Plexiform neurofibroma of the occipital scalp.
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Biomedical subjects
Publications and source records attributed to L Maiuri.
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A rare case of Sturge-Weber syndrome without facial nevus and epileptic seizures is reported. The other cases of incomplete form of the disease reported in the literature showed occipital calcification and epileptic seizures without facial nevus, while in the present case also the convulsions were absent. The possible pathogenic mechanism is discussed. The CT findings of these incomplete forms include unilateral or often bilateral occipital calcifications with no evidence of contrast enhancement. The Authors conclude that the radiologic finding of bilateral gyriform calcifications in the occipital region must suggest the diagnosis of Sturge-Weber disease even in the absence of facial nevus and epileptic seizures.
The Authors report a case of a 7-year-old boy with focal epileptic seizures; cerebral panangiography revealed a "coiling" of the right internal carotid artery, a scarcely visualized right posterior cerebral artery, and a diffuse hypovascularisation of the right cerebral hemisphere. They emphasize the possible relation between the vascular insufficiency and the epileptic seizures and they point out the possible role of congenital vasal malformations, as coiling and hypoplasia, in the origin of epilepsy in infancy.
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The authors report six cases of malignant extradural spinal tumors in childhood (3 sarcomas, 2 neuroblastomas and one metastasis). They emphasize the different frequency of these tumors in infants and in adults, the clinical diversity between the primitive and metastatic tumors and the necessity of an early surgical treatment followed by the radiotherapy in the primitive tumors, because of the possibility of long survival.
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