Cerebrovascular Moyamoya disease. 1984

Y Yamashiro, and H Takahashi, and K Takahashi

Clinical features of ten Japanese children with cerebrovascular Moyamoya disease are reported and non-Japanese paediatric cases of the disease are reviewed from the world literature. The most common initial manifestations were headache in four cases (40%), motor deficit and convulsion in three cases (30%). As their recurrent and/or residual symptoms, eight children (80%) developed motor deficit consisting of hemiplegia in five cases and paresis or weakness of the extremities in three cases, and four (40%) had headaches. The mode of presentation in our cases was similar to that of non-Japanese cases, in addition to a female preponderance. Electroencephalographic findings of prominent high voltage delta bursts following hyperventilation and slowness of returning to the normal pattern, seen in all hyperventilated cases, is one of the features. Of 15 carotid arteries visualised in ten patients, sites of occlusion or stenosis were seen between the bifurcation of the posterior communicating artery and that of the anterior cerebral artery or the middle cerebral artery in 13 arteries, with a vascular network in the basal ganglia. This study suggests that if hyperventilation procedures produce prominent high voltage delta bursts during electroencephalography in children with headache and/or motor deficit, cerebrovascular disease, especially Moyamoya disease, should be suspected.

UI MeSH Term Description Entries
D008297 Male Males
D009072 Moyamoya Disease A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults. Cerebrovascular Moyamoya Disease,Progressive Intracranial Occlusive Arteropathy (Moyamoya),Moya-Moya Disease,Moyamoya Disease, Classic,Moyamoya Disease, Primary,Moyamoya Disease, Secondary,Moyamoya Syndrome,Classic Moyamoya Disease,Disease, Classic Moyamoya,Disease, Moya-Moya,Disease, Primary Moyamoya,Moya Moya Disease,Moyamoya Diseases, Primary,Primary Moyamoya Disease,Primary Moyamoya Diseases,Secondary Moyamoya Disease
D002339 Carotid Arteries Either of the two principal arteries on both sides of the neck that supply blood to the head and neck; each divides into two branches, the internal carotid artery and the external carotid artery. Arteries, Carotid,Artery, Carotid,Carotid Artery
D002533 Cerebral Angiography Radiography of the vascular system of the brain after injection of a contrast medium. Angiography, Cerebral,Angiographies, Cerebral,Cerebral Angiographies
D002561 Cerebrovascular Disorders A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others. Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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