1989 Fiscal Year Final Research Report Summary
The basic pathogenesis of West syndrome : Epileptogenesis and its implications of brain maturation disturbance and dysmyelination
| Project/Area Number |
63570457
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Department of Pediatrics, Tokyo Women's Medical College |
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Principal Investigator |
IZUMI Tatsuro Tokyo Women's Medical College, Assistant Prof., 医学部・小児科学教室, 講師 (80119891)
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| 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)
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| Project Period (FY) |
1988 – 1989
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| Keywords | West syndrome / Age-dependent epilepsy / CSF gangliosides / Brain maturation disturbance / Dysmyelination |
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| 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.
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[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.
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