriever complex, these genes play a role in endosomal membrane protein recycling. We aimed to identify the gene abnormality responsible for the pathogenicity in siblings with a 3C/Ritscher-Schinzel-like syndrome, displaying cranio-cerebello-cardiac dysplasia, coloboma, microphthalmia, chondrodysplasia punctata and complicated skeletal malformation.

</sec><sec><title>Methods</title>Exome sequencing was performed to identify pathogenic variants. Cellular biological analyses and generation of knockout mice were carried out to elucidate the gene function and pathophysiological significance of the identified variants.

</sec><sec><title>Results</title>We identified compound heterozygous pathogenic variants (c.1097dup; p.Cys366Trpfs*28 and c.2755G&gt;A; p.Ala919Thr) in the <italic>VPS35L</italic> gene, which encodes a core protein of the retriever complex. The identified missense variant lacked the ability to form the retriever complex, and the frameshift variant induced non-sense-mediated mRNA decay, thereby confirming biallelic loss of function of VPS35L. In addition, <italic>VPS35L</italic> knockout cells showed decreased autophagic function in nutrient-rich and starvation conditions, as well as following treatment with Torin 1. We also generated <italic>Vps35l^−/−</italic> mice and demonstrated that they were embryonic lethal at an early stage, between E7.5 and E10.5.

</sec><sec><title>Conclusions</title>Our results suggest that biallelic loss-of-function variants in <italic>VPS35L</italic> underlies 3C/Ritscher-Schinzel-like syndrome. Furthermore, VPS35L is necessary for autophagic function and essential for early embryonic development. The data presented here provide a new insight into the critical role of the retriever complex in fetal development.

</sec>

DOI： 10.1136/jmedgenet-2019-106213

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The authors recently reported a novel subsecond analysis method of analyzing EEG-functional MRI (fMRI) to improve the detection rate of epileptic focus. This study aims to validate the utility of this method for presurgical evaluation in pharmacoresistant focal epilepsy. METHODS: Among 13 patients with focal epilepsy undergoing presurgical examinations including simultaneous EEG-fMRI at 3T, 11 patients had interictal epileptiform discharges (IEDs) during fMRI. The authors used the sequence of topographic maps during the IEDs as a reference to obtain subsecond fMRI activation maps with the same temporal resolution as the EEG data, and constructed "spike-and-slow-wave-activation-summary" (SSWAS) maps that showed the activation frequency of voxels during IEDs. Clusters were defined by thresholding the SSWAS maps (voxel value > 10), and those containing voxels with the top 3 highest activation frequencies were considered significant. Significant hemodynamic responses using conventional event-related (ER) analysis and SSWAS maps were compared with the resection areas and surgical outcomes at 1 year after surgery. RESULTS: Using ER analysis, 4 (36%) of 11 patients had significant hemodynamic responses. One of 4 patients had significant hemodynamic responses in the resection area and good surgical outcome. Using SSWAS maps, 10 (91%) of 11 patients had significant hemodynamic responses. Six of 10 patients had significant hemodynamic responses in the resection area, and 5 of the 6 patients achieved good surgical outcomes. The remaining 4 patients had significant hemodynamic responses distant from the resection area, and only 1 of the 4 patients achieved good surgical outcomes. The sensitivity, specificity, positive predictive value, and negative predictive value of SSWAS maps were 83.3%, 75.0%, 83.3%, and 75.0%, respectively. CONCLUSIONS: This study demonstrated the clinical utility of SSWAS maps for presurgical evaluation of pharmacoresistant focal epilepsy. The findings indicated that subsecond EEG-fMRI analysis may help surgeons choose the resection areas that could lead to good surgical outcomes.

DOI： 10.3171/2020.1.JNS192567

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.braindev.2019.11.006

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

OBJECTIVE: The objective of this study was to describe a novel amplitude-integrated electroencephalography (aEEG) pattern in infants with hypoxic-ischemic encephalopathy (HIE) and to assess the clinical significance. METHODS: The aEEG traces of infants with HIE who were treated with therapeutic hypothermia (TH) from 2012 to 2017 were analyzed. A pseudo-sawtooth (PST) pattern was defined as a periodic increase of the upper and/or lower margin of the trace on aEEG without showing seizure activities on conventional EEG (CEEG). RESULTS: Of the 46 infants, 6 (13%) had the PST pattern. The PST pattern appeared following a flat trace or a continuous low-voltage pattern and was followed by a burst-suppression pattern. On CEEG, the PST pattern consists of alternating cycles of low-voltage irregular activities and almost flat tracing. The PST pattern was associated with neuroimaging abnormalities and with various degrees of neurodevelopmental outcomes. Positive predictive values of the PST or worse pattern for adverse outcomes were high at 12 h after birth. CONCLUSION: A novel aEEG background pattern in infants with HIE was reported. The PST pattern likely indicates a suppressed background pattern and may be linked to unfavorable outcomes. Further multicenter validation study is needed to clarify its clinical significance.

DOI： 10.1038/s41390-019-0567-5

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PubMed

Other Link： http://www.nature.com/articles/s41390-019-0567-5

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

<p>  Myelin oligodendrocyte glycoprotein antibody (MOG-Ab) is identified in some patients with ADEM. Whether serial change of MOG-Ab titer is useful for deciding on the therapeutic strategy for multiphasic ADEM has not been clarified. We report the case of multiphasic ADEM who was treated with intravenous immunoglobulin (IVIG) therapy and whose MOG-Ab was serially evaluated. The patient is 9-year-old boy. He showed acute onset of headache and delirium. T2-weighted and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) showed multiple hyperintense lesions in bilateral subcortical and cortical areas, and we diagnosed the patient with ADEM. Although symptoms and MRI lesions were resolved by steroid pulse therapy, he experienced three time relapses characterized by headache and abnormal behavior as well as new MRI lesions during tapering of the therapy. We started monthly IVIG from 5 months after onset, and no relapse of ADEM was encountered. MOG-Ab titer ranged from 4,096 to 2,048 before IVIG therapy. After starting IVIG, MOG-Ab titer gradually decreased. We were able to decrease and discontinue oral steroids. After steroid discontinuation, MOG-Ab titer remained low. We started to decrease the dose of IVIG at 1 year and 3 months after the onset of ADEM and terminated IVIG at 1 year and 9 months after onset by checking low titer of MOG-Ab. Our results suggest that serial evaluations of MOG-Ab may contribute to the monitoring of disease activity and decision-making on drug tapering and termination for multiphasic ADEM. The utility of serial evaluations should be validated in studies of larger numbers of patients.</p>

DOI： 10.11251/ojjscn.52.414

Scopus

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：金原出版  

DOI： 10.18888/sh.0000001131

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

Web of Science

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.13939

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Congress on Neurological Emergencies  

<p>It is important to detect non-convulsive seizures in the treatment of critically ill children in ICU. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by a febrile status epilepticus as the initial neurological symptom, followed by secondary seizures and subcortical white matter edema at day 4 to 6. As MRI shows no acute abnormality at the onset, EEG is useful to distinguish AESD from febrile status epilepticus in early stage. Recently therapeutic hypothermia is applied as a treatment of AESD, and continuous EEG monitoring during the therapy is also important. Amplitudeintegrated EEG (aEEG) and dense spectral array (DSA) are useful for the identification of non-convulsive seizures in long-term EEG monitoring in ICU.</p>

DOI： 10.11170/jjsnecc.31.2_22

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：National Institute of Infectious Diseases  

<p>Human metapneumovirus (HMPV) and respiratory syncytial virus (RSV) are the leading causes of acute respiratory tract infection in children, and clinical manifestations of these virus infections are considered similar. To investigate the differences in clinical characteristics between HMPV and RSV infections in young children, we prospectively enrolled children ＜ 3 years old who required hospitalization with acute respiratory tract infection due to HMPV or RSV at 10 hospitals in Japan. We enrolled 48 children with HMPV infection and 141 with RSV infection. Patients with HMPV infection were older than those with RSV infection. High-grade fever was more frequently observed in patients with HMPV infection, whereas no significant differences in respiratory symptoms were apparent. Abnormal serum lactate dehydrogenase values and consolidation shadows on chest X-ray were more frequently observed in patients with HMPV infection. During hospitalization, nasal mucus suction was more frequently required in patients with RSV infection. On the other hand, β2-adrenergic agonists, corticosteroids, and leukotriene receptor antagonists were more frequently used in patients with HMPV infection. These findings suggest that HMPV and RSV infections show similar respiratory symptoms, but HMPV infection is more likely to lead to the development of pneumonia, at least among hospitalized young children.</p>

DOI： 10.7883/yoken.JJID.2018.480

Open Access

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PubMed

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

OBJECTIVE: To assess hippocampal signal changes on diffusion-weighted imaging (DWI) during the acute period after febrile status epilepticus (FSE) and to examine the relationship between DWI and subsequent epilepsy. METHODS: A prospective, multicenter study of children with a first episode of FSE was performed. The patients underwent magnetic resonance imaging (MRI) within 3 days of FSE, and signal intensity was evaluated on DWI. Electroencephalography studies within 3 days of FSE were also assessed. Nine to 13 years after FSE, information on subsequent epilepsy was obtained. RESULTS: Twenty-two children with FSE were evaluated. DWI showed unilateral hippocampal hyperintensity in six patients (27%). Three of six patients with hippocampal hyperintensity had ipsilateral thalamic hyperintensity. On EEG within 3 days of FSE, five of six patients with hippocampal hyperintensity had ipsilateral focal slowing, spikes, or attenuation. Nine to 13 years later, the outcomes could be determined in five patients with hippocampal hyperintensity and in 10 without. All 5 patients with hippocampal hyperintensity had hippocampal atrophy and developed focal epilepsy, whereas only 1 of 10 patients without hippocampal hyperintensity developed epilepsy (P = 0.002). Ictal semiology was concordant with temporal lobe seizures in all patients. Ipsilateral temporal epileptiform abnormalities were seen on EEG in four of five at last follow-up. SIGNIFICANCE: Acute DWI hippocampal hyperintensity was seen in 27% of patients with FSE. Acute DWI hyperintensity suggests cytotoxic edema caused by prolonged seizure activity. Hippocampal DWI hyperintensity is related to mesial temporal lobe epilepsy and can be a target of neuroprotective treatments to prevent the onset of epilepsy.

DOI： 10.1111/epi.16059

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PubMed

Other Link： https://onlinelibrary.wiley.com/doi/full-xml/10.1111/epi.16059

Language：English   Publishing type：Research paper (scientific journal)  

Neonates who swallow a considerable amount of maternal blood may exhibit vomiting and suckling disorder during the first few days of the postnatal period. Some clinicians treat these neonates with gastric lavage (GL) to prevent vomiting and the establishment of enteral feeding empirically, but there was no study assessing the effect of GL for neonates with coffee-ground emesis. We designed a multicenter randomized controlled trial to evaluate the efficacy and safety of GL in neonates with coffee-ground emesis. Vigorous neonates with birth weight ranging from 2500 g to 3999 g and gestational age between 37w0d and 41w6d who presented with coffee-ground emesis on more than twice and diagnosed as false melena, were divided into two groups using computerized randomization. We defined feeding intolerance (FI) as (1) ≥2 vomiting episodes in 4h or ≥3 episodes in 24h and/or (2) feeding failure on at least two occasions because of retching or poor sucking. Primary outcome is percentage of infants who present FI within 24 hours from admission. We also assessed the residual volumes, number of vomiting episodes, percentage of weight reduction at postnatal day 4, rates of body weight gain at 1 month of age, and peak serum total bilirubin value before discharge. To our knowledge, this is the first study to evaluate the safety and efficacy of GL for neonates with coffee-ground emesis. This trial is registered at UMIN Clinical Trials Registry as UMIN000026483.

DOI： 10.18999/nagjms.81.2.227

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Mycoplasma pneumoniae pneumonia (MPP) is generally a self-limiting disease, but it may become refractory. It is thought that refractory MPP is linked to the excessive immunologic responses of the host. Consequently, the use of adjunctive systemic corticosteroids may have beneficial effects. In this study, we compared the effects of high- and low-dose corticosteroid therapy in a pediatric population with refractory MPP. METHODS: We retrospectively collected data from 91 pediatric MPP patients treated with adjunctive systemic corticosteroids between April 2014 and October 2016. The patients were divided into the following two groups: high-dose corticosteroid group (2 mg/kg/day or more of prednisolone equivalents; n = 38) and low-dose corticosteroid group (<2 mg/kg/day; n = 53). Additionally, we compared the number of febrile days post-corticosteroid administration. We used 25 paired patients in a propensity score matching analysis to correct for confounding factors both by age and by days (from onset till corticosteroid therapy initiation). RESULTS: We observed that in the high-dose corticosteroid group defervescence following corticosteroid therapy initiation was achieved significantly earlier and length of hospitalization was significantly shorter (0.8 ± 1.0 vs. 1.5 ± 1.4 days and 8.2 ± 2.4 vs. 10.7 ± 2.7 days, respectively). In the propensity score matching, we observed that significant differences in the length of fever following corticosteroid therapy initiation and hospitalization were still present. Further, neither of the groups developed corticosteroid-related adverse events. CONCLUSION: Our results suggest that patients with refractory MPP treated with high-dose corticosteroid could achieve defervescence earlier and have a shorter hospitalization.

DOI： 10.1016/j.jiac.2019.01.003

Open Access

Web of Science

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Japanese Society of Clinical Neurophysiology  

<p>It is important to detect non-convulsive seizures in the treatment of critically ill children in ICU. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by a febrile status epilepticus as the initial neurological symptom, followed by secondary seizures and subcortical white matter edema at day 4 to 6. As MRI shows no acute abnormality at the onset, EEG is useful to distinguish AESD from febrile status epilepticus in early stage. Recently therapeutic hypothermia is applied as a treatment of AESD, and continuous EEG monitoring during the therapy is also important. Amplitude-integrated EEG (aEEG) and dense spectral array (DSA) are useful for the identification of non-convulsive seizures in long-term EEG monitoring in ICU.</p>

DOI： 10.11422/jscn.47.53

Open Access

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：メディカルレビュー社  

DOI： 10.34449/j0055.10.03_0023-0028

CiNii Research

Other Link： http://search.jamas.or.jp/link/ui/2019083970

Language：English   Publishing type：Research paper (scientific journal)  

[This corrects the article DOI: 10.1371/journal.pone.0192688.].

DOI： 10.1371/journal.pone.0218205

Open Access

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：American Society of Neuroradiology ({ASNR})  

DOI： 10.3174/ajnr.A5772

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：BMJ  

OBJECTIVE: To compare magnetic resonance spectroscopy (MRS) metabolite ratios in preterm infants at term-equivalent age with those in term infants and to evaluate the association between MRS metabolites and neurodevelopmental outcomes at 18 months corrected age in preterm infants. DESIGN: We studied infants born at a gestational age <37 weeks and weighing <1500 g during 2009-2013 using MRS at term-equivalent age. Infants with major brain abnormalities were excluded. The ratios of N-acetylaspartate (NAA) to creatine (Cre), NAA to choline-containing compounds (Cho) and Cho to Cre in the frontal white matter and thalamus were measured using multivoxel point-resolved proton spectroscopy sequence. Neurodevelopmental outcomes were assessed at 18 months corrected age. RESULTS: Thirty-three preterm infants and 16 term infants were enrolled in this study. Preterm infants with normal development at 18 months showed significantly lower NAA/Cho ratios in the frontal white matter than term infants. There were no differences in the Cre/Cho ratios between preterm and term infants. At 18 months corrected age, 9 preterm infants with a mild developmental delay showed significantly lower NAA/Cho ratios in the thalamus than 24 preterm infants with normal development. CONCLUSIONS: Preterm infants at term-equivalent age showed reduced MRS metabolites (NAA/Cho) compared with term infants. Decreased NAA/Cho ratios in the thalamus were associated with neurodevelopmental delay at 18 months corrected age in preterm infants.

DOI： 10.1136/archdischild-2016-311403

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The aim of this prospective multicenter randomized controlled trial was to compare the efficacy of silver nitrate cauterization against that of topical steroid ointment in the treatment of neonatal umbilical granuloma. METHODS: An open-label, non-inferiority randomized controlled trial was conducted from January 2013 to January 2016. The primary endpoint for the silver nitrate cauterization and topical steroid ointment groups was the healing rate after 2 weeks of treatment, applying a non-inferiority margin of 10%. The healing rate was evaluated until completion of 3 weeks of treatment. RESULTS: Participants comprised 207 neonates with newly diagnosed umbilical granuloma, randomized to receive silver nitrate cauterization (n = 104) or topical steroid ointment (n = 103). Healing rates after 2 weeks of treatment were 87.5% (91/104) in the silver nitrate cauterization and 82% (82/100) in the topical steroid ointment group group. The difference between groups was -5.5% (95% confidence interval, -19.1%, 8.4%), indicating that the non-inferiority criterion was not met. After 3 weeks of treatment, the healing rate with topical steroid ointment treatment was almost identical to that of silver nitrate cauterization (94/104 [90.4%] vs. 91/100 [91.0%]; 0.6% [-13.2 to 14.3]). No major complications occurred in either group. CONCLUSIONS: This study did not establish non-inferiority of topical steroid ointment treatment relative to silver nitrate cauterization, presumably due to lower healing rates than expected leading to an underpowered trial. However, considering that silver nitrate cauterization carries a distinct risk of chemical burns and that the overall efficacy of topical steroid ointment treatment is similar to that of silver nitrate cauterization, topical steroid ointment might be considered as a good alternative in the treatment of neonatal umbilical granuloma due to its safety and simplicity. To clarify non-inferiority, a larger study is needed.

DOI： 10.1371/journal.pone.0192688

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

OBJECTIVE: To clarify longitudinal changes in white matter microstructures from the onset of disease in patients with West syndrome (WS) of unknown etiology. METHODS: Diffusion tensor imaging (DTI) was prospectively performed at onset and at 12 and 24 months old in 17 children with WS of unknown etiology. DTI was analyzed using tract-based spatial statistics (TBSS) and tract-specific analysis (TSA) of 13 fiber tracts, and fractional anisotropy (FA) and mean diffusivity (MD) were compared with those of 42 age-matched controls. Correlations of FA and MD with developmental quotient (DQ) at age 24 months were analyzed. Multiple comparisons were adjusted for using the false discovery rate (q-value). RESULTS: TBSS analysis at onset showed higher FA and lower MD in the corpus callosum and brainstem in patients. TSA showed lower MD in bilateral uncinate fasciculi (UF) (right: q < 0.001; left: q = 0.03) at onset in patients. TBSS showed a negative correlation between FA at onset and DQ in the right frontal lobe, whereas FA at 24 months old exhibited a positive correlation with DQ in the diffuse white matter. MD for bilateral UF at 24 months old on TSA correlated positively with DQ (q = 0.04, both). SIGNIFICANCE: These findings may indicate the existence of cytotoxic edema in the immature white matter and dorsal brainstem at onset, and subsequent alterations in the diffuse white matter in WS of unknown etiology. Microstructural development in the UF might play important roles in cognitive development in WS.

DOI： 10.1111/epi.13988

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(公社)日本小児科学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Nature Publishing Group  

Objective:Nonspecific manifestations and a varied distribution of brain lesions can delay the diagnosis of herpes simplex encephalitis (HSE) in neonates. The aim of this study was to report predominant brain lesions in neonatal HSE, and then to investigate the association between pattern of predominant brain lesions, clinical variables and neurodevelopmental outcome.Study Design:A multicenter retrospective study was performed in neonates diagnosed with HSE between 2009 and 2014. Magnetic resonance (MR) images, including diffusion-weighted images, were obtained in the acute and chronic phase.Results:Three predominant areas of brain injury could be defined based on characteristic MRI findings in 10 of the 13 infants (77%). The inferior frontal/temporal pole area was involved in five (38%) patients. The watershed distribution was present in six (46%) patients. Four (31%) infants involved the corticospinal tract area. No significant association was found between any predominant distribution of brain lesion pattern and sex, country, viral type or viral load. However, the corticospinal tract involvement was significantly associated with motor impairment (P=0.045).Conclusion:Three predominant areas of brain lesion could be recognized in neonatal HSE. Recognition of those areas can improve prediction of neurodevelopmental outcome.

DOI： 10.1038/jp.2017.114

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Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

Inherited GPI (glycosylphosphatidylinositol) deficiencies (IGDs), a recently defined group of diseases, show a broad spectrum of symptoms. Hyperphosphatasia mental retardation syndrome, also known as Mabry syndrome, is a type of IGDs. There are at least 26 genes involved in the biosynthesis and transport of GPI-anchored proteins; however, IGDs constitute a rare group of diseases, and correlations between the spectrum of symptoms and affected genes or the type of mutations have not been shown. Here, we report four newly identified and five previously described Japanese families with PIGO (phosphatidylinositol glycan anchor biosynthesis class O) deficiency. We show how the clinical severity of IGDs correlates with flow cytometric analysis of blood, functional analysis using a PIGO-deficient cell line, and the degree of hyperphosphatasia. The flow cytometric analysis and hyperphosphatasia are useful for IGD diagnosis, but the expression level of GPI-anchored proteins and the degree of hyperphosphatasia do not correlate, although functional studies do, with clinical severity. Compared with PIGA (phosphatidylinositol glycan anchor biosynthesis class A) deficiency, PIGO deficiency shows characteristic features, such as Hirschsprung disease, brachytelephalangy, and hyperphosphatasia. This report shows the precise spectrum of symptoms according to the severity of mutations and compares symptoms between different types of IGD.

DOI： 10.1002/humu.23219

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Public Library of Science (PLoS)  

OBJECTIVES: First, to apply a recently extended scoring system for preterm brain injury at term-equivalent age (TEA-)MRI in a regional extremely preterm cohort; second, to identify independent perinatal factors associated with this score; and third, to assess the prognostic value of this TEA-MRI score with respect to early neurodevelopmental outcome. STUDY DESIGN: 239 extremely preterm infants (median gestational age [range] in weeks: 26.6 [24.3-27.9]), admitted to the Wilhelmina Children's Hospital between 2006 and 2012 were included. Brain abnormalities in white matter, cortical and deep grey matter and cerebellum and brain growth were scored on T1- and T2-weighted TEA-MRI using the Kidokoro scoring system. Neurodevelopmental outcome was assessed at two years corrected age using the Bayley Scales of Infant and Toddler Development, third edition. The association between TEA-MRI and perinatal factors as well as neurodevelopmental outcome was evaluated using multivariable regression analysis. RESULTS: The distribution of brain abnormalities and brain metrics in the Utrecht cohort differed from the original St. Louis cohort (p < .05). Mechanical ventilation >7 days (β [95% confidence interval, CI]: 1.3 [.5; 2.0]) and parenteral nutrition >21 days (2.2 [1.2; 3.2]) were independently associated with higher global brain abnormality scores (p < .001). Global brain abnormality scores were inversely associated with cognitive (β in composite scores [95% CI]: -.7 [-1.2; -.2], p = .004), fine motor (β in scaled scores [95% CI]: -.1 [-.3; -.0], p = .007) and gross motor outcome (β in scaled scores [95% CI]: -.2 [-.3; -.1], p < .001) at two years corrected age, although the explained variances were low (R2 ≤.219). CONCLUSION: Patterns of brain injury differed between cohorts. Prolonged mechanical ventilation and parenteral nutrition were identified as independent perinatal risk factors. The prognostic value of the TEA-MRI score was rather limited in this well-performing cohort.

DOI： 10.1371/journal.pone.0177128

Open Access

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The aim of this study was to clarify the characteristics of paroxysmal nonepileptic events (PNEs) suspected as being epileptic seizures by families of children with epilepsy. METHODS: The video-EEG (vEEG) recordings of habitual paroxysmal events in children with epilepsy at Nagoya University Hospital between October 2006 and January 2016 were reviewed. Based on the doctor's suspicion before the vEEG, the PNEs were divided into two groups that included PNEs suspected as epileptic seizures and PNEs suspected as PNEs. PNEs in the former group were classified based on the suspected seizure type. RESULTS: Of 886 habitual paroxysmal events, vEEG confirmed that 83 events (68 children) were PNEs. The median age of the 68 children was 3.2 years. Concurrent epilepsies included focal epilepsies (n=33), infantile spasms (n=16), and other types (n=19). The most common types of PNEs were sleep myoclonus (n=11), followed by stereotypies (n=9), awake myoclonus (n=8), paroxysmal ocular deviations (PODs, n=8), and tonic posturing (n=8). Even after direct observation or video viewing, the doctors suspected epileptic seizures in all three of the PODs and two of the tonic posturing children. Before the vEEG, however, the accurate visual information led to the speculation that the four psychogenic and two sleep myoclonus events were all PNEs. Myoclonus, stereotypies, and head drops were often misdiagnosed as epileptic spasms, while PODs and tonic posturing were often misdiagnosed as focal seizures with motor components. Additionally, staring and motion arrest during a drowsy state were often misdiagnosed as focal dyscognitive seizures. Seven of eight patients with PODs had epileptic spasms that were concurrent with epileptic seizures. A diffuse cerebral lesion or reduced visual acuity was seen in seven patients with PODs. CONCLUSION: We re-emphasize that vEEG is essential for accurate diagnosis and provides evidence for listing POD in the differential diagnosis of oculomotor paroxysmal events.

DOI： 10.1016/j.eplepsyres.2017.02.009

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PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)  

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)  

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1371/journal.pone.0177128

Open Access

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Child Neurology  

<p>  Recent advances in perinatal and neonatal care of preterm infants have reduced the mortality rate and the incidence of severe brain injuries including periventricular leukomalacia, periventricular hemorrhagic infarction, and post-hemorrhagic hydrocephalus. However, the rates of cognitive and behavioral challenges remain high and stable; approximately 50% of extremely premature infants exhibit disabilities in these domains. Recent magnetic resonance imaging and electroencephalographic studies have revealed structural and functional changes in the preterm brain. Brain injury does not fully explain neurodevelopmental impairment; rather, alterations in brain development are in play. Subplate neurons (which manifest a transient existence during fetal life) play important roles in development of the thalamo-cortical networks that, in turn, form the somatosensory body map. Also, these neurons contribute to development of the cortical inhibitory system. Alterations in the activities of these neurons may contribute to the underlying mechanisms of the cognitive/behavioral and psychological impairments evident in extremely premature infants.</p>

DOI： 10.11251/ojjscn.49.315

CiNii Research

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J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

J-GLOBAL

Language：Japanese   Publisher：(株)東京医学社  

＜Key Points＞(1)小児脳波では基礎律動をはじめとする正常所見が年齢によって大きく変化する。(2)デジタル脳波計ではモンタージュやフィルタを変更して判読することが可能となった。(3)てんかん診療では発作時脳波や発作間欠時のてんかん性突発波を評価する。(4)aEEGは長時間モニタリングや発作検出に有用であるが、発作捕捉のうえで限界もある。(著者抄録)

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

Language：English   Publisher：(一社)日本小児血液・がん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：English   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：English   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

救急・ICU管理において脳波モニタリングが有用な疾患として、感染などを契機に発症する急性脳症がある。「二相性発作と遅発性拡散能低下を示す急性脳症(AESD)」と呼ばれる急性脳症は、発症時は熱性けいれん重積と鑑別が困難で、数日後に二相目の発作群発が起こるとともに高度の大脳白質の浮腫が出現する。発症時のMRIでは異常がみられないため、早期の熱性けいれんとの鑑別のために脳波が重要である。またICUで鎮静下に治療を行うため臨床観察のみでは発作の診断が困難で、脳波モニタリングが治療の指標になる。近年は急性脳症に対して低体温療法を試みることが増えており、低体温療法中の脳波所見も知っておく必要がある。これらのICU脳波モニタリングにはamplitude-integrated EEGやdense spectral arrayなどのトレンドグラムが有用である。(著者抄録)

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Language：Japanese   Publisher：(公社)日本小児科学会  

Language：English   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

J-GLOBAL

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：日本妊娠高血圧学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(株)診断と治療社  

Language：Japanese   Publisher：(公社)日本新生児成育医学会  

Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

J-GLOBAL

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(一社)日本小児神経学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(公社)日本小児科学会  

Language：Japanese   Publisher：(一社)日本てんかん学会  

Language：English   Presentation type：Oral presentation (invited, special)  

Language：English   Presentation type：Oral presentation (invited, special)  

Language：English   Presentation type：Oral presentation (general)  

Language：English   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Language：English   Presentation type：Oral presentation (invited, special)  

Language：English   Presentation type：Oral presentation (invited, special)