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[Celiac disease, cerebral calcifications and epilepsy syndrome]
J C Cuvellier1, L Vallée, J P Nuyts
1Service des maladies infectieuses et de neurologie infantiles, centre hospitalier régional et universitaire de Lille, hôpital B, France.
Abstract:
The syndrome of coeliac disease, epilepsy and cerebral calcifications is a rare complication of coeliac disease. The pathological changes consist in a patchy pial angiomatosis and resemble those of Sturge-Weber syndrome, whose variant without port-wine angioma must be ruled out. Typical course includes three stages leading to a severe encephalopathy. However, the mental impairment is extremely variable. The pathogenetic process is so for unknown; main clues involve a chronic folic acid deficiency or a HLA-related autoimmune disorder. Treatment requires early gluten-free diet and anti-epileptic drug.