[A case of Sturge-Weber syndrome with the abnormal angiographic findings in the cerebral venous system (author's transl)].
A case of Sturge-Weber syndrome with marked abnormalities in the cerebral venous system was reported. The patient was a 2-year old boy who was admitted to the Department of Neurosurgery with the chief complaints of left hemiparesis and left focal seizures. He had vascular nevi on the forehead and upper lip of his face, scalp, right forearm and thigh (Fig. 1). Neurological examination of admission revealed left hemiparesis. Plain skull films indicated no intracranial calcification. EEG showed paroxysmal focus in the right parieto-temporal area. Plain CT scan showed calcium deposits in the right parietooccipital area and contrast enhancement occurred around the areas of calcification (Fig. 2). Venous phases of bilateral CAGs showed abnormalities of the cortical veins and sinuses and abnormal drainage from the cerebral cortex to the deep veins. It also demonstrated persistence of the primitive straight sinus (Fig. 3, 4). From the neurological and neuroradiological findings, this case was diagnosed as the Sturge-Weber syndrome with marked abnormalities in the cerebral venous system. These abnormal findings of veins and sinuses seemed to be brought about by development abnormalities of veins and sinuses which continuously occurred following Streeter's primordial plexus, which has been considered to be a cause of the Sturge-Weber syndrome.
D000798:Angiomatosis / Q000150:complications*; D001921:Brain / Q000098:blood supply*; D002533:Cerebral Angiography; D002675:Child, Preschool; D003392:Cranial Sinuses / Q000002:abnormalities*; D006801:Humans; D008297:Male; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014680:Veins / Q000002:abnormalities