Biomaterial Database

[Enzephalo-trigeminal angiomatosis. Sturge-Weber-Krabbe (author's transl)]. 1977

W D Sagar, and H Schmidberger

Following a case presentation clinical and radiological signs of the Sturge-Weber-Krabbe syndrome as well as its differentiation into three clinical entities according to Poser and Taveras are discussed. Calcification within the atrophic cerebral cortex is a predominant sign. Angiomatous lesions of the external ascending and descending cerebral veins, and occasionally of the internal cerebral veins may be demonstrated by angiography. Calcifications of the cerebral cortex demonstrable on plain skull radiograms diminish the diagnostic value of angiography because of their pathognomic significance. Hyperplasia of the frontal and ethmoid sinuses and thickening of the calcaria are probably directly related to the cerebral atrophy.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D002114	Calcinosis	Pathologic deposition of calcium salts in tissues.	Calcification, Pathologic,Calcinosis, Tumoral,Microcalcification,Microcalcinosis,Pathologic Calcification,Calcinoses,Calcinoses, Tumoral,Microcalcifications,Microcalcinoses,Tumoral Calcinoses,Tumoral Calcinosis
D002533	Cerebral Angiography	Radiography of the vascular system of the brain after injection of a contrast medium.	Angiography, Cerebral,Angiographies, Cerebral,Cerebral Angiographies
D002540	Cerebral Cortex	The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulci. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.	Allocortex,Archipallium,Cortex Cerebri,Cortical Plate,Paleocortex,Periallocortex,Allocortices,Archipalliums,Cerebral Cortices,Cortex Cerebrus,Cortex, Cerebral,Cortical Plates,Paleocortices,Periallocortices,Plate, Cortical
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000798	Angiomatosis	A condition with multiple tumor-like lesions caused either by congenital or developmental malformations of BLOOD VESSELS, or reactive vascular proliferations, such as in bacillary angiomatosis. Angiomatosis is considered non-neoplastic.	Angiomatoses
D013341	Sturge-Weber Syndrome	A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects.	Neuroretinoangiomatosis,Phakomatosis, Sturge-Weber,Angiomatosis Oculoorbital-Thalamic Syndrome,Encephalofacial Hemangiomatosis Syndrome,Meningo-Oculo-Facial Angiomatosis,Meningofacial Angiomatosis-Cerebral Calcification Syndrome,Parkes Weber Syndrome,Parkes-Weber Syndrome,Sturge Disease,Sturge Syndrome,Sturge's Syndrome,Sturge-Kalischer-Weber Syndrome,Sturge-Weber-Dimitri Syndrome,Sturge-Weber-Krabbe Syndrome,Angiomatosis, Meningo-Oculo-Facial,Hemangiomatosis Syndrome, Encephalofacial,Meningo Oculo Facial Angiomatosis,Phakomatosis, Sturge Weber,Sturge Kalischer Weber Syndrome,Sturge Weber Dimitri Syndrome,Sturge Weber Krabbe Syndrome,Sturge Weber Syndrome,Sturge-Weber Phakomatosis,Syndrome, Encephalofacial Hemangiomatosis,Syndrome, Parkes Weber,Syndrome, Parkes-Weber,Syndrome, Sturge,Syndrome, Sturge's,Syndrome, Sturge-Kalischer-Weber,Syndrome, Sturge-Weber,Syndrome, Sturge-Weber-Dimitri,Syndrome, Sturge-Weber-Krabbe