Updated on 2023/04/18

写真a

 
KIDOKORO Hiroyuki
 
Organization
Nagoya University Hospital Pediatrics Lecturer
Graduate School
Graduate School of Medicine
Title
Lecturer

Degree 1

  1. Doctor(Medicine) ( 2010.3   Nagoya University ) 

Research Interests 4

  1. Neuroscience

  2. Neonatal Neurology

  3. Pediatric Neurology

  4. CaMK

Research Areas 2

  1. Life Science / Neuroscience-general

  2. Life Science / Embryonic medicine and pediatrics  / 小児神経学

Research History 1

  1. Nagoya University Hospital   Pediatrics

    2014

Education 2

  1. Nagoya University   Graduate School, Division of Medical Sciences

    - 2010.3

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    Country: Japan

  2. Nagoya University   Faculty of Medicine

    1992.4 - 1998.3

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    Country: Japan

Professional Memberships 8

  1. 日本小児科学会

  2. 日本小児神経学会   評議員

  3. 日本てんかん学会

  4. 日本周産期新生児医学会

  5. 日本新生児成育医学会

  6. 日本神経科学会

  7. Infantile Seizure Society

  8. Newborn Brain Society

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Committee Memberships 1

  1. 日本小児神経学会   教育委員会  

    2022.1   

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    Committee type:Academic society

    教育委員長

 

Papers 62

  1. Impact of maternal hypertensive disorders of pregnancy on brain volumes at term-equivalent age in preterm infants: A voxel-based morphometry study. International journal

    Takafumi Ushida, Hiroyuki Kidokoro, Noriyuki Nakamura, Satoru Katsuki, Kenji Imai, Tomoko Nakano-Kobayashi, Yoshinori Moriyama, Yoshiaki Sato, Masahiro Hayakawa, Jun Natsume, Hiroaki Kajiyama, Tomomi Kotani

    Pregnancy hypertension   Vol. 25   page: 143 - 149   2021.8

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    Language:Japanese   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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  2. Repetitive sleep starts: An important differential diagnosis of infantile spasms. International journal

    Yuki Maki, Hiroyuki Kidokoro, Akihisa Okumura, Hiroyuki Yamamoto, Tomohiko Nakata, Tatsuya Fukasawa, Tetsuo Kubota, Masahiro Kawaguchi, Takeshi Suzuki, Masaharu Tanaka, Yu Okai, Yoko Sakaguchi, Atsuko Ohno, Tamiko Negoro, Yoshiyuki Takahashi, Jun Natsume

    Epilepsy & behavior : E&B   Vol. 121 ( Pt A ) page: 108075 - 108075   2021.8

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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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  3. Age estimates from brain magnetic resonance images of children younger than two years of age using deep learning. International journal

    Masahiro Kawaguchi, Hiroyuki Kidokoro, Rintaro Ito, Anna Shiraki, Takeshi Suzuki, Yuki Maki, Masaharu Tanaka, Yoko Sakaguchi, Hiroyuki Yamamoto, Yosiyuki Takahashi, Shinji Naganawa, Jun Natsume

    Magnetic resonance imaging   Vol. 79   page: 38 - 44   2021.6

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    Authorship:Corresponding author   Language:Japanese   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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  4. MRI findings in children with congenital cytomegalovirus infection retrospectively diagnosed with dried umbilical cord Reviewed International journal

    Hiroyuki Kidokoro, Anna Shiraki, Yuka Torii, Masaharu Tanaka, Hiroyuki Yamamoto, Hirokazu Kurahashi, Koichi Maruyama, Akihisa Okumura, Jun Natsume, Yoshinori Ito

    Neuroradiology   Vol. 63 ( 5 ) page: 761 - 768   2021.5

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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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  5. The eldest case of MICPCH with CASK mutation exhibiting gross motor regression. Reviewed International journal

    Yosuke Nishio, Hiroyuki Kidokoro, Toshiki Takeo, Hajime Narita, Fumi Sawamura, Kotaro Narita, Yoshihiko Kawano, Tomohiko Nakata, Hideki Muramatsu, Shinya Hara, Tadashi Kaname, Jun Natsume

    Brain & development   Vol. 43 ( 3 ) page: 459 - 463   2021.3

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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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  6. Delta Brushes Are Not Just a Hallmark of EEG in Human Preterm Infants. Reviewed International journal

    Hiroyuki Kidokoro

    Pediatrics international : official journal of the Japan Pediatric Society   Vol. 63 ( 2 ) page: 130 - 136   2021.2

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    Authorship:Lead author, Corresponding author   Language:Japanese   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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  7. Acute Necrotizing Encephalopathy: A Disease Meriting Greater Recognition

    Hiroyuki Kidokoro

    American Journal of Neuroradiology   Vol. 41 ( 12 ) page: 2255 - 2256   2020.12

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

    File: ajnr.A6821.full.pdf

    DOI: 10.3174/ajnr.A6821

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  8. Effect of spontaneous electrical activity on the developing cortex. Invited Reviewed International journal

    Hiroyuki Kidokoro

    Pediatrics international : official journal of the Japan Pediatric Society   Vol. 62 ( 10 ) page: 1131 - 1132   2020.10

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

    File: ped.14407.pdf

    DOI: 10.1111/ped.14407

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  9. High-amplitude fast activity in EEG: An early diagnostic marker in children with beta-propeller protein-associated neurodegeneration (BPAN) Reviewed

    Hiroyuki Kidokoro, Hiroyuki Yamamoto, Tetsuo Kubota, Mitsuo Motobayashi, Yusaku Miyamoto, Tomohiko Nakata, Kyoko Takano, Naoko Shiba, Yu Okai, Masaharu Tanaka, Yoko Sakaguchi, Yuki Maki, Masahiro Kawaguchi, Takeshi Suzuki, Kazuhiro Muramatsu, Jun Natsume

    Clinical Neurophysiology   Vol. 131 ( 9 ) page: 2100 - 2104   2020.9

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    Language:Japanese   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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  10. Thirteen-Year Outcomes in Very Preterm Children Associated with Diffuse Excessive High Signal Intensity on Neonatal Magnetic Resonance Imaging Reviewed

    Ines M. Mürner-Lavanchy, Hiroyuki Kidokoro, Deanne K. Thompson, Lex W. Doyle, Jeanie L.Y. Cheong, Rod W. Hunt, Terrie E. Inder, Peter J. Anderson

    The Journal of Pediatrics   Vol. 206   page: 66 - +   2019.3

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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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  11. Gait performance and dual-task costs in school-aged children with Down syndrome. International journal

    Yuji Ito, Tadashi Ito, Atsuko Ohno, Tetsuo Kubota, Kaori Tanemura, Sho Narahara, Erina Kataoka, Reina Hyodo, Yuichiro Sugiyama, Tetsuo Hattori, Hiroyuki Kidokoro, Hideshi Sugiura, Koji Noritake, Jun Natsume, Nobuhiko Ochi

    Brain & development   Vol. 45 ( 3 ) page: 171 - 178   2023.3

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    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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  12. Pathological gait in Rett syndrome: Quantitative evaluation using three-dimensional gait analysis. International journal

    Takeshi Suzuki, Yuji Ito, Tadashi Ito, Hiroyuki Kidokoro, Koji Noritake, Keita Tsujimura, Shinji Saitoh, Hiroyuki Yamamoto, Nobuhiko Ochi, Naoko Ishihara, Izumi Yasui, Hideshi Sugiura, Tomohiko Nakata, Jun Natsume

    European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society   Vol. 42   page: 15 - 21   2023.1

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    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

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  13. Virus and child neurology

    Kumada Satoko, Kidokoro Hiroyuki

    NO TO HATTATSU   Vol. 55 ( 1 ) page: 64 - 65   2023

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

    DOI: 10.11251/ojjscn.55.64

    CiNii Research

  14. Impact of the Coronavirus Disease 2019 Pandemic on the Clinical Features of Pediatric Respiratory Syncytial Virus Infection in Japan

    Ozeki Shoko, Kawada Jun-Ichi, Yamashita Daiki, Yasufuku Chika, Akano Takuya, Kato Masahiro, Suzuki Konomi, Tano Chihiro, Matsumoto Kazuki, Mizutani Shu-Hei, Mori Ayumi, Nishio Nobuhiro, Kidokoro Hiroyuki, Yasui Yoshihiro, Takahashi Yoshiyuki, Sato Yoshiaki

    OPEN FORUM INFECTIOUS DISEASES   Vol. 9 ( 11 ) page: ofac562   2022.11

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

    DOI: 10.1093/ofid/ofac562

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  15. Involvement of the Thalamus, Hippocampus, and Brainstem in Hypsarrhythmia of West Syndrome: Simultaneous Recordings of Electroencephalography and fMRI Study. International journal

    Y Maki, J Natsume, Y Ito, Y Okai, E Bagarinao, H Yamamoto, S Ogaya, T Takeuchi, T Fukasawa, F Sawamura, T Mitsumatsu, S Maesawa, R Saito, Y Takahashi, H Kidokoro

    AJNR. American journal of neuroradiology   Vol. 43 ( 10 ) page: 1502 - 1507   2022.10

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    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

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  16. Forster resonance energy transfer-based kinase mutation phenotyping reveals an aberrant facilitation of Ca2+/calmodulin-dependent CaMKII alpha activity in de novo mutations related to intellectual disability

    Fujii Hajime, Kidokoro Hiroyuki, Kondo Yayoi, Kawaguchi Masahiro, Horigane Shin-ichiro, Natsume Jun, Takemoto-Kimura Sayaka, Bito Haruhiko

    FRONTIERS IN MOLECULAR NEUROSCIENCE   Vol. 15   page: 970031   2022.9

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

    DOI: 10.3389/fnmol.2022.970031

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  17. Whole-exome analysis of 177 pediatric patients with undiagnosed diseases

    Narita Kotaro, Muramatsu Hideki, Narumi Satoshi, Nakamura Yuji, Okuno Yusuke, Suzuki Kyogo, Hamada Motoharu, Yamaguchi Naoya, Suzuki Atsushi, Nishio Yosuke, Shiraki Anna, Yamamori Ayako, Tsumura Yusuke, Sawamura Fumi, Kawaguchi Masahiro, Wakamatsu Manabu, Kataoka Shinsuke, Kato Kohji, Asada Hideyuki, Kubota Tetsuo, Muramatsu Yukako, Kidokoro Hiroyuki, Natsume Jun, Mizuno Seiji, Nakata Tomohiko, Inagaki Hidehito, Ishihara Naoko, Yonekawa Takahiro, Okumura Akihisa, Ogi Tomoo, Kojima Seiji, Kaname Tadashi, Hasegawa Tomonobu, Saitoh Shinji, Takahashi Yoshiyuki

    SCIENTIFIC REPORTS   Vol. 12 ( 1 ) page: 14589   2022.8

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

    DOI: 10.1038/s41598-022-14161-6

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  18. Quantitative Three-Dimensional Gait Evaluation in Patients With Glucose Transporter 1 Deficiency Syndrome. International journal

    Takeshi Suzuki, Yuji Ito, Tadashi Ito, Hiroyuki Kidokoro, Koji Noritake, Ayako Hattori, Shin Nabatame, Jun Natsume

    Pediatric neurology   Vol. 132   page: 23 - 26   2022.7

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    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

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  19. Acute encephalopathy with biphasic seizures and late reduced diffusion: Predictive EEG findings. International journal

    Atsuko Ohno, Akihisa Okumura, Tatsuya Fukasawa, Tomohiko Nakata, Motomasa Suzuki, Masaharu Tanaka, Yu Okaia, Yuji Ito, Hiroyuki Yamamoto, Takeshi Tsuji, Hiroyuki Kidokoro, Shinji Saitoh, Jun Natsume

    Brain & development   Vol. 44 ( 3 ) page: 221 - 228   2022.3

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    Language:Japanese   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

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  20. NF1関連悪性末梢神経鞘腫瘍の予後改善をめざした科横断的診療体制の確立と運用

    西田 佳弘, 生田 国大, 夏目 敦至, 森川 真紀, 城所 博之, 野々部 典枝, 武市 拓也, 神戸 未来, 尾崎 紀夫, 今釜 史郎

    日本整形外科学会雑誌   Vol. 96 ( 3 ) page: S602 - S602   2022.3

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

  21. Involvement of brain structures in childhood epilepsy with centrotemporal spikes. International journal

    Yuji Ito, Yuki Maki, Yu Okai, Hiroyuki Kidokoro, Epifanio Bagarinao, Tomoya Takeuchi, Atsuko Ohno, Tomohiko Nakata, Naoko Ishihara, Akihisa Okumura, Hiroyuki Yamamoto, Satoshi Maesawa, Jun Natsume

    Pediatrics international : official journal of the Japan Pediatric Society   Vol. 64 ( 1 ) page: e15001   2022.1

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    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.

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  22. Proceedings of the 13th International Newborn Brain Conference: Fetal and/or neonatal brain development, both normal and abnormal. International journal

    Khadar Abdi, Ramy Abramsky, Nickie Andescavage, Jephté Bambi, Sudeepta Basu, Cynthia Bearer, Eric J Benner, Thérèse Biselele, Nikolay Bliznyuk, Jeroen Breckpot, Galen Carey, Agnes Chao, Line Iadsatian Christiansen, Silvia Comani, Pierpaolo Croce, Maarten De Vos, Anneleen Dereymaeker, Laura Dubois, Amelia J Eisch, Adrian Epstein, Neta Geva, Yael Geva, Marc Gewillig, Sheyenne Gillis, Ronald N Goldberg, Magnus Gram, Simon Gregory, Danielle Guez-Barber, Masahiro Hayakawa, Nicole Lind Henriksen, Tim Hermans, Reli Hershkovitz, Kristine Holgersen, Bo Holmqvist, Vaibhav Jain, Katrien Jansen, Vinay Kandula, Kushal Kapse, Masahiro Kawaguchi, Abdulhafeez Khair, Mohammad Khazaei, Hiroyuki Kidokoro, Frederico C Kiffer, Katherine Kisilewicz, Sumire Kumai, Helene Lacaille, David Ley, Catherine Limperopoulos, Sandy Ebba Hallengreen Lindholm, Prosper Lukusa, Rebecca Lundberg, Peter MacFarlane, Pavle Matak, Laetitia Mavinga, Catherine Mayer, Gloire Mbayabo, Takamasa Mitsumatsu, Gerrye Mubungu, Jonathan Murnick, Tomohiko Nakata, Hajime Narita, Parvathi Nataraj, Jun Natsume, Gunnar Naulaers, Rahul Nikam, Niklas Ortenlöf, Katherine Ottolini, Xiaoyu Pan, Stanislava Pankratova, Kelly Pegram, Anna A Penn, Subechhya Pradhan, Khadijeh Raeisi, Nicholas Rickman, Blaire Rikard, Reut Rotem, Per Torp Sangild, Yoshiaki Sato, Fumi Sawamura, Eilon Shany, Ilan Shelef, Anna Shiraki, Laura Smets, Livia Sura, Ryosuke Suzui, Takeshi Suzuki, Bruno-Paul Tady, Gentaro Taga, Gabriella Tamburro, Liesbeth Thewissen, J Will Thompson, Thomas Thymann, Cansu Tokat, Claire-Marie Vacher, Cyndi Valdes, Suvi Vallius, Sergei Vatolin, Hama Watanabe, Adi Yehuda Weintraub, Michael Weiss, Hiroyuki Yamamoto, Salem Shimrit Yaniv, Noelle Younge, Sanghee Yun, Filippo Zappasodi

    Journal of neonatal-perinatal medicine   Vol. 15 ( 2 ) page: 411 - 426   2022

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    DOI: 10.3233/NPM-229002

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  23. Effectiveness of lacosamide in children and young adults previously treated with other sodium channel blockers

    Suzuki Takeshi, Natsume Jun, Kumai Sumire, Maki Yuki, Yamamoto Hiroyuki, Numoto Shingo, Narahara Sho, Kubota Tetsuo, Tsuji Takeshi, Kato Toru, Yamada Keitaro, Maruyama Koichi, Okumura Akihisa, Takahashi Yoshiyuki, Kidokoro Hiroyuki

    EPILEPSY & BEHAVIOR   Vol. 125   page: 108397   2021.12

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  24. Physical functions and gait performance in school-aged children born late preterm

    Yuji Ito, Tadashi Ito, Hideshi Sugiura, Hiroyuki Kidokoro, Yuichiro Sugiyama, Jun Mizusawa, Jun Natsume, Koji Noritake, Yuichi Kato, Nobuhiko Ochi

    Early Human Development   Vol. 163   page: 105478 - 105478   2021.12

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    DOI: 10.1016/j.earlhumdev.2021.105478

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  25. Hypertensive disorders of pregnancy and alterations in brain metabolites in preterm infants: A multi-voxel proton MR spectroscopy study. International journal

    Satoru Katsuki, Takafumi Ushida, Hiroyuki Kidokoro, Noriyuki Nakamura, Yukako Iitani, Kazuya Fuma, Kenji Imai, Tomoko Nakano-Kobayashi, Yoshiaki Sato, Masahiro Hayakawa, Jun Natsume, Hiroaki Kajiyama, Tomomi Kotani

    Early human development   Vol. 163   page: 105479 - 105479   2021.12

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    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.

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  26. Trajectory of the incidence of brushes on preterm electroencephalogram and its association with neurodevelopment in extremely low birth weight infants. International journal

    Takashi Maeda, Hiroyuki Kidokoro, Takashi Tachibana, Anna Shiraki, Hiroyuki Yamamoto, Tomohiko Nakata, Tatsuya Fukasawa, Tetsuo Kubota, Yoshiaki Sato, Toru Kato, Jun Natsume, Akihisa Okumura, Masahiro Hayakawa

    Brain & development   Vol. 43 ( 10 ) page: 979 - 987   2021.11

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    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.

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  27. Bilateral asymmetry in the gait deviation index in school-aged children with the trait of developmental coordination disorder. International journal

    Tadashi Ito, Yuji Ito, Akio Nakai, Hideshi Sugiura, Koji Noritake, Hiroyuki Kidokoro, Jun Natsume, Nobuhiko Ochi

    Gait & posture   Vol. 88   page: 174 - 179   2021.7

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    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.

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  28. Establishment of in-hospital clinical network for patients with neurofibromatosis type 1 in Nagoya University Hospital. International journal

    Yoshihiro Nishida, Kunihiro Ikuta, Atsushi Natsume, Naoko Ishihara, Maki Morikawa, Hiroyuki Kidokoro, Yukako Muramatsu, Norie Nonobe, Kanako Ishizuka, Takuya Takeichi, Miki Kanbe, Seiji Mizuno, Shiro Imagama, Norio Ozaki

    Scientific reports   Vol. 11 ( 1 ) page: 11933 - 11933   2021.6

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    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.

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  29. 【臨床脳波の現状と未来:clinical toolかresearch topicか?】小児の脳機能評価におけるresearchおよびclinical toolとしての脳波

    夏目 淳, 伊藤 祐史, 白木 杏奈, 城所 博之

    臨床神経生理学   Vol. 49 ( 3 ) page: 141 - 144   2021.6

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    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)

    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

  30. Risks of ACTH therapy for West syndrome following BCG vaccination. International journal

    Yuki Maki, Jun Natsume, Ikumi Hori, Tomoya Takeuchi, Yutaka Negishi, Tetsuo Kubota, Koichi Maruyama, Tomohiko Nakata, Hiroyuki Yamamoto, Masaharu Tanaka, Masahiro Kawaguchi, Takeshi Suzuki, Anna Shiraki, Fumi Sawamura, Hiroyuki Kidokoro

    Epilepsy & behavior : E&B   Vol. 118   page: 107924 - 107924   2021.5

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    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.

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  31. Initial treatment of seizures in children in an emergency department in rural Japan. Reviewed International journal

    Anna Shiraki, Masahiro Yasui, Hiroyuki Kidokoro, Shinji Kido, Hideo Ando, Yoshiyuki Takahashi, Jun Natsume

    Brain & development   Vol. 43 ( 2 ) page: 288 - 293   2021.2

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    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.

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  32. Shuffling babies and autism spectrum disorder. Reviewed International journal

    Yu Okai, Tomohiko Nakata, Kiyokuni Miura, Atsuko Ohno, Rie Wakako, Osamu Takahashi, Yuki Maki, Masaharu Tanaka, Yoko Sakaguchi, Yuji Ito, Hiroyuki Yamamoto, Hiroyuki Kidokoro, Yoshiyuki Takahashi, Jun Natsume

    Brain & development   Vol. 43 ( 2 ) page: 181 - 185   2021.2

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    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.

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  33. Lenticular nuclei to thalamic ratio on PET is useful for diagnosis of GLUT1 deficiency syndrome. Reviewed International journal

    Jun Natsume, Naoko Ishihara, Yoshiteru Azuma, Tomohiko Nakata, Tomoya Takeuchi, Masaharu Tanaka, Yoko Sakaguchi, Yu Okai, Yuji Ito, Hiroyuki Yamamoto, Atsuko Ohno, Hiroyuki Kidokoro, Ayako Hattori, Shin Nabatame, Katsuhiko Kato

    Brain & development   Vol. 43 ( 1 ) page: 69 - 77   2021.1

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    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.

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  34. Gait characteristics of children with Williams syndrome with impaired visuospatial recognition: a three-dimensional gait analysis study. Reviewed International journal

    Yuji Ito, Tadashi Ito, Naoko Kurahashi, Nobuhiko Ochi, Koji Noritake, Hideshi Sugiura, Seiji Mizuno, Hiroyuki Kidokoro, Jun Natsume, Miho Nakamura

    Experimental brain research   Vol. 238 ( 12 ) page: 2887 - 2895   2020.12

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    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.

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  35. Change of White Matter Integrity in Children With Hematopoietic Stem Cell Transplantation. Reviewed International journal

    Yoko Sakaguchi, Jun Natsume, Hiroyuki Kidokoro, Masaharu Tanaka, Yu Okai, Yuji Ito, Hiroyuki Yamamoto, Atsuko Ohno, Tomohiko Nakata, Toshiki Nakane, Hisashi Kawai, Toshiaki Taoka, Hideki Muramatsu, Shinji Naganawa, Yoshiyuki Takahashi

    Pediatric neurology   Vol. 111   page: 78 - 84   2020.10

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    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.

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  36. Evaluation of interobserver variability in application of the new neonatal seizure classification proposed by the ILAE Task Force. Reviewed International journal

    Tetsuo Kubota, Hiroyuki Kidokoro, Sho Narahara, Tatsuya Fukasawa, Tomohiko Nakata, Jun Natsume, Akihisa Okumura

    Epilepsy & behavior : E&B   Vol. 111   page: 107292 - 107292   2020.10

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    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.

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  37. Splenial Lesions in Benign Convulsions With Gastroenteritis Associated With Rotavirus Infection. Reviewed International journal

    Chikako Ogawa, Hiroyuki Kidokoro, Naoko Ishihara, Takeshi Tsuji, Hirokazu Kurahashi, Ayako Hattori, Motomasa Suzuki, Shunsuke Ogaya, Yuji Ito, Tatsuya Fukasawa, Tetsuo Kubota, Akihisa Okumura, Shinji Saitoh, Jun Natsume

    Pediatric neurology   Vol. 109   page: 79 - 84   2020.8

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    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.

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  38. Transient cortical diffusion restriction in children immediately after prolonged febrile seizures. Reviewed International journal

    Takeshi Suzuki, Hiroyuki Kidokoro, Tetsuo Kubota, Tatsuya Fukasawa, Ryosuke Suzui, Takeshi Tsuji, Toru Kato, Hiroyuki Yamamoto, Atsuko Ohno, Tomohiko Nakata, Shinji Saitoh, Akihisa Okumura, Jun Natsume

    European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society   Vol. 27   page: 30 - 36   2020.7

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    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.

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  39. Clinical findings in patients with febrile seizure after 5 years of age: A retrospective study. Reviewed International journal

    Motoko Ogino, Mitsuru Kashiwagi, Takuya Tanabe, Chizu Oba, Shohei Nomura, Shuichi Shimakawa, Hiroyuki Kidokoro, Jun Natsume, Akihisa Okumura, Hiroshi Tamai, Akira Ashida

    Brain & development   Vol. 42 ( 6 ) page: 449 - 456   2020.6

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    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.

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  40. Biallelic VPS35L pathogenic variants cause 3C/Ritscher-Schinzel-like syndrome through dysfunction of retriever complex Reviewed

    Kohji Kato, Yasuyoshi Oka, Hideki Muramatsu, Filipp F Vasilev, Takanobu Otomo, Hisashi Oishi, Yoshihiko Kawano, Hiroyuki Kidokoro, Yuka Nakazawa, Tomoo Ogi, Yoshiyuki Takahashi, Shinji Saitoh

    Journal of Medical Genetics   Vol. 57 ( 4 ) page: 245 - 253   2020.4

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    <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<sup>−/−</sup></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>

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  41. Subsecond EEG-fMRI analysis for presurgical evaluation in focal epilepsy. Reviewed International journal

    Yuji Ito, Satoshi Maesawa, Epifanio Bagarinao, Yu Okai, Daisuke Nakatsubo, Hiroyuki Yamamoto, Hiroyuki Kidokoro, Naotaka Usui, Jun Natsume, Minoru Hoshiyama, Toshihiko Wakabayashi, Gen Sobue, Norio Ozaki

    Journal of neurosurgery   Vol. 134 ( 4 ) page: 1027 - 1036   2020.4

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    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.

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  42. Pseudo-sawtooth pattern on amplitude-integrated electroencephalography in neonatal hypoxic-ischemic encephalopathy. Reviewed International journal

    Masaharu Tanaka, Hiroyuki Kidokoro, Tetsuo Kubota, Tatsuya Fukasawa, Yu Okai, Yoko Sakaguchi, Yuji Ito, Hiroyuki Yamamoto, Atsuko Ohno, Tomohiko Nakata, Tamiko Negoro, Akihisa Okumura, Toru Kato, Kazuyoshi Watanabe, Yoshiyuki Takahashi, Jun Natsume

    Pediatric research   Vol. 87 ( 3 ) page: 529 - 535   2020.2

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    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.

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

  43. Novel biallelic FA2H mutations in a Japanese boy with fatty acid hydroxylase-associated neurodegeneration Reviewed

    Masahiro Kawaguchi, Takayuki Sassa, Hiroyuki Kidokoro, Tomohiko Nakata, Kohji Kato, Hideki Muramatsu, Yusuke Okuno, Hiroyuki Yamamoto, Tadashi Kaname, Akio Kihara, Jun Natsume

    Brain and Development   Vol. 42 ( 2 ) page: 217 - 221   2020.2

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    DOI: 10.1016/j.braindev.2019.11.006

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  44. A Case of a 14-Year-Old Boy who had Difficulty Walking Due to Drug-Induced Neuropathy During T-Cell Acute Lymphocytic Leukemia Treatment

    Yoshida Taro, Sawamura Aya, Muramatsu Hideki, Imaya Masayuki, Yamamori Ayako, Wakamatsu Manabu, Miwata Shunsuke, Narita Kotaro, Kitazawa Hironobu, Taniguchi Rieko, Ichikawa Daisuke, Nishikawa Eri, Hamada Motoharu, Kawashima Nozomu, Narita Atsushi, Nishio Nobuhiro, Kojima Seiji, Nakata Tomohiko, Kidokoro Hiroyuki, Natxume Jun, Takahashi Yoshiyuki

    PEDIATRIC BLOOD & CANCER   Vol. 66   page: S30 - S30   2019.12

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  45. Risk factors for absence of catch-up growth in small for gestational age very low-birthweight infants

    Arai Sakiko, Sato Yoshiaki, Muramatsu Hideki, Yamamoto Hidenori, Aoki Fumiko, Okai Yu, Kataoka Shinsuke, Hanada Yu, Hamada Motoharu, Morimoto Yoshihito, Kojima Seiji, Natsume Jun, Takahashi Yoshiyuki, Sugiyama Yuichiro, Hoshino Shin, Kawada Junichi, Kidokoro Hiroyuki, Hayakawa Masahiro, Hattori Tetsuo, Kato Yuichi, Yasuda Ayako, Oshiro Makoto, Takemoto Koji, Nishimura Naoko, Hayashi Seiji, Hyodo Reina, Ito Masatoki, Narahara Shou, Ieda Kuniko, Yamamoto Hikaru

    PEDIATRICS INTERNATIONAL   Vol. 61 ( 9 ) page: 889 - 894   2019.9

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    DOI: 10.1111/ped.13939

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  46. ステロイド減量に伴い再発を繰り返した抗MOG抗体陽性急性散在性脳脊髄炎(ADEM)の1例

    隈井 すみれ, 山本 啓之, 川口 将宏, 牧 祐輝, 田中 雅大, 岡井 佑, 坂口 陽子, 中田 智彦, 城所 博之, 高橋 義行, 夏目 淳, 濱口 直子, 柴田 元博, 金子 仁彦, 高橋 利幸

    日本小児科学会雑誌   Vol. 123 ( 8 ) page: 1310 - 1310   2019.8

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  47. Comparison of Clinical Characteristics of Human Metapneumovirus and Respiratory Syncytial Virus Infections in Hospitalized Young Children

    Taniguchi Akinobu, Kawada Jun-ichi, Go Kiyotaka, Fujishiro Naozumi, Hosokawa Yosuke, Maki Yuki, Sugiyama Yuichiro, Suzuki Michio, Tsuji Takeshi, Hoshino Shin, Muramatsu Hideki, Kidokoro Hiroyuki, Kinoshita Fumie, Hirakawa Akihiro, Takahashi Yoshiyuki, Sato Yoshiaki, Natsume Jun, Nagoya Collaborative Clinical Research Team

    Japanese Journal of Infectious Diseases   Vol. 72 ( 4 ) page: 237 - 242   2019.7

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    <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>

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  48. Hippocampal diffusion abnormality after febrile status epilepticus is related to subsequent epilepsy Reviewed International journal

    Setsuri Yokoi, Hiroyuki Kidokoro, Hiroyuki Yamamoto, Atsuko Ohno, Tomohiko Nakata, Tetsuo Kubota, Takeshi Tsuji, Masashi Morishita, Takashi Kawabe, Misako Naiki, Koichi Maruyama, Kazuya Itomi, Toru Kato, Komei Ito, Jun Natsume

    Epilepsia   Vol. 60 ( 7 ) page: 1306 - 1316   2019.7

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    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.

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

  49. Design of a prospective multicenter randomized controlled trial evaluating the effects of gastric lavage on coffee-ground emesis in neonates: study protocol. Reviewed

    Takashi Maeda, Yoshiaki Sato, Akihiro Hirakawa, Masahiro Nakatochi, Fumie Kinoshita, Takeshi Suzuki, Shintaro Ichimura, Ryoichi Ito, Ryuji Kudo, Michio Suzuki, Shin Hoshino, Yuichiro Sugiyama, Hideki Muramatsu, Hiroyuki Kidokoro, Jun-Ichi Kawada, Yoshiyuki Takahashi

    Nagoya journal of medical science   Vol. 81 ( 2 ) page: 227 - 232   2019.5

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    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.

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  50. Comparison of high-dose and low-dose corticosteroid therapy for refractory Mycoplasma pneumoniae pneumonia in children. Reviewed International journal

    Toshihiko Okumura, Jun-Ichi Kawada, Masaharu Tanaka, Kotaro Narita, Tomonori Ishiguro, Yuji Hirayama, Sho Narahara, Genki Tsuji, Yuichiro Sugiyama, Michio Suzuki, Takeshi Tsuji, Shin Hoshino, Masahiro Nakatochi, Hideki Muramatsu, Hiroyuki Kidokoro, Yoshiyuki Takahashi, Yoshiaki Sato

    Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy   Vol. 25 ( 5 ) page: 346 - 350   2019.5

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    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.

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  51. Continuous EEG monitoring in children with acute encephalopathy

    Natsume Jun, Ohno Atsuko, Yamamoto Hiroyuki, Kidokoro Hiroyuki, Numaguchi Atsushi

    Japanese Journal of Clinical Neurophysiology   Vol. 47 ( 1 ) page: 53 - 57   2019.2

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    <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

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  52. Correction: Treatment with silver nitrate versus topical steroid treatment for umbilical granuloma: A non-inferiority randomized control trial. Reviewed International journal

    Chikako Ogawa, Yoshiaki Sato, Chiyo Suzuki, Azusa Mano, Atsushi Tashiro, Takafumi Niwa, Sayako Hamazaki, Yoshihiro Tanahashi, Midori Suzumura, Satoshi Hayano, Masahiro Hayakawa, Takeshi Tsuji, Shin Hoshino, Yuichiro Sugiyama, Hiroyuki Kidokoro, Jun-Ichi Kawada, Hideki Muramatsu, Akihiro Hirakawa, Masahiko Ando, Jun Natsume, Seiji Kojima

    PloS one   Vol. 14 ( 6 ) page: e0218205   2019

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

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  53. Longitudinal Findings of MRI and PET in West Syndrome with Subtle Focal Cortical Dysplasia Reviewed

    Y. Sakaguchi, H. Kidokoro, C. Ogawa, Y. Okai, Y. Ito, H. Yamamoto, A. Ohno, T. Nakata, T. Tsuji, T. Nakane, H. Kawai, K. Kato, S. Naganawa, J. Natsume

    American Journal of Neuroradiology   Vol. 39 ( 10 ) page: 1932 - 1937   2018.10

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  54. Magnetic resonance spectroscopy in preterm infants: association with neurodevelopmental outcomes. Reviewed International journal

    Reina Hyodo, Yoshiaki Sato, Miharu Ito, Yuichiro Sugiyama, Chikako Ogawa, Hisashi Kawai, Toshiki Nakane, Akiko Saito, Akihiro Hirakawa, Hiroyuki Kidokoro, Jun Natsume, Masahiro Hayakawa

    Archives of disease in childhood. Fetal and neonatal edition   Vol. 103 ( 3 ) page: F238 - F244   2018.5

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    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.

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  55. Treatment with silver nitrate versus topical steroid treatment for umbilical granuloma: A non-inferiority randomized control trial. Reviewed International journal

    Chikako Ogawa, Yoshiaki Sato, Chiyo Suzuki, Azusa Mano, Atsushi Tashiro, Takafumi Niwa, Sayako Hamazaki, Yoshihiro Tanahashi, Midori Suzumura, Satoshi Hayano, Masahiro Hayakawa, Takeshi Tsuji, Shin Hoshino, Yuichiro Sugiyama, Hiroyuki Kidokoro, Jun-Ichi Kawada, Hideki Muramatsu, Akihiro Hirakawa, Masahiko Ando, Jun Natsume, Seiji Kojima

    PloS one   Vol. 13 ( 2 ) page: e0192688   2018.2

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    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

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    PubMed

  56. Cytotoxic edema at onset in West syndrome of unknown etiology: A longitudinal diffusion tensor imaging study Reviewed International journal

    Chikako Ogawa, Hiroyuki Kidokoro, Tatsuya Fukasawa, Hiroyuki Yamamoto, Naoko Ishihara, Yuji Ito, Yoko Sakaguchi, Yu Okai, Atsuko Ohno, Tomohiko Nakata, Yoshiteru Azuma, Ayako Hattori, Tetsuo Kubota, Takeshi Tsuji, Akihiro Hirakawa, Hisashi Kawai, Jun Natsume

    Epilepsia   Vol. 59 ( 2 ) page: 440 - 448   2018.2

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    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

  57. Predominant area of brain lesions in neonates with herpes simplex encephalitis Reviewed

    H. Kidokoro, L. S. De Vries, C. Ogawa, Y. Ito, A. Ohno, F. Groenendaal, S. Saitoh, A. Okumura, Y. Ito, J. Natsume

    Journal of Perinatology   Vol. 37 ( 11 ) page: 1210 - 1214   2017.11

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    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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    PubMed

  58. Phenotype-genotype correlations of PIGO deficiency with variable phenotypes from infantile lethality to mild learning difficulties. Reviewed International journal

    Junpei Tanigawa, Haruka Mimatsu, Seiji Mizuno, Nobuhiko Okamoto, Daisuke Fukushi, Koji Tominaga, Hiroyuki Kidokoro, Yukako Muramatsu, Eriko Nishi, Shota Nakamura, Daisuke Motooka, Noriko Nomura, Kiyoshi Hayasaka, Tetsuya Niihori, Yoko Aoki, Shin Nabatame, Masahiro Hayakawa, Jun Natsume, Keiichi Ozono, Taroh Kinoshita, Nobuaki Wakamatsu, Yoshiko Murakami

    Human mutation   Vol. 38 ( 7 ) page: 805 - 815   2017.7

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    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

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  59. Preterm brain injury on term-equivalent age MRI in relation to perinatal factors and neurodevelopmental outcome at two years. Reviewed International journal

    Margaretha J Brouwer, Karina J Kersbergen, Britt J M van Kooij, Manon J N L Benders, Ingrid C van Haastert, Corine Koopman-Esseboom, Jeffrey J Neil, Linda S de Vries, Hiroyuki Kidokoro, Terrie E Inder, Floris Groenendaal

    PloS one   Vol. 12 ( 5 ) page: e0177128 - e0177128   2017.5

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    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

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    PubMed

  60. Paroxysmal nonepileptic events in children with epilepsy. Reviewed International journal

    Yuji Ito, Hiroyuki Kidokoro, Tamiko Negoro, Masaharu Tanaka, Yu Okai, Yoko Sakaguchi, Chikako Ogawa, Tomoya Takeuchi, Atsuko Ohno, Hiroyuki Yamamoto, Tomohiko Nakata, Satoshi Maesawa, Kazuyoshi Watanabe, Yoshiyuki Takahashi, Jun Natsume

    Epilepsy research   Vol. 132   page: 59 - 63   2017.5

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    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

  61. Preterm brain injury and reduced brain growth parameters on term MRI: relation with clinical factors and neurodevelopmental outcome Reviewed International coauthorship

    城所 博之

    PLOS ONE   Vol. 印刷中   2017

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    CiNii Research

  62. Preterm Brain Injury on Term-Equivalent Age MRI in relation to Perinatal Factors and Neurodevelopmental Outcome at Two Years Reviewed International coauthorship

    城所 博之

    PLOS ONE   Vol. 印刷中   2017

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MISC 28

  1. 【知っておきたい小児分野の医療機器】各論 医療機器の仕組みと効果 検査機器 脳波 amplitude-integrated EEGを含めて

    鈴木 健史, 城所 博之

    小児内科   Vol. 52 ( 4 ) page: 511 - 516   2020.4

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    <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)

  2. FDG-PETとEEG-fMRIを用いててんかん焦点、てんかん性ネットワークの継時的評価を行った結節性硬化症の1例

    牧 祐輝, 伊藤 祐史, 岡井 佑, 白木 杏奈, 川口 将宏, 鈴木 健史, 田中 雅大, 坂口 陽子, 山本 啓之, 中田 智彦, 城所 博之, 前澤 聡, バガリナオ・エピファニオ, 寳珠山 稔, 夏目 淳

    てんかん研究   Vol. 37 ( 3 ) page: 838 - 838   2020.1

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  3. INTACTを目指した脳室内出血管理 超早産児IVHと神経発達予後

    城所 博之

    日本新生児成育医学会雑誌   Vol. 31 ( 3 ) page: 623 - 623   2019.10

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  4. West症候群におけるてんかんネットワークの継時的変化

    牧 祐輝, 伊藤 祐史, 岡井 佑, 白木 杏奈, 川口 将宏, 鈴木 健史, 田中 雅大, 坂口 陽子, 山本 啓之, 中田 智彦, 城所 博之, 前澤 聡, バガリナオ・エピファニオ, 寶珠山 稔, 夏目 淳

    臨床神経生理学   Vol. 47 ( 5 ) page: 432 - 432   2019.10

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  5. ILAEが提唱する新しい新生児発作分類の妥当性について

    久保田 哲夫, 奥村 彰久, 楢原 翔, 深沢 達也, 根来 民子, 中田 智彦, 城所 博之, 夏目 淳

    てんかん研究   Vol. 37 ( 2 ) page: 539 - 539   2019.9

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  6. Sub-second EEG-fMRI analysisによる焦点性てんかんの術前評価(Sub-second EEG-fMRI analysis for presurgical evaluation in focal epilepsy)

    Ito Yuji, 前澤 聡, バガリナオ・エピファニオ, 岡井 佑, 中坪 大輔, 山本 啓之, 城所 博之, 臼井 直敬, 夏目 淳, 寳子山 稔, 若林 俊彦, 祖父江 元, 尾崎 紀夫

    てんかん研究   Vol. 37 ( 2 ) page: 513 - 513   2019.9

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  7. West症候群患児におけるBCG接種からACTH療法開始までの期間に関する検討

    牧 祐輝, 白木 杏奈, 川口 将宏, 鈴木 健史, 田中 雅大, 坂口 陽子, 山本 啓之, 中田 智彦, 城所 博之, 石原 尚子, 夏目 淳

    てんかん研究   Vol. 37 ( 2 ) page: 575 - 575   2019.9

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  8. Mental retardation and microcephaly with pontine and cerebellar hypoplasia(MICPCH)の成人例

    西尾 洋介, 城所 博之, 中田 智彦, 原 紳也, 渡邊 一功, 夏目 淳

    脳と発達   Vol. 51 ( Suppl. ) page: S361 - S361   2019.5

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  9. 中心・側頭部に棘波をもつ良性小児てんかんに類似するてんかんを合併したWorster-Drought syndromeの1例

    中田 智彦, 大野 敦子, 川口 将宏, 鈴木 健史, 牧 祐輝, 田中 雅大, 岡井 佑, 坂口 陽子, 伊藤 祐史, 山本 啓之, 城所 博之, 渡邊 一功, 夏目 淳

    脳と発達   Vol. 51 ( Suppl. ) page: S327 - S327   2019.5

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  10. EBウィルス感染に伴った急性脳症の一女児例

    重松 拓朗, 内木 美紗子, 水谷 修平, 竹内 陽平, 尾池 直子, 加藤 俊輔, 前田 徹, 小林 貴江, 足達 武憲, 星野 伸, 河邊 太加志, 奥村 俊彦, 城所 博之, 伊藤 嘉規, 夏目 淳, 谷内江 昭宏

    日本小児科学会雑誌   Vol. 123 ( 2 ) page: 367 - 367   2019.2

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  11. 【「脳波が主役:意識障害・神経救急の診断学」】小児の急性脳症、熱性けいれん重積における脳波

    夏目 淳, 大野 敦子, 山本 啓之, 城所 博之, 沼口 敦

    臨床神経生理学   Vol. 47 ( 1 ) page: 53 - 57   2019.2

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    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)

    Other Link: https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J03414&link_issn=&doc_id=20190314300008&doc_link_id=%2Fff2rinsy%2F2019%2F004701%2F009%2F0053-0057%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fff2rinsy%2F2019%2F004701%2F009%2F0053-0057%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

  12. 先天性横隔膜ヘルニアにおける中期的神経学的予後と頭部MRI画像についての検討

    伊藤 美春, 脇田 浩正, 郷間 環, 上田 一仁, 田中 亮, 三浦 良介, 呉 尚治, 兵藤 玲奈, 浅田 英之, 北瀬 悠磨, 鈴木 俊彦, 見松 はるか, 齊藤 明子, 村松 友佳子, 城所 博之, 佐藤 義朗, 夏目 淳, 高橋 義行, 早川 昌弘

    日本小児科学会雑誌   Vol. 123 ( 2 ) page: 337 - 337   2019.2

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  13. 優秀演題賞候補 重症新生児仮死児におけるaEEGの新たな背景活動パターンとその臨床的意義

    田中 雅大, 城所 博之, 川口 将宏, 牧 祐輝, 岡井 佑, 坂口 陽子, 山本 啓之, 中田 智彦, 渡邊 一功, 高橋 義行, 夏目 淳, 久保田 哲夫, 深沢 達也, 根来 民子, 奥村 彰久, 加藤 徹

    日本小児科学会雑誌   Vol. 123 ( 1 ) page: 102 - 102   2019.1

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  14. 妊娠高血圧症候群を発症した母から生まれた早産児の長期的な身体的・神経学的影響の検討

    牛田 貴文, 城所 博之, 小谷 友美, 早川 昌弘, 吉川 史隆

    日本妊娠高血圧学会雑誌   Vol. 25   page: 87 - 87   2018.11

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  15. 先天性横隔膜ヘルニアにおける頭部MRI画像と退院時予後、1歳半の神経学的予後との関連についての検討

    伊藤 美春, 脇田 浩正, 上田 一仁, 田中 亮, 三浦 良介, 呉 尚治, 兵藤 玲奈, 浅田 英之, 北瀬 悠磨, 鈴木 俊彦, 齊藤 明子, 村松 友佳子, 城所 博之, 佐藤 義朗, 早川 昌弘

    日本新生児成育医学会雑誌   Vol. 30 ( 3 ) page: 628 - 628   2018.10

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  16. 先天性横隔膜ヘルニアにおける頭部MRI画像評価と周生期因子との関連についての検討

    伊藤 美春, 脇田 浩正, 田中 亮, 三浦 良介, 呉 尚治, 兵藤 玲奈, 浅田 英之, 北瀬 悠磨, 鈴木 俊彦, 見松 はるか, 齊藤 明子, 村松 友佳子, 城所 博之, 佐藤 義朗, 早川 昌弘

    日本新生児成育医学会雑誌   Vol. 30 ( 3 ) page: 628 - 628   2018.10

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  17. 【<小児神経の診断・治療アップデート-関連領域とのつながりを探る->】小児神経医に必要な新生児神経学update

    城所 博之

    小児神経学の進歩   Vol. 47   page: 54 - 61   2018.6

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  18. CHRNG変異を有するEscobar症候群の3例

    中田 智彦, 水野 誠司, 牧 祐輝, 田中 雅大, 岡井 佑, 坂口 陽子, 山本 啓之, 大野 敦子, 城所 博之, 井本 逸勢, 大野 欽司, 夏目 淳

    脳と発達   Vol. 50 ( Suppl. ) page: S363 - S363   2018.5

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  19. PHACE症候群の1例

    大野 敦子, 牧 祐輝, 田中 雅大, 岡井 佑, 坂口 陽子, 山本 啓之, 中田 智彦, 村松 友佳子, 城所 博之, 夏目 淳

    脳と発達   Vol. 50 ( Suppl. ) page: S363 - S363   2018.5

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  20. SENDAに認める脳波の異常高振幅速波

    城所 博之, 本林 光雄, 久保田 哲夫, 高野 亨子, 柴 直子, 坂口 陽子, 岡井 佑, 田中 雅大, 牧 祐輝, 山本 啓之, 大野 敦子, 中田 智彦, 村松 一洋, 横地 健治, 夏目 淳

    脳と発達   Vol. 50 ( Suppl. ) page: S332 - S332   2018.5

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  21. shuffling babyの神経発達予後

    岡井 佑, 三浦 清邦, 若子 理恵, 高橋 脩, 田中 雅大, 坂口 陽子, 伊藤 祐史, 山本 啓之, 大野 敦子, 中田 智彦, 城所 博之, 高橋 義行, 夏目 淳

    日本小児科学会雑誌   Vol. 122 ( 2 ) page: 370 - 370   2018.2

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  22. 入浴後のてんかん発作を繰り返した乳児の発作時脳波

    中田 智彦, 田中 雅大, 岡井 佑, 坂口 陽子, 山本 啓之, 大野 敦子, 城所 博之, 根来 民子, 渡邊 一功, 石井 敦士, 廣瀬 伸一, 夏目 淳

    てんかん研究   Vol. 35 ( 3 ) page: 734 - 734   2018.1

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  23. 原因不明のWest症候群におけるACTH療法後の脳波所見と発作予後の関連

    岡井 佑, 田中 雅大, 坂口 陽子, 伊藤 祐史, 山本 啓之, 大野 敦子, 中田 智彦, 城所 博之, 服部 文子, 齋藤 伸治, 倉橋 宏和, 奥村 彰久, 根来 民子, 渡邊 一功, 夏目 淳

    てんかん研究   Vol. 35 ( 2 ) page: 449 - 449   2017.9

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  24. aEEGによる早産児の脳成熟度評価 受胎後齢の判定基準とその精度

    加藤 徹, 辻 健史, 早川 文雄, 久保田 哲夫, 城所 博之, 夏目 淳, 奥村 彰久

    脳と発達   Vol. 49 ( Suppl. ) page: S357 - S357   2017.5

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  25. てんかん脳磁図のdipole distribution解析による焦点、伝播経路診断

    山本 啓之, 寳珠山 稔, 白石 秀明, 岡西 徹, 前澤 聡, 田中 雅大, 坂口 陽子, 岡井 祐, 伊藤 祐史, 大野 敦子, 中田 智彦, 城所 博之, 夏目 淳

    脳と発達   Vol. 49 ( Suppl. ) page: S433 - S433   2017.5

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  26. 二相性に拡散強調画像の異常を認めた急性脳症の1例

    中田 智彦, 細川 洋輔, 梶田 光春, 田中 雅大, 岡井 佑, 坂口 陽子, 伊藤 祐史, 山本 啓之, 大野 敦子, 城所 博之, 夏目 淳

    脳と発達   Vol. 49 ( Suppl. ) page: S385 - S385   2017.5

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  27. けいれん重積後の海馬障害にステロイドパルス療法は無効か?

    島川 修一, 畑中 マリ, 利川 寛実, 福井 美保, 柏木 充, 城所 博之, 夏目 淳, 奥村 彰久, 玉井 浩

    日本小児科学会雑誌   Vol. 121 ( 2 ) page: 431 - 431   2017.2

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  28. エベロリムス週2〜3回内服による結節性硬化症の上衣下巨細胞性星細胞腫の体積変化

    夏目 淳, 坂口 陽子, 岡井 佑, 伊藤 祐史, 大野 敦子, 中田 智彦, 山本 啓之, 城所 博之, 根来 民子, 渡辺 一功

    てんかん研究   Vol. 34 ( 3 ) page: 660 - 661   2017.1

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▼display all

Presentations 5

  1. The pathophysiology of preterm brain injuries Invited

    Hiroyuki Kidokoro

    The 20th annual meeting of Infantile seizure society international symposium on neonatal seizures  2019.5.31 

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  2. 大脳新皮質形成の仕組み~ヒト早産児研究から~ Invited

    城所博之

    日本学術会議第一部 心理学・教育学委員会主催  2021.2.8 

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    Language:English   Presentation type:Symposium, workshop panel (nominated)  

  3. 【特別講演】胎児脳の発達と早産児脳障害 Invited

    城所博之

    第25回信州小児神経研究会  2017.7.22 

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  4. 【特別講演】新生児脳画像の現在と未来 Invited

    城所博之

    第12回京都NICU懇話会  2019.6.24 

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  5. 【特別講演】NICU環境と新生児脳の発達 Invited

    城所博之

    第20回奈良新生児研究会  2018.3.1 

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KAKENHI (Grants-in-Aid for Scientific Research) 7

  1. 先進的MRI解析によるDuchenne型筋ジストロフィーの脳構造・機能異常の解明

    Grant number:20K08204  2020.4 - 2023.3

    日本学術振興会  科学研究費助成事業  基盤研究(C)

    夏目 淳, 久保田 一生, 城所 博之, 服部 文子

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    Authorship:Coinvestigator(s) 

    D/BMD患者30名、健常小児20名のMRI撮像、2022年度に画像解析を行い、以下を明らかにし、D/BMD患者における認知・精神発達特性の基盤を明らかにし、リハビリテーション、呼吸補助装置、意思伝達デバイスを用いる際の参考になる情報を得る。研究成果を学会発表し、論文として報告する。
    1) 健常者と比較したD/BMD患者の脳の微細構造、脳機能の異常を明らかにする。2) D/BMDのうち、知的障害、発達障害を持つ患者の脳構造、脳機能の特徴を明らかにする。3) D/BMDのうち、遺伝子検査でDp140の有無による脳の微細構造、脳機能の異常を明らかにする。
    研究を開始するにあたって、Duchenne型およびBecker型の筋ジストロフィー(DMD/BMD)の患者および健常小児で安全に高磁場MRI検査を行い、結果の解析が可能なことを再確認した。その上で、研究協力施設において本研究の広報を行い研究参加希望者を確認した。研究内容の説明を行い文書による同意の上で、2022年3月までに6歳から20歳のDMD/BMD患者21名、コントロールとして年齢をマッチさせた健常小児・若年成人27例でMRI撮像を行った。合わせて患者群においてレーヴン色彩マトリックス検査、絵画語彙発達検査、自閉症スペクトラム指数(AQ)、ADHD-Rating scale(ADHD-RS)の評価を行った。そして画像解析プログラムを用いて拡散テンソル画像から白質線維の脳各部位の結合性の評価し患者と健常小児の脳ネットワーク構造の違いの検出を行った。現時点の解析では患者において左右の上後頭回、左中後頭回、左楔前部など後頭葉の視覚認知に関連する領域の結合性の低下が示唆されている。画像や臨床情報の保存、管理も支障なく行っている。研究成果の一部を2022年6月に開催される日本小児神経学会学術集会で発表する予定である。
    今後は、本研究に参加する患者数、健常小児を増やし、Voxel-based morphometry、安静時-機能的MRIの評価を加えていく。また、画像解析結果と神経心理学的検査との相関、遺伝子変異との関連を評価していく。以上の研究によってDMD/BMD患者の知的障害、発達障害に関連する脳の微細構造、脳機能の異常を明らかにして、患者の日常的ケアやコンピュータデバイスを用いた意思疎通にも有用な情報を提供する。得られた研究成果を国内および国際学会、医学雑誌で公表する。
    患者および家族への研究の広報や説明、コントロールとして用いる健常小児の募集を予定通り行った。患者21名、健常小児27例の撮像を行い、被検者の体調不良などの問題なく続けられている。研究分担者、研究補助者、研究協力者との連携も問題なく行えている。MRIの撮像、結果の中間解析も進められている。更なる患者および健常小児の撮像、画像解析の継続を行っていく。
    本研究に参加する患者数を増やすために、東海地区の医療機関に対象患者の確認を行い、また健常小児の撮像について広報をしていく。Voxel-based morphometry、安静時-機能的MRIの評価を加えていく。また、画像解析結果と神経心理学的検査との相関、遺伝子変異との関連を評価していく。以上の研究によってDMD/BMD患者の知的障害、発達障害に関連する脳の微細構造、脳機能の異常を明らかにして、患者の日常的ケアやコンピュータデバイスを用いた意思疎通にも有用な情報を提供する。得られた研究成果を国内および国際学会、医学雑誌で公表する。

  2. 脳波-近赤外分光法同時記録を用いたサブプレート・ニューロンの脳血流動態の解明

    Grant number:20K08228  2020.4 - 2023.3

    科学研究費助成事業  基盤研究(C)

    城所 博之, 多賀 厳太郎, 夏目 淳

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    Grant amount:\4160000 ( Direct Cost: \3200000 、 Indirect Cost:\960000 )

    サブプレート・ニューロンは、発達期の脳皮質の形成や脳内ネットワークの構築に重要な役割を果たす。ヒト早産児に高率に認める発達障害の病態にも、サブプレート・ニューロンの障害が大きく関与すると考えるが、ヒトでは未解明である。動物実験を通じて、ヒト早産児脳波に見られるブラッシュがサブプレート・ニューロンの活動を反映することが示された。本研究では、1) 脳波に機能的近赤外分光法(fNIRS)を同時記録することでサブプレート・ニューロンの脳局所血流動態を明らかにする。さらに、2) サブプレート・ニューロンの活動が、生後18か月の神経発達や脳皮質構造や脳内ネットワークに及ぼす効果を明らかにする。
    本研究の目的は、ヒト早産児を対象に、1)脳波と機能的近赤外分光法(fNIRS)の同時記録を多チャンネルで行い、サブプレート・ニューロンの神経活動に応答する脳血流反応を明らかにすることである。さらに、2) サブプレート・ニューロンの活動動態が、将来の脳構造や脳機能、神経ネットワーク形成に及ぼす影響を明らかにすることである。本研究は、げっ歯類の実験から導かれた仮説である「サブプレート・ニューロンが胎生期の脳発達や神経ネットワーク構築に重要な役割を果たす」ことをヒトで実証する意義がある。
    令和2年度は、名古屋大学医学部附属病院における在胎22~32週の早産児で、研究参加に同意が得られた早産児30例に対し、安静睡眠時に約60分の脳波-fNIRS同時記録をNICU内あるいは脳波室で施行した。記録は修正30週~44週まで反復して行い、各症例で1~4記録(合計82記録)を行った。次に、得られた脳波データを用い、睡眠覚醒あるいは動睡眠と静睡眠の各ステート分類を視察的に行い、NIRSデータとの関係を検討した。その結果、動睡眠では静睡眠と比較し、NIRSが捉える安静時ネットワークのチャネル間同期性が高値となることを明らかにした。このことは、安静時ネットワークが睡眠ステージで異なる可能性を強く示唆する重要な成果である。さらに、安静時ネットワークが在胎週数が増すにつれて発達的に変化することが示唆された。今後は、症例を積み重ね、集団解析を施行する予定である。
    一方、脳波上に観察されるデルタブラッシュを自動検出するアルゴリズムの開発については、令和2年度は、研究分担者らとともに抽出すべき波形の特徴やモンタージュの選択について議論を重ね試行錯誤を重ねた。令和3年度での完成を目標としている。
    当初見込まれた研究対象者の数を上回るペースで脳波-機能的NIRSの測定を施行できており、脳波やNIRSのデータ解析も順調に進行中である。次年度も引き続き、研究対象者のリクルートを行う予定である。
    令和3年度は、早産児脳波からデルタブラッシュを検出するアルゴリズムの開発と検証を中心に研究を進める。早産児に対する脳波-機能的NIRSの記録は令和3年度も引き続き行い、データを収集する。さらに、対象の早産児を前方視的に追跡し、名古屋大学医学部附属病院にある3テスラ高磁場MRIを用いて、18か月で三次元脳構造MRI画像及び安静時機能的MRIを撮像する。解析には専用のソフトウエア(MATLAB,SPM,Carretなど)を使用し、脳構造画像からは、大脳皮質容量、皮質厚、脳表面積や脳回指数などを計測する。安静時機能的MRIからは、脳内ネットワークの解析を、独立成分分析法、グラフ理論、クラスター解析を用いて行う。また、修正18か月に当院発達外来で神経学的評価及び行動発達評価を行う。小児神経専門医が包括的な神経学的評価を行う。発達検査は、臨床心理士がBayleyⅢ発達評価とM-chatを含む自閉症スクリーニングを行う。

  3. 脳溝形成の個人差に着目した早産児神経発達予後予測モデルの開発

    Grant number:19H04900  2019.4 - 2021.3

    新学術領域研究(研究領域提案型)

    城所 博之

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    Authorship:Principal investigator 

    Grant amount:\4680000 ( Direct Cost: \3600000 、 Indirect Cost:\1080000 )

    ヒトの大脳の脳溝パターンは遺伝要因と環境要因が織りなすフィンガー・プリント(指紋)であり、脳溝パターンには個性が宿る。近年、ヒト成人の脳溝パターンと認知機能や神経発達症との関連が報告され始めている。本研究は、環境要因が強く反映するヒト早産児の多様な脳溝パターンと多彩な神経発達症との関係を最新のMRI技術から明らかにし、脳溝パターンに着目した神経発達予後予測モデルを開発する。さらに、最近私達が見出した「特異な中心溝」をもつ早産児の運動機能、高次脳機能を明らかにすることで、個々の脳溝パターンの臨床意義を明らかにする。
    ヒトの大脳の脳溝パターンは遺伝要因と環境要因が織りなすフィンガー・プリント(指紋)であり、脳溝パターンには個性が宿る。近年、ヒト成人の脳溝パターンと認知機能や神経発達症との関連が報告され始めている。本研究の目的は、環境要因が強く反映するヒト早産児の多様な脳溝パターンと多彩な神経発達症との関係を最新のMRI技術から明らかにし、脳溝パターンに着目した神経発達予後予測モデルを開発することである。また、私達が見出した「特異な中心溝」をもつ早産児の運動機能、高次脳機能を明らかにすることである。
    初年度は、名古屋大学医学部附属病院NICUで入院管理された在胎30週以下の早産児(コホートA)の6歳で施行された脳構造MRI画像約20例と共同研究施設である安城更生病院NICUで入院管理された在胎30週以下の早産児(コホートB)の6歳時の脳構造MRI画像約100例のうち、早産児特有の重篤な脳障害例を除外した合計120例の脳構造MRIを対象として抽出した。各症例のMRI画像をFreeSurferを用い、脳各領域の体積や大脳皮質表面積や皮質厚を解析した。次に、脳溝解析(surface-based analysis:VanEssen.NeuroImage 2012)は、caretver5.65ソフトウエアを用いて構築された3次元脳表面画像から、脳表面積、脳回指数などの各種パラメータや、中心溝の深さ、長さ、脳溝ピット解析(sulcal pit and pattern analysis:Im K and Grant PE.NeuroImage2017)を症例毎に解析を施行した。
    本研究の目的は、2つのコホートを用いて、ヒト早産児の多様な脳溝パターンと多彩な神経発達症との関係を主に6歳時データから明らかにすること、さらに、特異な中心溝形成をもつ超早産児の臨床像と運動・感覚野の機能を神経心理学的あるいは神経画像の両面から明らかにすることである。
    このうち、初年度は6歳時の頭部MRI構造画像データを解析し、神経症状との関係を明らかにすることが目標であった。
    現在までに、私たちは6歳の構造画像から脳各領域の体積や脳表面積を計測し、神経心理学的評価バッテリーとの相関解析を施行できている。一方で、集団画像解析が計画より遅れて進行中であり、次年度に達成する予定で調整中である。
    1.コホートAの新生期と1歳半のMRIを用いた脳溝パターンと周産期因子との関係を明らかにする。コホートAの修正40週(新生児期)および1歳半で施行された構造MRIは約100~120例である。脳溝画像は、昨年度と同様の手法を用い解析し、周産期因子(低栄養、感染・炎症、慢性肺障害、各種薬剤など)と脳溝パターンの関係を明らかにする。
    2.「特異な中心溝」を呈する児に対する機能解析
    6歳時MRIの脳溝解析の結果から、特異な中心溝パターンを認める症例に対し、詳細な神経心理学的評価、利き手、微細運動評価、機能的MRI(functional MRI)撮像を行う。過去の私達の検討から在胎30週以下の早産児の約10%に観察されると推定され、本研究では13~14名が研究対象者と見込まれる。機能的MRIは名古屋大学「脳とこころの研究センター」に設置された3テスラMRI機器を用いて、四肢、顔面、体幹の運動や感覚刺激課題に対するBOLD(Blood Oxygen Level Dependent)信号の変化に加え、安静時機能的MRIを撮像する。
    得られた脳画像を解析し、運動・感覚課題に対する脳領域を同定し、一次運動感覚野のマッピングを行う。また、安静時機能的MRIはFSL(FMRIB Software Library)、SPM(Statistical Parametric Mapping)などの脳画像解析のためのソフトウエアを使用し、感覚運動野ネットワークの変容の有無、異常な脳回・脳溝部位を関心領域としたネットワーク解析を行う。また、中心溝周囲の脳溝に与える影響を、グラフ理論を用いて解析する。

  4. Elucidation of altered neural network in the preterm brain based on spindle bursts in EEG, and its clinical regulation

    Grant number:17K10075  2017.4 - 2020.3

    Kidokoro Hiroyuki

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    Authorship:Principal investigator 

    Grant amount:\4550000 ( Direct Cost: \3500000 、 Indirect Cost:\1050000 )

    During human brain development, spindles burst is a crucial spontaneous activity in EEG. The aim of this study was to elucidate the effect of the electrical activity on functional and structural brain development. We found that in extremely premature infants brushes corresponding to spindle bursts in animals occurred most frequently at 36 postmenstrual weeks. Additionally, the brush occurrence at 36 postmenstrual weeks was associated with white matter abnormality on brain MRI and in turn associated with neurodevelopmental outcomes at 18 months of corrected age. We also showed that functional and structural MRI reveals associations with neurodevelopment at 18 months of corrected age. These data indicated that brushes in EEG contribute to the altered brain development in preterm infants.

  5. From elderly person to children, evaluating development and aging from the viewpoint of functional MRI and physiological indicators

    Grant number:16K10313  2016.4 - 2020.3

    Japan Society for the Promotion of Science  Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

    Nakane Toshiki

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    Authorship:Coinvestigator(s) 

    We measured, analyzed, and evaluated the relationship between the data obtained from physiological indices such as heart rate variability and the neural activity calculated from brain functional images. Regarding heartbeat, it is a factor that causes imaging artifacts in daily clinical image examination, but this time we analyzed brain functional images in relation to the aspect of the autonomic nervous system calculated from it. It was observed that resting brain activity formed networks known as the default mode network. The relationship with heart rate is currently under investifation.

  6. simultaneous EEG-fMRI analysis in West syndrome

    Grant number:16K09987  2016.4 - 2019.3

    Natsume Jun

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    Authorship:Coinvestigator(s) 

    We examined 7 patients with West syndrome (6 patients with cryptogenic West syndrome and 1 with tuberous sclerosis complex) by EEG-fMRI at the onset of West syndrome before ACTH therapy. The temporal information of periodic hypsarrhythmia was used to perform event-related analysis for detecting hypsarrhythmia-related blood oxygenation level-dependent (BOLD) changes on fMRI. As a result, positive BOLD changes were seen in brainstem and multiple cerebral cortices in 6 of 7 patients. After ACTH therapy epileptic spasms disappeared in all patients. However spasms relapsed in 2 patients and focal seizures were seen in 2. At 12 months of age, 3 patients had focal epileptiform discharges. EEG-fMRI at 12 months of age in these 3 patients revealed bilateral frontal positive BOLD in 2 patients and left hippocampal positive BOLD in 1. Positive BIOLD signal in the brainstem disappeared at 12 months of age in all 3 patients.

  7. Functional analysis of fetal acetylcholine receptor in patients with Escobar syndrome

    Grant number:16K19639  2016.4 - 2019.3

    Japan Society for the Promotion of Science  Grants-in-Aid for Scientific Research Grant-in-Aid for Young Scientists (B)  Grant-in-Aid for Young Scientists (B)

    Nakata Tomohiko, NATSUME jun, KIDOKORO hiroyuki, OHNO kinji, ITHO mikako, MIZUNO seiji, Engel Andrew G., Shen Xin-Ming

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    The nonlethal Escobar syndrome is caused by mutation in the CHRNG gene, which encodes the fetal gamma subunit of the acetylcholine receptor. We performed sequencing of CHRNG for patients with multiple joint contractures and found heterozygous mutations including one missense mutation in three patients from two families. We described clinical features and studied functional analysis using culture cells for the missense mutation. Although the clinical features of Escobar syndrome are variable but the condition is characterized by pterygia, all three patients had very mild pterygia. We conducted expression experiments and channel functional analyses using culture cells. We revealed that the mutation cause the fetal fast channel syndrome with abnormally brief opening and activity of the channel.

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Teaching Experience (On-campus) 1

  1. 小児神経学

    2014

 

Academic Activities 2

  1. ILAE_virtual academy 2021

    Role(s):Panel moderator, session chair, etc.

    2021.4

  2. ILAE virtual epilepsy academy 2020

    Role(s):Panel moderator, session chair, etc.

    2020.5

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    Type:Academic society, research group, etc. 

    File: PEEG_9 Course schedule 2020.pdf