title: Coexistence of Chiari 2 malformation and moyamoya syndrome in a 17-year-old girl.
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.
authors.affiliation: Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. ksuyama@nagasaki-u.ac.jp
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Suyama
authors.forename: Koichi
authors.initials: K
authors.lastname: Yoshida
authors.forename: Kentaro
authors.initials: K
authors.lastname: Hayashi
authors.forename: Hideaki
authors.initials: H
authors.lastname: Takahata
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yonekura
authors.forename: Izumi
authors.initials: I
authors.lastname: Nagata
mesh_terms: D000086:Acetazolamide; D000293:Adolescent; D061085:Agenesis of Corpus Callosum; D000714:Anastomosis, Surgical; D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000601:surgery; D002343:Carotid Artery, Internal / Q000000981:diagnostic imaging / Q000473:pathology; D002533:Cerebral Angiography; D002544:Cerebral Infarction / Q000209:etiology; D003097:Collateral Circulation; D004677:Encephalocele / Q000209:etiology; D005260:Female; D005625:Frontal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging; D020022:Genetic Predisposition to Disease; D006801:Humans; D008591:Meningomyelocele / Q000150:complications / Q000601:surgery; D020768:Middle Cerebral Artery / Q000601:surgery; D009072:Moyamoya Disease / Q000150:complications* / Q000601:surgery; D010291:Paresis / Q000209:etiology; D010296:Parietal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging; D013699:Temporal Arteries / Q000601:surgery; D015899:Tomography, Emission-Computed, Single-Photon; D017287:Ventriculoperitoneal Shunt
chemical_list: D000086:Acetazolamide