Epilepsy and Celiac Disease

November 1993. European Journal of Pediatrics. Authors Hilhorst MI. Brink M. Wauters EA. Houwen RH. Institution: Department of gastro-enterology, Wilhelmina Childrens Hospital, Utrecht, The Netherlands.

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Among 783 patients referred to our institute with different types of seizures as presenting symptom, systematic evaluation of antigliadin and antiendomysial antibodies in the serum has identified nine in whom jejunal biopsy has subsequently confirmed the diagnosis of celiac disease (celiac disease). In three of them brain imaging showed the presence of calcified areas in the occipital region. They had complex partial seizures (CPS), associated in two with transient episodes of blindness.

In another patient with CPS and generalized tonic-clonic seizures (GTCS) progressive multifocal cerebral calcifications were noted. In the other six patients with CPS and/or GTCS cerebral calcifications were absent. Symptoms of celiac disease in all these cases were either not previously taken into account, or they were very mild or completely absent. In a group of 36 patients with clinically manifest celiac disease, regular follow-up, and good compliance with the dietary regimen, no clinical seizures were reported. The pathogenic mechanism and the relationship between epilepsy and an early diagnosis and treatment of celiac disease are discussed (Footnote #1).

Bilateral occipital calcifications, occurring in celiac disease, are factors coming under a particular cerebral syndrome, which also includes epilepsy, migraine-like headache, visual troubles and mental deterioration. They seem to arise from hypofolatemia following gluten-induced enteropathy (Footnote #2).

There have been anecdotal reports of an association between celiac disease and epilepsy with cerebral calcifications that resemble those of the Sturge-Weber syndrome. A series of patients who had epilepsy with calcifications, in whom celiac disease (celiac disease) was incidentally observed, prompted us to study this association. 43 patients (15 male, age range 4.6-30.7 years) were selected from two series. 31 patients with cerebral calcifications of unexplained origin and epilepsy (series A) underwent intestinal biopsy. 12 patients with celiac disease and epilepsy (series underwent computed tomography. Antibodies to gluten, folic acid serum concentrations, were measured, and HLA typing was done in most patients. 24 of the series A patients were identified as having celiac disease on the basis of a flat intestinal mucosa (15/22 with a high concentration of serum antigluten), and 5 series B patients showed cerebral calcifications, giving a total of 29 cases with the combination of celiac disease, epilepsy, and cerebral calcifications (CEC). In 27 of these CEC patients, calcifications were located in the parieto-occipital regions. Only 2 of the series A patients had gastrointestinal symptoms at the time of intestinal biopsy; most patients had recurrent diarrhea, anemia, and other symptoms suggestive of celiac disease in the first 3 years of life. The epilepsy in CEC patients was poorly responsive to antiepileptic drugs. Gluten-free diet beneficially affected the course of epilepsy only when started soon after epilepsy onset. Cases of atypical Sturge-Weber syndrome (characterized by serpiginous cerebral calcifications and epilepsy without facial port-wine naevus) should be reviewed, and celiac disease should be ruled out in all cases of epilepsy and cerebral calcifications of unexplained origin (Footnote #3).

4. We report the electroclinical findings of four epileptic patients with clinically asymptomatic celiac disease (celiac disease). Celiac disease diagnosis was suspected by past history and/or computed tomography (CT) findings in all patients and confirmed by laboratory tests and jejunal biopsy. All patients had paroxysmal visual manifestations and ictal EEG discharges arising from the occipital lobe. Epilepsy evolution was favorable in two patients and severe in 2, regardless of CT evidence of occipital corticosubcortical calcifications in 2 patients. Occipital lobe seizures may be characteristic of the epilepsy related to celiac disease, and epileptic patients with these seizures of unknown etiology should be carefully investigated for malabsorption. If past history and/or laboratory tests suggest gastrointestinal (GI) dysfunction they should also undergo small intestinal biopsy even if they do not have GI tract symptoms (Footnote #4).

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A MEDLINE search showed these additional articles on celiac and epilepsy:

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