Celiac disease (CD), or gluten sensitivity, is considered to be a state of heightened immunologic responsiveness to ingested gluten proteins in genetically predisposed individuals.
The disease manifests in the gastrointestinal tract as a severe enteropathy of the small intestine, with malabsorption, steatorrhea, and weight loss because of a deranged mucosal immune response.
Neurologic complications also occur, especially epilepsy. This may be associated with occipital calcifications or folate deficiency and cerebellar ataxia.
|Focal white-matter lesions may be extraintestinal manifestation of celiac disease.|
There have been reports of brain white-matter lesions as an extraintestinal manifestation in Crohn's disease and ulcerative colitis, but not in CD.
A study conducted by the Departments of Pediatrics and Neuroradiology, at the Johann Wolfgang Goethe University, in Frankfurt/Main, Germany, has examined 75 diet-treated pediatric patients with biopsy-proven CD.
The patients who enrolled into the prospective study underwent clinical neurologic examinations, laboratory investigations, electroencephalography, computed tomography, and magnetic resonance imaging.
Each patient had been exposed to gluten for a mean period of 2.4 years, and ranged in age from 2.8 to 24.2 years (mean age 11.6 years).
Neurologic findings were identified in 10 patients. These included febrile seizures, single generalized seizures, mild ataxia, and muscular hypotonia with retarded motor development.
The study found no folate deficiencies, and hippocampal regions showed no abnormalities.
Computed tomography did not reveal any cerebral calcifications, but magnetic resonance imaging detected unilateral and bilateral T2-hyperintensive white-matter lesions in 15 patients.
There was no correlation between these lesions and dietary compliance, or neurologic or electroencephalographic abnormalities.
Although the mean gluten exposure time of these patients was slightly increased, this was not significant.
The research team concludes from their investigations that focal white-matter lesions in the brain may represent an extraintestinal manifestation of CD.
They add that these lesions may be ischemic in origin, as a result of a vasculitis, or caused by inflammatory demyelination.
Also, they seem to be more typical of pediatric CD than cerebral calcifications.
Although the group caution that the prognostic value of focal white-matter lesions in the brain is unclear, and needs to be elucidated in additional studies, they finish by saying that CD should be suggested as a differential diagnosis in children with unclear white-matter lesions, even without intestinal symptoms.