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Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
A Case of Klippel-Trenaunay-Weber Syndrome Accompanied by Congenital Hydrocephalus and Micropolygyria
Hideaki ShimeRyusuke ArakiHiroyoshi KoideToru MiyajiKei Shioda
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JOURNAL FREE ACCESS

1992 Volume 24 Issue 4 Pages 353-357

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Abstract
We report a patient with the Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus, which was slowly progressive and an Ommaya's reservoir was set up. However, the hydrocephalus remained stable even when the shunt was removed due to infection. Generalized tonic clonic convulsions had appeared from six months after birth and were treated with valproic acid. The electroencephalogram showed hypsarrhythmia. He died at eight months of age. Autopsy revealed extensive micropolygyria of the bilateral cerebral hemispheres and hydrocephalus.
To our knowledge, it is rare for the Klippel-Trenaunay-Weber syndrome to be accompanied by congenital hydrocephalus, and there has been no previous report of its occurrence with micropolygyria.
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© Japanese Society of Child Neurology
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