Cuvellier J C, Vallée L, Nuyts J P
Service des maladies infectieuses et de neurologie infantiles, centre hospitalier régional et universitaire de Lille, hôpital B, France.
Arch Pediatr. 1996 Oct;3(10):1013-9. doi: 10.1016/0929-693x(96)81725-8.
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.
乳糜泻、癫痫和脑钙化综合征是乳糜泻的一种罕见并发症。病理变化表现为散在性软脑膜血管瘤病,与斯特奇-韦伯综合征相似,必须排除无葡萄酒色斑血管瘤的该综合征变体。典型病程包括导致严重脑病的三个阶段。然而,智力损害程度差异极大。发病机制目前尚不清楚;主要线索包括慢性叶酸缺乏或与人类白细胞抗原相关的自身免疫性疾病。治疗需要早期采用无麸质饮食和抗癫痫药物。