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The use of home-based image sensors for biological and environmental monitoring provides novel insight into health and development but it is difficult to evaluate people during their normal activities in their home. Therefore, we developed a low-cost infrared (IR) technology-based motion, location, temperature and thermal environment detection system that can be used non-invasively for long-term studies in the home environment. We tested this technology along with the associated analysis algorithm to visualize the effects of parental care and thermal environment on developmental state change in a non-human primate model, the common marmoset ( Callithrix jacchus ). To validate this system, we first compared it to a manual analysis technique and we then assessed the development of circadian rhythms in common marmosets from postnatal day 15-45. The semi-automatically tracked biological indices of locomotion velocity (BV) and body surface temperature (BT) and the potential psychological index of place preference toward the door (BD), showed age-dependent shifts in circadian phase patterns. Although environmental variables appeared to affect circadian rhythm development, principal component analysis and signal superimposing imaging methods revealed a novel phasic pattern of BD-BT correlation day/night switching in animals older than postnatal day 38 (approximately equivalent to one year of age in humans). The origin of this switch was related to earlier development of body temperature (BT) rhythms and alteration of psychological behavior rhythms (BD) around earlier feeding times. We propose that this cost-effective, inclusive sensing and analytic technique has value for understanding developmental care conditions for which continual home non-invasive monitoring would be beneficial and further suggest the potential to adapt this technique for use in humans.

Background: We previously reported the nationwide, epidemiological data of acute encephalopathy in Japan during 2007-2010. Here we conducted the second national survey of acute encephalopathy during 2014-2017, and compared the results between the two studies to elucidate the trends in the seven years' interval as well as the influence of changes in pediatric viral infections and guidelines for acute encephalopathy in Japan., Methods: The Research Committee on Acute Encephalopathy supported by the Japanese Government sent a questionnaire to 507 hospitals throughout Japan, and collected the responses by mail., Results: A total of 1115 cases from 267 hospitals reportedly had acute encephalopathy during April 2014-June 2017. In this study, the age at onset was younger, the ratios of recently established syndromes, such as acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) and clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS), were higher, and the ratio of influenza-associated encephalopathy was lower, than in the previous study. The age at onset of influenza-associated encephalopathy was lower, and that of HHV-6/7-associated encephalopathy higher, compared to the first survey. The outcomes of entire acute encephalopathy remained unchanged., Conclusion: Some of these changes reflected the recent trends of viral infectious diseases including 2009 influenza pandemic, and others the standardization of the diagnosis of acute encephalopathy in Japan., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Background: Holoprosencephaly (HPE) is a congenital malformation with an estimated prevalence of 0.10-6.06 per 10 000 births but with no nationwide data specific to Japan., Methods: This nationwide retrospective questionnaire survey was conducted from 2011 to 2013. All 467 training hospitals for perinatal and neonatal care certified by the Japan Society of Perinatal and Neonatal Medicine were contacted. The birth prevalence rate (BPR) was assessed from the primary survey and clinical characteristics from the secondary survey., Results: We received valid responses from 253 hospitals in the primary survey (54.6%). Of 390 342 live births, 60 were diagnosed with HPE (23 males and 37 females), resulting in an actual BPR of 1.54 per 10 000 live births. The point estimate for HPE cases was 100 (95% confidence interval [CI]: 80.7-120), and the estimated BPR of HPE was calculated to be 0.32 per 10 000 live births (95% CI: 0.26-0.38) based on 3 117 853 live births according to Japanese national statistics during the study period. In the secondary survey, we obtained data for 49 cases (19 males and 30 females). Of these, 20 were alobar (40.8%), 20 were semilobar (40.8%), five were lobar (10.4%), and four were of unknown type. Genetic examination was performed in 37 of the 49 HPE patients and revealed that chromosomes 13, 18, and 7 were affected in eight, six, and four patients, respectively., Conclusions: This is the most extensive survey on holoprosencephaly to date in Japan. The estimated BPR was consistent with that reported in previous research., (© 2019 Japan Pediatric Society.)

There has been long taxonomic debate on mulberry species (genus Morus) because the classification of mulberry species has relied on morphological characteristics. Although attempts for classifying mulberry species using molecular markers have been performed, phylogenetic relationships among diploid mulberry species remain unclear. In this study, we aim to investigate the genetic relationship between 54 diploid mulberry varieties belonging to seven different Morus species (M. alba, M. indica, M. bombycis, M. acidosa, M. latifolia, M. kagayamae, and M. rotundiloba) and one unspecified Morus species ('Enbu') using genome-wide SNP discovery and phylogenetic analysis via double-digest restriction site-associated DNA sequencing (ddRAD-seq). Genome-wide 2229 homozygous SNPs of 54 mulberry varieties in the eight species were identified by ddRAD-seq. Results of the phylogenetic analysis identified only three clear monophyletic clades in two Japanese native species, M. acidosa and M. kagayamae, which are found on different geographically isolated islands and a Thai species, M. rotundiloba, whereas the other species were non-monophyletic. Varieties of M. bombycis, another Japanese native species, were roughly classified into three groups. Of these, two M. bombycis groups were monophyletic with M. acidosa and M. kagayamae, respectively, while another M. bombycis group was not monophyletic. Varieties of M. indica, an Indian native species, were classified into two different monophyletic clades. Of these, one clade was clearly monophyletic with an indigenous variety in Kenya, 'Enbu', while another clade was monophyletic with M. rotundiloba and one M. latifolia variety. There were no clear monophyletic clades within M. alba and M. latifolia varieties, which could be a result of several hybridization events after their introductions from China to Japan. Our results suggested that it was difficult to clearly classify the hybridized mulberry varieties even with genome-wide DNA markers. In addition to phylogenetic analysis, we also evaluated morphological characteristics of mulberry leaves for each variety. The results of morphological evaluation indicated that leaf tip ratio may correlate to genetic difference among the two M. bombycis groups in monophyletic clades and another M. bombycis group in non-monophyletic clades. These results suggested that leaf tip ratio might be used for evaluating hybridization of M. bombycis varieties. Over all, our results may provide new insights into taxonomic debate of mulberry species., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: The aim of this study was to evaluate the clinical outcomes of implant-retained obturator rehabilitation after maxillectomy, based on quality of life (QOL) and masticatory function., Study Design: The present study included 12 patients who underwent dental implant surgery after maxillectomy. Oral health-related QOL and masticatory function before and after completion of implant-retained obturator rehabilitation were evaluated., Results: Oral Health Impact Profile-Short Form (OHIP-14) scores showed significant differences in the domain for functional limitation (before, 4.08 ± 2.47; after, 1.33 ± 0.98; P < .01); physical pain (before, 2.75 ± 2.05; after, 0.42 ± 0.51; P < .01); physical disability (before, 3.24 ± 2.11; after, 1.33 ± 1.30; P < .01), psychological disability (before, 3.83 ± 2.48; after, 1.67 ± 2.50; P < .01); social disability (before, 2.17 ± 1.75; after, 1.08 ± 1.38; P < .05); handicap (before, 3.17 ± 1.90; after, 1.08 ± 1.00; P < .01); and total score (before, 22.41 ± 10.17; after, 8.83 ± 6.82; P < .01) Furthermore, masticatory function score was significantly higher after completing implant-retained obturator rehabilitation (before, 38.75 ± 22.97; after, 69.17 ± 21.41; P < .01)., Conclusions: Implant-retained obturator rehabilitation contributed to improved masticatory function and oral health-related QOL after maxillectomy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The catechin content in green tea leaves varies according to cultivation conditions such as intensity of solar radiation, temperature, and precipitation, and thus, there is ambiguity about the best harvest time for obtaining optimal functional effects. In this study, the Yabukita (ordinary) and Benifuki varieties, which contain methylated catechin, were used to determine the difference in green tea catechins according to harvest times and tea manufacturing processes. Caffeine determination was also carried out to provide information about green tea intake for all age-groups of children and pregnant women. Determining the quantity of each catechin was difficult because of degradation, polymerization, and isomerization that had occurred during heat-drying in the refining process. In addition, the absorption of catechin compounds was tested using miniature swine because of their functional and physiological similarity to humans. Benifuki tea leaves contained epigallocatechin-3-(3"-O-methyl) gallate (EGCg3"Me) instead of epigallocatechin-3-(4"-O-methyl) gallate (EGCg4"Me). However, EGCg4"Me was detected during the entire intake period, but EGCg3"Me was not detected in the blood of miniature swine fed Benifuki tea. It is possible that the position of the methyl group was modified by the pig metabolism. Furthermore, caffeine from both Yabukita and Benifuki tea varieties was found to be easily accumulated in miniature swine. These results suggest that nonrefined September-October picking tea (autumn and winter tea) of the Benifuki variety is preferable over the Yabukita variety for consumption by children and pregnant women owing to its lower caffeine content and higher content of methylated catechin., Competing Interests: The authors declare that they do not have any conflict of interest.

Background: Two recent large randomized controlled trials did not show the superiority of high-frequency oscillatory ventilation (HFOV) in adults with ARDS. These two trials had differing results, and possible causes could be the different oscillators used and their different settings, including inspiratory time % (IT%). The aims of this study were to obtain basic data about the ventilation characteristics in two adult oscillators and to elucidate the effect of the oscillator and IT% on ventilation efficiency., Methods: The Metran R100 or SensorMedics 3100B was connected to an original lung model internally equipped with a simulated bronchial tree. The actual stroke volume (aSV) was measured with a flow sensor placed at the Y-piece. Carbon dioxide (CO 2 ) was continuously insufflated into the lung model ([Formula: see text]CO 2 ), and the partial pressure of CO 2 (PCO 2 ) in the lung model was monitored. Alveolar ventilation ([Formula: see text]A; L/min) was estimated as [Formula: see text]CO 2 divided by the stabilized value of PCO 2 . [Formula: see text]A was evaluated with several stroke volume settings in the R100 (IT = 50%) or several airway pressure amplitude settings in the 3100B (IT = 33%, 50%) at a frequency of 6 and 8 Hz, a mean airway pressure of 25 cmH 2 O, and a bias flow of 30 L/min. Assuming that [Formula: see text]A = frequency a  × aSV b , values of a and b were determined. Ventilation efficiency was calculated as [Formula: see text]A divided by actual minute ventilation., Results: The relationship between aSV and [Formula: see text]A or ventilation efficiency were different depending on the oscillator and IT%. The values of a and b were 0 < a < 1 and 1 < b < 2 and were different for three conditions at both frequencies. [Formula: see text]A and ventilation efficiency were highest with R100 (IT = 50%) and lowest with 3100B (IT = 33%) for high aSV ranges at both frequencies., Conclusions: In this lung model study, ventilation characteristics were different depending on the oscillator and IT%. Ventilation efficiency was highest with R100 (IT = 50%) and lowest with 3100B (IT = 33%) for high aSV ranges.

Background: Bias flow (BF) is essential to maintain mean airway pressure (MAP) and to washout carbon dioxide (CO 2 ) from the oscillator circuit during high-frequency oscillatory ventilation (HFOV). If the BF rate is inadequate, substantial CO 2 rebreathing could occur and ventilation efficiency could worsen. With lower ventilation efficiency, the required stroke volume (SV) would increase in order to obtain the same alveolar ventilation with constant frequency. The aim of this study was to assess the effect of BF rate on ventilation efficiency during adult HFOV., Methods: The R100 oscillator (Metran, Japan) was connected to an original lung model internally equipped with a simulated bronchial tree. The actual SV was measured with a flow sensor placed at the Y-piece. Carbon dioxide (CO 2 ) was continuously insufflated into the lung model ([Formula: see text]CO 2 ), and the partial pressure of CO 2 (PCO 2 ) in the lung model was monitored. Alveolar ventilation ([Formula: see text]A) was estimated as [Formula: see text]CO 2 divided by the stabilized value of PCO 2 . [Formula: see text]A was evaluated by setting SV from 80 to 180 mL (10 mL increments, n = 5) at a frequency of 8 Hz, a MAP of 25 cmH 2 O, and a BF of 10, 20, 30, and 40 L/min (study 1). Ventilation efficiency was calculated as [Formula: see text]A divided by the actual minute volume. The experiment was also performed with an actual SV of 80, 100, and 120 mL and a BF from 10 to 60 L/min (10 L/min increments: study 2)., Results: Study 1: With the same setting SV, the [Formula: see text]A with a BF of 20 L/min or more was significantly higher than that with a BF of 10 L/min. Study 2: With the same actual SV, the [Formula: see text]A and the ventilation efficiency with a BF of 30 L/min or more were significantly higher than those with a BF of 10 or 20 L/min., Conclusions: Increasing BF up to 30 L/min or more improved ventilation efficiency in the R100 oscillator.

BACKGROUND Psoriasis is known as the most frequent disease treated by long-term topical steroids. It is also known that patients with thick, chronic plaques require the highest potency topical steroids. However, the treatment is limited to up to four weeks due to risk of systemic absorption. CASE REPORT An 80-year-old man was diagnosed with type 2 diabetes 16 years before, and was being administered insulin combined with alpha glucosidase inhibitor. He was diagnosed with plaque psoriasis and his oral steroid treatment was switched to topical steroid treatment due to lack of improvement and poorly controlled blood glucose level. The hypoglycemic events improved after the psoriatic lesions improved. CONCLUSIONS Control of blood glucose level is difficult at the very beginning of topical steroid treatment for psoriasis especially if a patient is receiving insulin treatment. Intense monitoring of blood glucose level during initiation of topical steroid treatment is necessary to prevent unfavorable complications.

Background: This study aimed to clarify the characteristics of acute encephalopathic episodes in patients with congenital adrenal hyperplasia (CAH), which we termed "CAH-associated encephalopathy (CAHE).", Methods: This retrospective study was conducted using a questionnaire as a nationwide survey of patients with CAH with acute encephalopathy and related episodes., Results: Fifteen patients were recruited on the bases of clinical data that supported a diagnosis of CAHE. Fourteen patients displayed seizures at onset, and 12 patients exhibited refractory seizures. Deep coma lasting >24h was noted in 12 patients. Neuroimaging studies revealed some heterogeneous features. Diffuse or focal edematous lesions in the cerebrum, which produce high signal intensity on diffusion-weighted magnetic resonance imaging or low density on computer tomography, were found in the acute period in all 15 patients. In the chronic period, 14 patients survived, 11 of whom had some degree of neurological sequelae. Moreover, various degrees of cerebral shrinkage were observed in 11 of 14 surviving patients. Surprisingly, there were no abnormal neuroimaging findings in the basal ganglia, brainstem, and cerebellum in any patient., Conclusion: Our results indicated that patients with CAH have a risk of developing CAHE, and thus, they should be followed closely because not only status epilepticus or deep coma but also minor symptoms, such as fever and nausea, may lead to CAHE. Because CAHE may feature some heterogeneous encephalopathic episodes, further validation is needed to clarify its etiology., (Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Neurodevelopmental disorders (NDDs) are highly prevalent and severely debilitating brain illnesses caused by aberrant brain growth and development. Resulting in cognitive, social, motor, language and affective disabilities, common NDDs include autism spectrum disorder (ASD), intellectual disability, communication/speech disorders, motor/tic disorders and attention deficit hyperactivity disorder. Affecting neurogenesis, glia/neuronal proliferation and migration, synapse formation and myelination, aberrant neural development occurs over a substantial period of time. Genetic, epigenetic, and environmental factors play a key role in NDD pathogenesis. Animal models are an indispensable tool to study NDDs. Paralleling clinical findings, we comprehensively evaluate various preclinical tests and models which target key (social, cognitive, motor) neurobehavioral domains of ASD and other common NDDs. Covering both traditional (rodent) and alternative NDD models, we outline the emerging areas of research and emphasize how preclinical models play a key role in gaining translational and mechanistic insights into NDDs and their therapy., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Chloramphenicol (CP) is recently one of the rarely-used antibiotics. In this study, we present four patients with intractable bacterial meningitis, who were successfully treated with CP and discuss the therapeutic indications of CP in these pediatric cases. The patients were diagnosed as bacterial meningitis at the ages ranging from 2 months to 1 year and 4 months. The causative organisms found in three of the patients were H. influenzae and in the fourth patient, S. pneumoniae. According to the microbial sensitivity tests, these organisms were highly sensitive to antibiotics including ceftriaxone, meropenem and/or panipenem/betamipron. Treatment with these antibiotics was initially effective; however, recurrences of meningitis appeared in all patients. Administration of CP (100 mg/kg/day) started between the 11th and the 58th days, and was continued for 9 days up to 19 days. Their fever had disappeared within four days after the administration of CP, and it was confirmed that all patients completely recovered from meningitis. Two of the patients developed a mild degree of anemia, but soon recovered after the discontinuation of CP. None of them had neurological sequela. We recommend CP as one of the choices for the treatment of intractable bacterial meningitis.

Introduction: Neurodevelopmental disorders (NDDs) are common and severely debilitating. Their chronic nature and reliance on both genetic and environmental factors makes studying NDDs and their treatment a challenging task., Areas Covered: Herein, the authors discuss the neurobiological mechanisms of NDDs, and present recommendations on their translational research and therapy, outlined by the International Stress and Behavior Society. Various drugs currently prescribed to treat NDDs also represent a highly diverse group. Acting on various neurotransmitter and physiological systems, these drugs often lack specificity of action, and are commonly used to treat multiple other psychiatric conditions. There has also been relatively little progress in the development of novel medications to treat NDDs. Based on clinical, preclinical and translational models of NDDs, our recommendations cover a wide range of methodological approaches and conceptual strategies., Expert Opinion: To improve pharmacotherapy and drug discovery for NDDs, we need a stronger emphasis on targeting multiple endophenotypes, a better dissection of genetic/epigenetic factors or "hidden heritability," and a careful consideration of potential developmental/trophic roles of brain neurotransmitters. The validity of animal NDD models can be improved through discovery of novel (behavioral, physiological and neuroimaging) biomarkers, applying proper environmental enrichment, widening the spectrum of model organisms, targeting developmental trajectories of NDD-related behaviors and comorbid conditions beyond traditional NDDs. While these recommendations cannot be addressed all in once, our increased understanding of NDD pathobiology may trigger innovative cross-disciplinary research expanding beyond traditional methods and concepts.

Educational treatment to support social development of children with autism spectrum disorder (ASD) is an important topic in developmental psychiatry. However, it remains difficult to objectively quantify the socio-emotional development of ASD children. To address this problem, we developed a novel analytical method that assesses subjects' complex behaviors using multivariate analysis, 'Behavior Output analysis for Quantitative Emotional State Translation' (BOUQUET). Here, we examine the potential for psycho-cognitive ASD therapy based on comparative evaluations of clinical (human) and experimental (animal) models. Our observations of ASD children (vs. their normally developing siblings) and the domestic chick in socio-sensory deprivation models show the importance of unimodal sensory stimulation, particularly important for tactile- and auditory-biased socialization. Identifying psycho-cognitive elements in early neural development, human newborn infants in neonatal intensive care unit as well as a New World monkey, the common marmoset, also prompted us to focus on the development of voluntary movement against gravity. In summary, striking behavioral similarities between children with ASD and domestic chicks' socio-sensory deprivation models support the role of multimodal sensory-motor integration as a prerequisite step for normal development of socio-emotional and psycho-cognitive functions. Data obtained in the common marmoset model also suggest that switching from primitive anti-gravity reflexes to complex voluntary movement may be a critical milestone for psycho-cognitive development. Combining clinical findings with these animal models, and using multivariate integrative analyses may facilitate the development of effective interventions to improve social functions in infants and in children with neurodevelopmental disorders.

We present characteristic magnetic resonance imaging (MRI) features in a pediatric female patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Muscle weakness developed at 8 years old and fluctuated during the clinical course over 7 years. Electrophysiological studies showed a demyelination pattern with moderately delayed nerve conduction velocity, as well as dispersion phenomenon. MRI showed marked changes in thickening of the spinal nerve roots and their peripheral nerves in the lumber and brachial plexuses, as well as in the bilateral trigeminal nerves. It is suggested that these MRI features are characteristic and strongly supportive of the diagnosis of CIDP with a prolonged clinical course., (Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

We report a 4-year-old boy with generalized seizures with abrupt falling and unresponsive staring provoked by somatosensory stimuli of the face. Ictal manifestation comprised a sudden fall immediately after wiping the face, followed by unresponsive staring for around 10 seconds, during which time the video-EEG recorded paroxysmal 3-Hz diffuse spike-wave complexes, which were followed by high-voltage 3.5 to 4-Hz delta wave discharges, predominantly appearing in the bilateral frontal areas. Seizures were not controlled by clonazepam, valproate or levetiracetam, but were successfully treated with lamotrigine added to valproate. This type of generalized reflex seizure has so far not been reported in the literature. [Published with video sequence].

Staphylococcus lugdunensis is a known pathogen of infective endocarditis, but not of urinary tract infection. We report a previously healthy neonate without congenital anomalies of the kidney and urinary tract who developed urinary tract infection due to Staphylococcus lugdunensis, illustrating that Staphylococcus lugdunensis can cause urinary tract infection even in those with no urinary tract complications., (© 2015 Japan Pediatric Society.)

As a diurnal experimental primate, the common marmoset (Callithrix jacchus) has recently contributed to numerous kinds of studies of neurobiological psychiatry as an essential pre-clinical model. The marmoset matures sexually within one or two years after birth. Thus, we can observe how the primate learns and develops psycho-cognitive functions through experiences in experimental environment for a much shorter period compared to that of humans. Longer daylight exposure may affect psychological development of children. In our research, we focus on raising marmosets under constant daylight from birth until various ages. In order to quantitatively evaluate the development of higher-ordered psychological functions, we designed a system of socio-behavioral tests and multivariate correlation analysis methods based on principal component analysis. With reference to the call and typical body movement expressed during a particular social context, we statistically inferred the emotional features of the subjects. In the current literature, we review our published results showing increased alert behaviors by constant light, and then, attempted to extend our additional analysis to seek age-dependent susceptibility to constant light. We then present the neurobiological mechanisms with reference to previous research reports. The current review suggests possible existence of a susceptible period earlier than three to five month-old in the environment-induced developmental disorder model, supposedly like attention deficit hyperactive disorders (ADHD) or oppositional defiant disorder (ODD)., (Copyright © 2014 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.)

Behaviour is one of the most powerful objective signals that connotes psychological functions regulated by neuronal network systems. This study searched for simple behaviours using smartphone sensors with three axes for measuring acceleration, angular speed and direction. We used quantitative analytic methodology of pattern recognition for work contexts, individual workers and seasonal effects in our own longitudinally recorded data. Our 13 laboratory members were involved in the care of common marmosets and domestic chicks, which lived in separate rooms. They attached a smartphone to their front waist-belts during feeding and cleaning in five care tasks. Behavioural characteristics such as speed, acceleration and azimuth, pitch, and roll angles were monitored. Afterwards, participants noted subjective scores of warmth sensation and work efficiency. The multivariate time series behavioral data were characterized by the subjective scores and environmental factors such as room temperature, season, and humidity, using the linear mixed model. In contrast to high-precision but stress-inducing sensors, the mobile sensors measuring daily behaviours allowed us to quantify the effects of the psychological states and environmental factors on the behavioural traits., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Multidimensional diagnosis plays a central role in infant developmental care, which leads to the prediction of future disabilities. Information consolidated from objective and subjective, early and late, central and peripheral data may reveal neuro-pathological mechanisms and realize earlier and more precise preventive intervention. In the current study, we retrospectively searched correlating factors to the following neurological and behavioral development of 'Head Control' and 'Roll Over' using multivariate correlation analysis of differ-ent diagnostic domains over age, subject/object information of the patients who were previously admitted in our neonatal intensive care unit (NICU) and could be developmentally followed up in our outpatient clinic. Based on the hematologic and biochemical data, MRI brain anatomy during NICU hospitalization, we characterized all the acquired data distribution from 31 infants with either 'appeared neurologically normal (ANN, n = 21)’ or 'appeared neurologically abnormal (ANA, n = 10)’ pro tempore, with a physician's clinical judgment before discharge. Besides single factor comparisons between ANN and ANA, we examined their development difference by using the multidimensional information processing, principal component analysis (PCA). The diagnostic predictors of neuro-behavioral development were selected by regression analysis with variable selection. It resulted that hematological and brain anatomical factors seemed correlated to both ‘Head Control’ and ‘Roll Over’. This report suggested certain possibility of the cross-domain translational approach between subjective and objective developmental information through multivariate analyses, with candidate markers preliminarily to be evaluated in further studies., (Copyright © 2014 The Authors. Published by Elsevier B.V.)

Background: Hemostatic resuscitation might improve the survival of severely injured trauma patients. Our objective was to establish a simplified screening system for determining the necessity of massive transfusions (MT) at an early stage in trauma cases., Methods: We retrospectively analyzed the cases of trauma patients who had been transported to our institution between November 2011 and October 2013. Patients who were younger than 18 years of age or who were confirmed to have suffered a cardiac arrest at the scene or on arrival were excluded. MT were defined as transfusions involving the delivery of ≥10 units of red blood cell concentrate within the first 24 h after arrival., Results: A total of 259 trauma patients were included in this study (males: 178, 69%). Their mean age was 49 ± 20, and their median injury severity score was 14.4. Thirty-three (13%) of the patients required MT. The presence of a shock index of ≥1, a base excess of ≤ -3 mmol/L, or a positive focused assessment of sonography for trauma (FAST) result was found to exhibit sensitivity and specificity values of 0.97 and 0.81, respectively, for predicting the necessity of MT. Furthermore, this method displayed an area under the receiver operating characteristic curve of 0.934 (95% confidence interval, 0.891-0.978), which indicated that it was highly accurate., Conclusions: Our screening method based on the shock index, base excess, and FAST result is a simple and useful way of predicting the necessity of MT early after trauma.

Objective: At the Dokkyo Medical University Hospital, we introduced a brain hypothermia therapy protocol for treating childhood status epilepticus and acute encephalitis/encephalopathy in 2004., Patients and Methods: This protocol focuses on infants with a minimum age of six months or 7.5 kg in weight. Applicable diseases include acute encephalitis/encephalopathy occurring from status epilepticus or seizures lasting for 30 minutes or longer, in cases such as near drowning, hypoxic-ischemic encephalopathy, post-resuscitation encephalopathy, cardio-respiratory arrest, severe head injury, or other diagnoses in which the pediatric neurologist recognizes the possibility of neurological complications. Brain hypothermia therapy is managed within the intensive care unit (ICU)., Results: The target body temperature is a bladder or rectum temperature of 34.0 to 35.0 degrees. This body temperature is reduced to the target temperature within six hours of the seizures. Hypothermia is maintained for 48 hours and concomitant steroid pulse therapy may be used at appropriate times. Sodium thiopental is used to sedate and rewarming is carried out at 0.5 degrees per 12 hours. Osmotic diuretics, muscle relaxants and circulatory antagonists may be concomitantly used at appropriate times., Conclusions: This paper reviews the brain hypothermia therapy protocol.

Status epilepticus is defined as epileptic seizure with the duration for over 30 min or clustered seizure without complete recovery of consciousness for 30 min or more. From the management point of view, therapeutic intervention is recommended for prolonged seizures for 5 min or more. Intranasal, buccal or intramuscular administration of midazolam is strongly evidence-based and highly recommended method for the treatment of prolonged seizures before the intravenous access. Diazepam or midazolam is the first choice as initial intravenous treatment. Intravenous bolus administration of fosphenytoin or phenobarbital is the second line treatment. General anesthesia using such as thiopental or propofol is indicated for refractory status epileptic, which should be managed in the intensive care unit.

Unlabelled: The present report describes the application of a miniplate in the hard palate of a 36-year-old patient with a large anterior maxillary defect. The combination of orthodontic elastics with a titanium miniplate improved the stability of the prefabricated prosthesis. This structure retained the ointment gauze covering the wounds and maintained the facial contour. In addition, contracture deformity was prevented by insertion of the prefabricated prosthesis intraoperatively or immediately postoperatively. Furthermore, a soft diet could be ingested immediately postoperatively. The miniplate also supported the anterior part and the definitive prosthesis. This prosthesis restored adequate masticatory, deglutitive, and speech functions and maintained the facial contour with minimum overloading of the remaining teeth., Keywords: Dental rehabilitation; Maxillary defect; Titanium miniplate.

We present here a long survival case of a patient with the mosaic form of trisomy 13 who died of aspiration pneumonia at the age of 7 years and 4 months. The autopsy revealed olfactory aplasia and fenestration of the septum pellucidum, and dilated lateral ventricles and atrophic hippocampus. Furthermore, there were numerous "torpedos" (i.e., swollen fusiform Purkinje cell axons), mostly in the granular layer underneath the Purkinje cell layer, and, occasionally, in the granular layer. Similar neuropathological findings have been reported in elderly cases of essential tremor, Parkinson's disease, or Alzheimer's disease. Precise mechanism for this axonal change is still unclear. These pathological changes have never previously been reported in the literature on trisomy 13, and the present patient is one of the oldest autopsied individuals with the mosaic trisomy 13.

We report herein a 1.5-year-old girl with methylmalonic acidemia (MMA) in whom two missense mutations were found: a novel I739T mutation located in exon 13 and the L494X mutation in exon 8. The results of organic acid test showed a pronounced increase in methylmalonate excretion with increased methylcitrate and 3-OH-propionate excretion, leading to a diagnosis of MMA, and Vitamin B12 administration was started. Analysis of the mut gene confirmed a T-to-A substitution at nucleotide position 1481 in exon 8 and a T-to-C substitution at nucleotide position 2216 in exon 13, leading to the amino acid isoleucine at position 739 being changed to threonine, resulting in c.2216T > C (p.I739T). The patient has now been on high-dose oral administration of Vitamin B12 and carnitine therapy (900 mg of levocarnitine chloride) for 5 years without experiencing further attacks, and her cognitive and motor development is normal. Further tests on residual enzyme activity, as well as experience with more cases, may shed light on the relationship between gene mutations and phenotypes in MMA.

Objective: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a childhood encephalopathy following severe febrile seizures, leaving neurologic sequelae in many patients. However, its pathogenesis remains unclear. In this study, we clarified that genetic variation in the adenosine A2A receptor (ADORA2A), whose activation is involved in excitotoxicity, may be a predisposing factor of AESD., Methods: We analyzed 4 ADORA2A single nucleotide polymorphisms in 85 patients with AESD. The mRNA expression in brain samples, mRNA and protein expression in lymphoblasts, as well as the production of cyclic adenosine monophosphate (cAMP) by lymphoblasts in response to adenosine were compared among ADORA2A diplotypes., Results: Four single nucleotide polymorphisms were completely linked, which resulted in 2 haplotypes, A and B. Haplotype A (C at rs2298383, T at rs5751876, deletion at rs35320474, and C at rs4822492) frequency in patients was significantly higher than in controls (p = 0.005). Homozygous haplotype A (AA diplotype) had a higher risk of developing AESD (odds ratio 2.32, 95% confidence interval 1.32-4.08; p = 0.003) via a recessive model. mRNA expression was significantly higher in AA than AB and BB diplotypes, both in the brain (p = 0.003 and 0.002, respectively) and lymphoblasts (p = 0.035 and 0.003, respectively). In lymphoblasts, ADORA2A protein expression (p = 0.024), as well as cellular cAMP production (p = 0.0006), was significantly higher in AA than BB diplotype., Conclusions: AA diplotype of ADORA2A is associated with AESD and may alter the intracellular adenosine/cAMP cascade, thereby promoting seizures and excitotoxic brain damage in patients.

Purpose: The purpose of this study is to evaluate the postmortem deformation of the aorta on postmortem computed tomography (CT) by comparison with the antemortem CT in the same patient., Materials and Methods: A total of 58 non-traumatic patients without hemorrhagic events who underwent torso CT before and shortly after death were enrolled. Antemortem chest and abdominal CT were obtained in 44 cases and in 57 cases, respectively. The lengths of the major and minor axes of the ascending and descending thoracic aorta and the abdominal aorta were measured on both antemortem and postmortem CT in the same patient. To evaluate the shape of the aorta, the major axis-minor axis ratio (Ma-MiR) was calculated. Mean values of the diameters of the aorta and Ma-MiRs on postmortem CT were compared with those on antemortem CT using the Wilcoxon signed-rank test. We also evaluated the major and minor axes and Ma-MiRs on both antemortem and postmortem CT in two age groups: 65 years and under (n=13) and over 65 years (n=45)., Results: At each level tested, the aorta significantly shrank after death (p<0.001) (ascending thoracic aorta, descending thoracic aorta, and abdominal aorta: 38.5 mm × 33.5 mm, 28.0 mm × 25.9 mm, and 24.4 mm × 21.8 mm on antemortem CT, 30.0 mm × 26.2 mm, 24.4 mm × 20.7 mm, and 21.5 mm × 14.5 mm on postmortem CT, respectively). The postmortem Ma-MiRs significantly increased at the descending thoracic aorta and the abdominal aorta (p<0.001). The diameters of the aorta are longer in older cases at all levels on both antemortem and postmortem CT. The reduction rates were larger in younger cases than older cases at all levels., Conclusions: After death, the aorta shrunk at all levels, and became oval in shape in descending thoracic and abdominal aorta. The contraction was greater in younger cases than older cases. Investigators who interpret postmortem imaging should be aware of the postmortem deformation of the aorta., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Recent progress in affective neuroscience and social neurobiology has been propelled by neuro-imaging technology and epigenetic approach in neurobiology of animal behaviour. However, quantitative measurements of socio-emotional development remains lacking, though sensory-motor development has been extensively studied in terms of digitised imaging analysis. Here, we developed a method for socio-emotional behaviour measurement that is based on the video recordings under well-defined social context using animal models with variously social sensory interaction during development. The behaviour features digitized from the video recordings were visualised in a multivariate statistic space using principal component analysis. The clustering of the behaviour parameters suggested the existence of species- and stage-specific as well as cross-species behaviour modules. These modules were used to characterise the behaviour of children with or without autism spectrum disorders (ASDs). We found that socio-emotional behaviour is highly dependent on social context and the cross-species behaviour modules may predict neurobiological basis of ASDs.

A 73-year-old Japanese man with Hashimoto's disease and diabetes mellitus received regular medical checkups for type 2 diabetes care. Blood tests indicated macrocytic anemia (red blood cell count, 279×104 /μL; hemoglobin, 12.2 g/dL; hematocrit, 34.0%; mean corpuscular volume, 121.9 fL). The laboratory data demonstrated a normal folic acid level with a low vitamin B12 level. An endoscopic examination indicated no signs of gastric or intestinal bleeding. Positive results for anti-intrinsic factor antibodies were strongly suggestive of pernicious anemia. The patient refused cobalamin injections to treat the anemia. However, the oral administration of mecobalamin for the treatment of diabetic neuropathy was simultaneously initiated. Subsequently, the anemia gradually improved. Oral mecobalamin was presumably effective for pernicious anemia management. Anemia is frequently observed in elderly patients, and the incidence of pernicious anemia increases with age. Anemia is conventionally treated with cobalamin injections. Currently, the oral administration of mecobalamin is not the typical treatment for anemia. However, as in our case, a few reports have documented positive results following oral mecobalamin treatment. Moreover, oral mecobalamin is a fairly recent, novel, noninvasive mode of treatment, making it ideal for elderly patients, who are generally frail. This case suggests the efficacy of mecobalamin for the treatment of pernicious anemia.

Attachment formation is the most pivotal factor for humans and animals in the growth and development of social relationships. However, the developmental processes of attachment formation mediated by sensory-motor, emotional, and cognitive integration remain obscure. Here we developed an animal model to understand the types of social interactions that lead to peer-social attachment formation. We found that the social interaction in a sensitive period was essential to stabilise or overwrite the initially imprinted peer affiliation state and that synchronised behaviour with others based on common motivations could be a driver of peer social attachment formation. Furthermore, feeding experience with supplementation of ubiquinol conferred peer social attachment formation even after the sensitive period. Surprisingly, the experience of feeding beyond the cage window was also effective to reduce the required amount ubiquinol, suggesting that peri-personal space modulation may affect socio-emotional cognition and there by lead to attachment formation.

Ring chromosome 14 (r14) is clinically characterized by early-onset epilepsy, mental retardation, delayed speech, microcephaly, extremely mild facial dysmorphisms and ophthalmologic abnormalities. We report a case presenting with partial seizures and delayed development in infancy in which r14 was diagnosed based on chromosomal analysis. The patient was a girl with a normal family and delivery history. Afebrile generalized convulsions developed at age 9 months, and phenobarbital was started, but was changed to zonisamide due to impaired liver function. Chromosome analysis led to a diagnosis of 46, XX, r(14) (p11.2q32.3). At age 5 years, while under treatment with zonisamide and clobazam, epilepsy was characterized by multiple daily episodes of complex partial seizures. Although there are no consistent brain MRI or electroencephalogram findings, experienced pediatric neurologists can make a diagnosis based on facial dysmorphisms. When refractory epilepsy is encountered in infancy with developmental delay of unknown cause, chromosome analysis should be performed.

A research committee supported by the Japanese government conducted a nationwide survey on the epidemiology of acute encephalopathy in Japan using a questionnaire. A total of 983 cases reportedly had acute encephalopathy during the past 3 years, 2007-2010. Among the pathogens of the preceding infection, influenza virus was the most common, followed by human herpesvirus-6 (HHV-6) and rotavirus. Among syndromes of acute encephalopathy, acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was the most frequent, followed by clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS), acute necrotizing encephalopathy (ANE) and hemorrhagic shock and encephalopathy syndrome (HSES). Influenza virus was strongly associated with ANE and MERS, HHV-6 with AESD, and rotavirus with MERS. Mortality was high in ANE and HSES, but was low in AESD, MERS and HHV-6-associated encephalopathy. Neurologic sequelae were common in AESD and ANE, but were absent in MERS., (Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Interferon (IFN)-α was reported to be effective in longterm intrathecal treatment of subacute sclerosing panencephalitis (SSPE). However, the side effects related with longterm use of IFN-α/β are unclear. We evaluated the therapeutic effects of IFN-α/β in a 13-years-old patient with SSPE. The cerebrospinal fluid (CSF) measles antibody titer was 64 × NT/128×HI, IgG-index was 4.5, and the SSPE diagnosis was based on electroencephalography (Jabbour-stage II on admission). With Inosiplex (INP) given orally, IFN-α (3 × 10(6) units) was infused intraventricularly twice-a-week for 1-year. Resultantly, CSF cell count was elevated (2502/3), total protein and glucose levels were normal; however, DIAM occurred repeatedly. Consequently, reduced IFN-α (5 × 10(5) units) with hydrocorton was administered at 2-months interval for 19 months, during which, DIAM occurred four times. Therefore, IFN-β (3 × 10(6) units; twice-a-week) therapy was started and continued for 3 years. Although the symptoms were improved considerably, DIAM recurred after 15-months therapy and CSF cell counts were also elevated (2121/3). Since SSPE progressed to Jabbour-stage IV, indicated by irreversible consciousness disorder, IFN therapy was discontinued and INP monotherapy was followed for another 3 years. We, therefore, concluded that the longterm intraventricular IFN-α/β infusion therapy of SSPE involved the potential risk of DIAM with serious irreversible neurological sequelae and should be monitored carefully.

Background: Acute encephalopathy/encephalitis is one of the most important causatives of mortality and neurological deficit during childhood. The aim of this retrospective observational study was to investigate clinical variables and therapeutic options associated with the outcome of children with acute encephalopathy/encephalitis., Methods: Relationships between the clinical information at admission and the neurological outcome evaluated using Pediatric Cerebral Performance Category Scale (PCPC) at 12 months after admission were assessed in 43 patients who were treated at 10 Japanese paediatric intensive care units., Results: Sixteen patients were cared for at normothermia, whereas mild hypothermia was applied to 27 children. In univariate analysis, ages ≤ 18 months, marked elevation in serum lactate dehydrogenase (LD) and aspartate transaminase, diagnosis of either acute necrotising encephalopathy or haemorrhagic shock and encephalopathy syndrome and longer hypothermic periods were associated with increased risks of death or severe neurological deficit, whereas hypothermia showed pivotal effects: the outcome of children cooled after 12 h of diagnosis was statistically invariant with normothermic children, but was significantly worse compared with children cooled ≤ 12 h. In multivariate analysis, younger ages and elevated serum LD were associated with adverse outcomes, whereas early initiation of cooling was related to favourable outcomes. For normothermic children, PCPC scores were dependent on the computed tomographic findings suggestive of cerebral oedema, serum LD levels and Glasgow Coma Scale at admission. For hypothermic children, PCPC scores depended on longer delays in cooling initiation., Conclusion: Without therapeutic hypothermia, the outcome of children was determined by variables suggestive of the severity of encephalopathy/encephalitis at admission. Hypothermia may have pivotal impacts on the outcome of children according to the timing of cooling initiation following acute encephalopathy/encephalitis.

A newborn baby boy presented with giant melanocytic nevi on the face, trunk and extremities, and focal cortical dysplasia on MRI. At 3 months of age, he developed intractable epilepsy, and MRI at 2 years of age revealed a high-intensity area in the bilateral cerebellum on T1-weighted images, indicative of melanosis. Based on the findings of the skin and MRI, we diagnosed the boy with neurocutaneous melanosis. Cytodiagnosis of cerebrospinal fluid showed no malignancies. EEG, magnetoencephalogram and ECD-SPECT indicated that the clonic seizures originated from a focus in the right focal cortical dysplasia. Complications also included sebaceous nevus of the head and face, which was characteristic of sebaceous nevus syndrome, lipoma of the face and cauda equina, and limbal dermoid. Sebaceous nevus syndrome may have been due to certain allelic defects that were independent of those for neurocutaneous melanosis.

The high incidence of acute encephalopathy in East Asia suggests the role of genetic factors in its pathogenesis. It has recently been reported that variations of the CPT II (carnitine palmitoyl transferase II) gene may be associated with fatal or severe cases of influenza-associated encephalopathy. In the present study, we examined the genotype of CPT II in cases of acute encephalopathy associated with various preceding infections. Twenty-nine Japanese patients with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) or acute necrotizing encephalopathy (ANE) were studied. The frequency of F352C of CPT II exon 4 was significantly higher in patients than in controls. All patients who had allele C in F352C had allele I in V368I and allele M in M647V (CIM haplotype), which reportedly decreases CPT II activity to one third of that with FIM or FVM haplotype. The frequency of CIM haplotype was significantly different between patients and controls, but not between AESD and ANE. Our results revealed that having at least one CIM allele is a risk factor for the onset of acute encephalopathy, regardless of its antecedent infections., (Copyright © 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Acute encephalopathy in childhood is a wide and heterogenous neurological category, composed of several syndromes or diseases yet to be fully established. Recent progresses in diagnostic radiology and laboratory tests has allowed us to elucidate the nature of this huge entity. Acute encephalopathy with febrile convulsive status epilepticus (AEFCSE) is the most common type of acute encephalopathy in childhood in Japan, which develops with prolonged febrile convulsion, followed by mild unconsciousness, then subsequently provoking a cluster of convulsions (late seizures) with comatous state. Diffusion-weighted MR images shows high signal in the subcortical white matter as with cerebral lobar distribution pattern. "Acute infantile encephalopathy predominantly affecting the frontal lobes (AIEF)" is the most common subtype of AEFCSE, which is unique in that frontal lobes are the primary focus for the neurological manifestations and radiological features.

The infiltration of 'primed' polymorphonuclear neutrophils into multiple organs has been reported in cases of traumatic or hemorrhagic shock. Since multiple injuries are usually observed in cases of physical abuse of the elderly, we investigated neutrophil infiltration into the heart, lung, liver and kidney in cases of abused elderly individuals using immunohistochemistry for myeloperoxidase (MPO). In addition, we examined the expression of molecules associated with neutrophil infiltration, including P-selectin as the adhesion molecule and IL-8 as the chemotactic factor. The number of neutrophils in the physically abused elder cases was increased significantly, particularly in the lung and liver, compared with that of control cases of sharp instrument injury, single fatal blunt injury and polytrauma. In addition, P-selection expression in the endothelium and the presence of IL-8-positive cells (mainly macrophages) in the lung and liver of abuse cases were significantly greater than those in control cases. In contrast, the number of MPO-, P-selectin- and IL-8-positive cells in cases of multiple organ failure (MOF) due to various causes was significantly greater than that in abuse cases. It is known that primed neutrophils accumulation may undergo MOF by 'activation' due to secondary insults. Thus, our results suggest that MPO immunostaining can distinguish cases of elderly physical abuse from non-abuse and MOF cases. In addition, our results indicate that MPO is a potential diagnostic marker for elder physical abuse, and that P-selectin and IL-8 may be useful for a more accurate diagnosis. Finally, our results also suggest that elder cases of physical abuse may be in a primed stage of MOF, and are at risk of falling into MOF by various secondary insults including those following abuse., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)