[Celiac disease, cerebral calcifications and epilepsy syndrome].
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.
Cuvellier
J C
JC
Service des maladies infectieuses et de neurologie infantiles, centre hospitalier régional et universitaire de Lille, hôpital B, France.
Vallée
L
L
Nuyts
J P
JP
D000293:Adolescent; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D002114:Calcinosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D002446:Celiac Disease / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007223:Infant; D013341:Sturge-Weber Syndrome / Q000175:diagnosis; D013577:Syndrome
Le syndrome maladie coeliaque, calcifications cérébrales et épilepsie.