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KMID : 0352819930090020151
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Kosin Medical Journal
1993 Volume.9 No. 2 p.151 ~ p.159
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Sturge-weber Syndrome of Incomplete Type accompanied by Proliferation of Peripheral Nerve Fibers in Leptomeninges -A Case Report-
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Abstract
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Sturge-weber syndrome is a variant of neurocutaneous syndrome, which is characterized by port-wine nevus of face, angiomatous lesion of ipsilateral meninges associated with calcification of underlying cerebral cortex, and epileptic seizure.
A case of incomplete type of stuge-Weber syndrome accompanied by proliferation of peripheral nerve fibers in an angiomatous leptomeningeal lesion along the Sylvian fissure of right temporal and occipital lobes in a 7-year-old boy was
pathologically
examined. This patient disclosed intermittent epileptic convulsion and left hemiplegia since 2 years of age. Skull X-ray and CT scan of brain showed calcification of rail-road appearance and gyriform calcification, respectively.
Authors report this case with a special reference on the clinico-pathological features of cerebral lesions and significance of the proliferation of nerve fibers in the leptomeningeal angioma.
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KEYWORD
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