《英文msh詞典》Sturge Weber Krabbe Syndrome
[入口詞] Sturge Weber Krabbe Syndrome
[主題詞] Sturge-Weber Syndrome
[英文釋義] A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES,progressive hemiparesis,GLAUCOMA,hemianopsia,and cognitive deficits in the first decade of life. By age two years,skull radiographs reveal tramline calcifications of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al.,Principles of Neurology,6th ed,pp1018-9)