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

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.