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The basic pathogenesis of West syndrome : Epileptogenesis and its implications of brain maturation disturbance and dysmyelination

Research Project

Project/Area Number 63570457
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionDepartment of Pediatrics, Tokyo Women's Medical College

Principal Investigator

IZUMI Tatsuro  Tokyo Women's Medical College, Assistant Prof., 医学部・小児科学教室, 講師 (80119891)

Co-Investigator(Kenkyū-buntansha) KOIZUMI Hiromi  Tokyo Women's Medical College, Research Associate, 医学部・小児科学教室, 研究生 (90183326)
NAKANO Kazutoshi  Tokyo Women's Medical College, Associate, 医学部・小児科学教室, 助手 (00172362)
NAGAKI Shigeru  Tokyo Women's Medical College, Associate, 医学部・小児科学教室, 助手 (20130271)
Project Period (FY) 1988 – 1989
Project Status Completed (Fiscal Year 1989)
Budget Amount *help
¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 1989: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1988: ¥900,000 (Direct Cost: ¥900,000)
KeywordsWest syndrome / Age-dependent epilepsy / CSF gangliosides / Brain maturation disturbance / Dysmyelination / CSFガングリオシド / 発達年齢的変化 / 点頭てんかん / ガングリオシド / GMIガングリオシド / 脳-ミエリン成熟障害
Research Abstract

The gangliosides pattern of cerebrospinal fluid ( CSF ) is similar to that of whole homogenate. We found the low levels of CSF ganglio-series gangliosides in patients with West syndrome and considered the possible implication of its brain maturation disturbance.
One ml each of CSF trom 9 patients with West syndrome and 13 patients of diseased control groups were analyzed separately with highly sensitive TLC/enzyme immunostaining with anti-asialo G_<Ml> ( G_<Al> ) antibody and densitometry.
The levels ( M <plus-minus> SD ) of G_<Ml>, G_<Dla> and total ganglio-series gangliosides of West syndrome and age matched control group were as follows; G_<Ml>, 31 <plus-minus> 27 and 98 <plus-minus> 23 ng/ml CSF, G_<Dla>, 147 <plus-minus> 59 and 266 <plus-minus> 57 ng/ml CSF, total, 692 <plus-minus> 280 and 1,486 <plus-minus> 644 ng/ml CSF, respectively. These differences were all statistically significant ( by two sample t-test ). The levels of G_<Dlb>, G_<Tlb> and G_<Qlb> of patients with West syndrome were clearly low compared with those of age matched control group, respectively. G_<Qlb> was not detected or trace in both patients with West syndrome and neonates.
Because gangliosides are enriched in the outer surface of neuronal plasma membranes, they may play an important role in the transformation of a neuroblast into a functionally mature neuron. The low levels of CSF ganglio-series gangliosides in patients with West syndrome might suggest the maturation disturbance of the brain from early developing stage.

Report

(3 results)
  • 1989 Annual Research Report   Final Research Report Summary
  • 1988 Annual Research Report
  • Research Products

    (19 results)

All Other

All Publications (19 results)

  • [Publications] Izumi T,Koizumi H,Fukuyama Y.: "The low levels of CSF ganglio-series gangliosides in patients with west syndrome and its implication of brain maturation disturbance."

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T,Koizumi H,Fukuyama Y.: "Cerebrospinal fluid gangliosides in patients with west syndrome." Epilepsia. 30. 637 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T,Fukuyama Y.: "Neurological manifestations and its epileptogenesis in neonatal and infantile hypoglycemia due to nesidioblastosis." Art and Science of Epilepsy,ed.by Suzuki J et al.Elsevier,1989.111-113 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T,Fukuyama Y,Tsuji A,Yamanaka T,Hirabayashi Y,Suzuki Y.: "G_<M2>-gangliosidosis:B_1 variant with thermostable β-hexosaminidase A and molecular analysis of the mutant enzyme." Lipid Storage Disorders,ed.by Salvayre R,Douste Blazy L,Gatt S.Biological Medical Aspects NATO ASI Series,New York.Plenum Press.237-245 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 泉達郎、福山幸夫: "乳児難治痙攣に対するビタミンB_6大量療法時の血清濃度の推移" 小児内科. 20. 1261-1265 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 泉達郎、小泉ひろみ、福山幸夫: "West症候群における髄液ガングリオシド分布型とその成熟障害の検討." てんかん治療研究振興財団研究年報. 1. 81-84 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi t, Koizumi H, Fukuyama Y.: "The low levels of CSF ganglio-series gangliosides in patients with West syndrome and its implication of brain maturation disturbance."

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T, Koizumi H, Fukuyama Y.: "Cerebrospinal fluid gangliosides in patients with West syndrome." Epilepsia 30, 637, 1989.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T, Fukuyama Y.: "Neurological manifestations and its epileptogenesis in neonatal and infantile hypoglycemia due to nesidioblatosis." Art and Science of Epilepsy, ed. by Suzuki J et al Elsevier, 111-113, 1989.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T, Fukuyama Y, Tsuji A, Yamanaka T, Hirabayashi Y, Suzuki Y.: "G_<M2>-gangliosidosis : B_1 variant with thermostable beta-hexosaminidase A and molecular analysis of the mutant enzyme." Lipid Storage Disorders, ed. by Salvayre R, Douste Blazy L, Gatt S. Biological Medical Aspects NATO ASI Series, New York. Plenum Press. 237-245, 1989.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T, Koizumi H, Fukuyama Y.: "CSF-gangliosides pattern in patients with West syndrome and ita maturation disturbance." Ann Pep Jpn Epil Res Found 1, 81-84, 1989.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Izumi T,Koizumi H,Fukuyama Y.: "The low levels of CSF ganglio-series gangliosides in patients with West syndrome and its implication of brain maturation disturbance."

    • Related Report
      1989 Annual Research Report
  • [Publications] Izumi T,Koizumi H,Fukuyama Y.: "Cerebrospinal fluid gangliosides in patients with West syndrome." Epilepsia. 30. 637 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] Izumi T,Fukuyama Y.: "Neurological manifestations and its epileptogenesis in neonatal and infantile hypoglycemia due to nesidioblastosis." Art and Science of Epilepsy,ed.by Suzuki J et al.Elsevier,1989. 111-113 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] Izumi T,Fukuyama Y,Tsuji A,Yamanaka T,Hirabayashi Y,Suzuki Y.: "G_<M2>-gangliosidosis:B_1 variant with thermostable β-hexosaminidase A and molecular analysis of the mutant enzyme." Lipid Storage Disorders,ed.by Salvayre R,Douste Blazy L,Gatt S.Biological Medical Aspects NATO ASI Series,New York.Plenum Press.237-245 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 泉達郎,福山幸夫: "乳児難治痙攣に対するビタミンB6大量療法時の血清濃度の推移" 小児内科. 20. 1261-1265 (1988)

    • Related Report
      1989 Annual Research Report
  • [Publications] 泉達郎,小泉ひろみ,福山幸夫: "West症候群における髄液ガングリオシド分布型とその成熟障害の検討" てんかん治療研究振興財団研究年報. 1. 81-84 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 泉達郎、福山幸夫: 小児内科. 20. 1209-12 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 泉達郎、小泉ひろみ、福山幸夫: 1989年7月第31回日本小児神経学会.

    • Related Report
      1988 Annual Research Report

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Published: 1988-04-01   Modified: 2016-04-21  

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