Brain white-matter lesions in celiac disease: A prospective study of 75 diet-treated patients

Objective. Celiac disease (CD), or gluten sensitivity, is considered to be a state of heightened immunologic responsiveness to ingested gluten protein s in genetically predisposed individuals. The gastrointestinal manifestatio n suggests a severe enteropathy of the small intestine with malabsorption, steatorrhea, and weight loss because of a deranged mucosal immune response. Neurologic complications occur, especially epilepsy, possibly associated w ith occipital calcifications or folate deficiency and cerebellar ataxia. Th ere have been reports of brain white-matter lesions as an extraintestinal m anifestation in Crohn disease and ulcerative colitis but not in CD. Methods. In this study, 75 diet-treated mainly pediatric patients with biop sy-proven CD underwent prospectively clinical neurologic examinations, labo ratory investigations, electroencephalography, computed tomography, and mag netic resonance imaging. The age range was 2.8 to 24.2 years with a mean of 11.6 years. The mean period of gluten exposure was 2.4 years. Results. Ten patients had neurologic findings such as febrile seizures, sin gle generalized seizures, mild ataxia, and muscular hypotonia with retarded motor development. No folate deficiency was found. The hippocampal regions showed no abnormalities. Computed tomography did not reveal any cerebral c alcifications, but magnetic resonance imaging detected unilateral and bilat eral T2-hyperintensive white-matter lesions in 15 patients (20%). There was no correlation between these lesions and dietary compliance or neurologic or electroencephalographic abnormalities. The mean gluten exposure time of these patients was slightly increased (not significant). Conclusions. Focal white-matter lesions in the brain may represent an extra intestinal manifestation of CD. They may be ischemic in origin as a result of a vasculitis or caused by inflammatory demyelination. They seem to be mo re typical of pediatric CD than cerebral calcifications. Their prognostic v alue is unclear and needs to be elucidated in additional studies. CD should be suggested as a differential diagnosis in children with unclear white-ma tter lesions even without intestinal symptoms.