Coexistence of Chiari 2 Malformation and Moyamoya Syndrome in a 17-Year-Old Girl -Case Report-:—Case Report—

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著者

    • SUYAMA Kazuhiko
    • Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences
    • HAYASHI Kentaro
    • Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences
    • NAGATA Izumi
    • Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences

抄録

A 17-year-old female with Chiari 2 malformation developed cerebral infarction with angiographically typical bilateral moyamoya vessels manifesting as sudden onset of moderate left hemiparesis. Magnetic resonance imaging revealed multiple infarcts in the right frontal lobe, agenesis of the corpus callosum, upward herniation of the dorsal cerebellum, tectal beak of the midbrain, and downward herniation of the cerebellar vermis. Cerebral angiography demonstrated occlusion of the bilateral internal carotid arteries and basal moyamoya vessels. Single photon emission computed tomography showed significantly reduced regional cerebral blood flow in the right frontoparietal cortex. The cerebral vascular reactivity to acetazolamide was diminished in both cerebral hemispheres. She underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephalo-myo-synangiosis on the right, and on the left 6 months later. Cerebral angiography performed 4 months after the second operation showed good patency of the bypasses and substantial collateral vessels in both cerebral hemispheres. This association may have happened by chance, and a common etiology is uncertain, but a currently undetermined genomic component might have contributed to the disease progression.

A 17-year-old female with Chiari 2 malformation developed cerebral infarction with angiographically typical bilateral moyamoya vessels manifesting as sudden onset of moderate left hemiparesis. Magnetic resonance imaging revealed multiple infarcts in the right frontal lobe, agenesis of the corpus callosum, upward herniation of the dorsal cerebellum, tectal beak of the midbrain, and downward herniation of the cerebellar vermis. Cerebral angiography demonstrated occlusion of the bilateral internal carotid arteries and basal moyamoya vessels. Single photon emission computed tomography showed significantly reduced regional cerebral blood flow in the right frontoparietal cortex. The cerebral vascular reactivity to acetazolamide was diminished in both cerebral hemispheres. She underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephalo-myo-synangiosis on the right, and on the left 6 months later. Cerebral angiography performed 4 months after the second operation showed good patency of the bypasses and substantial collateral vessels in both cerebral hemispheres. This association may have happened by chance, and a common etiology is uncertain, but a currently undetermined genomic component might have contributed to the disease progression.<br>

収録刊行物

  • Neurologia medico-chirurgica

    Neurologia medico-chirurgica 49(1), 47-49, 2009

    社団法人日本脳神経外科学会

各種コード

  • NII論文ID(NAID)
    110007028639
  • NII書誌ID(NCID)
    AN00358613
  • 本文言語コード
    ENG
  • 資料種別
    Journal Article
  • ISSN
    0470-8105
  • データ提供元
    NII-ELS  IR  J-STAGE 
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