A woman with ophthalmic migraine was found to have bilateral cerebella
r and cerebral calcifications. She progressively developed severe intr
acranial hypertension, with swelling of the brain and downward transte
ntorial and tonsillar herniation. Because steroid treatment was ineffe
ctive, the right occipital pole was resected. Histological study demon
strated meningo-cortical calcifying angiomatosis. Within 2 months, bra
in swelling and papilledema disappeared. Subtle signs of malabsorption
led to the hypothesis of celiac disease, confirmed by jejunal biopsy.
Similar cerebral histological findings have been reported in the brai
n of two young patients affected by epilepsy and celiac disease. The a
ssociation between cerebral calcifications and celiac disease is pecul
iar: the pathogenetic relationship is unknown. (C) 1998 Elsevier Scien
ce B.V. All rights reserved.