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Coexistence of Chiari 2 malformation and moyamoya syndrome in a 17-year-old girl.


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Title: Coexistence of Chiari 2 malformation and moyamoya syndrome in a 17-year-old girl.
Authors: Suyama, Kazuhiko / Yoshida, Koichi / Hayashi, Kentaro / Takahata, Hideaki / Yonekura, Masahiro / Nagata, Izumi
Issue Date: Jan-2009
Publisher: Japan Neurosurgical Society
Citation: Neurologia Medico-Chirurgica, 49(1), pp.47-49; 2009
Abstract: 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.
Keywords: moyamoya disease / Chiari 2 malformation
URI: http://hdl.handle.net/10069/21981
ISSN: 13498029
DOI: 10.2176/nmc.49.47
PubMed ID: 19169004
Relational Links: http://ci.nii.ac.jp/naid/110007028639
Rights: Copyright (c) 2009 by The Japan Neurosurgical Society / 本文データは学協会の許諾に基づきCiNiiから複製したものである。
Type: Journal Article
Text Version: publisher
Appears in Collections:Articles in academic journal

Citable URI : http://hdl.handle.net/10069/21981

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