Metabolite analysis for acute encephalopathy with biphasic seizures and late reduced diffusion
Project/Area Number |
15K09622
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Pediatrics
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Research Institution | Okayama University |
Principal Investigator |
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Project Period (FY) |
2015-04-01 – 2020-03-31
|
Project Status |
Completed (Fiscal Year 2019)
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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)
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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.
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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。
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Report
(6 results)
Research Products
(5 results)
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[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
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Journal Title
Mol Genet Metab
Volume: 125
Issue: 1-2
Pages: 174-180
DOI
NAID
Related Report
Peer Reviewed
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[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.
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Journal Title
Clinica Chimica Acta
Volume: 465
Pages: 5-10
DOI
Related Report
Peer Reviewed
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[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.
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Journal Title
Clinica Chimica Acta
Volume: 466
Pages: 1-5
DOI
Related Report
Peer Reviewed