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Metabolite analysis for acute encephalopathy with biphasic seizures and late reduced diffusion

Research Project

Project/Area Number 15K09622
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Pediatrics
Research InstitutionOkayama University

Principal Investigator

Akiyama Tomoyuki  岡山大学, 医歯薬学総合研究科, 准教授 (10379737)

Project Period (FY) 2015-04-01 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2019: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2018: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2017: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2016: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywordsけいれん重積型脳症 / 熱性けいれん / ピリドキサールリン酸 / アミノ酸 / メタボローム解析
Outline of Final Research Achievements

The initial presentation of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is indistinguishable from that of complex febrile seizures (FS). The aim of this study is to investigate our hypothesis that VB6 deficiency in the brain may play a role in AESD.
We obtained cerebrospinal fluid (CSF) samples from 5 patients with AESD and 18 patients with FS. In AESD, CSF pyridoxal phosphate (PLP) concentration was marginally lower and the PLP-to-pyridoxal ratio was significantly reduced compared to those in FS.
Although it is impossible to conclude that low PLP concentration and PLP-to-pyridoxal ratio are causative of AESD, this may be a risk factor for developing AESD. When combined with other markers, this finding may be useful in distinguishing AESD from FS upon initial presentation.

Academic Significance and Societal Importance of the Research Achievements

けいれん重積型脳症は発症時点において熱性けいれんとの区別が困難であり、かつ後遺症の頻度が高いのが問題である。発症時点でけいれん重積型脳症の可能性が高い患者を見つけることができれば、より積極的な治療を行うことにより数日後の発作(晩期発作)と後遺症の予防につなげられるかも知れない。
けいれん重積型脳症では、熱性けいれんではみられない髄液ビタミンB6代謝の変化が発症時点で起こっている可能性が本研究で示された。この成果は、けいれん重積型脳症を早い段階で熱性けいれんと区別するのに有用な可能性がある。

Report

(6 results)
  • 2019 Annual Research Report   Final Research Report ( PDF )
  • 2018 Research-status Report
  • 2017 Research-status Report
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (5 results)

All 2020 2018 2017

All Journal Article (5 results) (of which Peer Reviewed: 5 results)

  • [Journal Article] Vitamin B6 in acute encephalopathy with biphasic seizures and late reduced diffusion2020

    • Author(s)
      Akiyama T, Toda S, Kimura N, Mogami Y, Hanaoka Y, Tokorodani C, Ito T, Miyahara H, Hyodo Y, Kobayashi K
    • Journal Title

      Brain Dev

      Volume: 42 Issue: 5 Pages: 402-407

    • DOI

      10.1016/j.braindev.2020.02.002

    • NAID

      120007042402

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Pyridoxal 5'-phosphate and related metabolites in hypophosphatasia: Effects of enzyme replacement therapy2018

    • Author(s)
      Akiyama T, Kubota T, Ozono K, Michigami T, Kobayashi D, Takeyari S, Sugiyama Y, Noda M, Harada D, Namba N, Suzuki A, Utoyama M, Kitanaka S, Uematsu M, Mitani Y, Matsunami K, Takishima S, Ogawa E, Kobayashi K
    • Journal Title

      Mol Genet Metab

      Volume: 125 Issue: 1-2 Pages: 174-180

    • DOI

      10.1016/j.ymgme.2018.07.006

    • NAID

      120006845807

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the paired serum and cerebrospinal fluid of children2017

    • Author(s)
      Akiyama T, Hayashi Y, Hanaoka Y, Shibata T, Akiyama M, Tsuchiya H, Yamaguchi T, Kobayashi K
    • Journal Title

      Clin Chim Acta

      Volume: 472 Pages: 118-122

    • DOI

      10.1016/j.cca.2017.07.032

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] Simultaneous measurement of monoamine metabolites and 5-methyltetrahydrofolate in the cerebrospinal fluid of children2017

    • Author(s)
      Akiyama, T., Hayashi, Y., Hanaoka, Y., Shibata, T., Akiyama, M., Nakamura, K., Tsuyusaki, Y., Kubota, M., Yoshinaga, H., Kobayashi, K.
    • Journal Title

      Clinica Chimica Acta

      Volume: 465 Pages: 5-10

    • DOI

      10.1016/j.cca.2016.12.005

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [Journal Article] Measurement of pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the cerebrospinal fluid of children2017

    • Author(s)
      Akiyama, T., Akiyama, M., Hayashi, Y., Shibata, T., Hanaoka, Y., Toda, S., Imai, K., Hamano, S.-I., Okanishi, T., Yoshinaga, H., Kobayashi, K.
    • Journal Title

      Clinica Chimica Acta

      Volume: 466 Pages: 1-5

    • DOI

      10.1016/j.cca.2016.12.027

    • Related Report
      2016 Research-status Report
    • Peer Reviewed

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Published: 2015-04-16   Modified: 2021-02-19  

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