[A case of Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus and micropolygyria].

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.

• Authors: H Shime, R Araki, H Koide, T Miyaji, K Shioda
• Journal: No to hattatsu = Brain and development (No To Hattatsu) Vol. 24 Issue 4 Pg. 353-7 (Jul 1992) ISSN: 0029-0831 [Print] Japan
• PMID: 1325816 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Valproic Acid

Topics

• Brain (abnormalities, pathology)
• Electroencephalography
• Epilepsy, Generalized (drug therapy, etiology)
• Humans
• Hydrocephalus (complications, pathology)
• Infant, Newborn
• Klippel-Trenaunay-Weber Syndrome (complications, pathology)
• Male
• Valproic Acid (therapeutic use)