CELIAC-DISEASE, BILATERAL OCCIPITAL CALCIFICATIONS AND INTRACTABLE EPILEPSY - MECHANISMS OF SEIZURE ORIGIN

Purpose: To elucidate the mechanisms of seizure origin ill patients wi th celiac disease and bilateral occipital calcifications (CEBOC). Indi viduals with CEBOC frequently present with occipital lobe seizures, bu t additional lesions and additional attack patterns may occur. Methods : We studied two men and one woman who had CEBOC. Villous atrophy was revealed in the two patients who underwent duodenal biopsy. All had a comprehensive presurgical evaluation, including prolonged video-EEG re cordings. Two had magnetic resonance imaging (MRI) with volumetric stu dy of mesial temporal structures (MRIV). One patient had undergone ste reotactic intracranial depth electrode studies (SEEG). Results: All pa tients presented with intractable complex partial seizures. Two had pa rtial simple seizures with visual aura. Neurologic examination was nor mal; one was of normal intelligence, and two were mildly retarded. Neu roimaging studies showed that each had bilateral occipital calcificati ons as well as epileptiform abnormalities over temporal lobes. In one, MRT showed an additional right frontal lesion, but SEEG demonstrated right occipital lobe seizure origin with anterior spread: this male pa tient later underwent a right occipital lobe resection. Another with a history of prolonged febrile convulsions had bilateral hippocampal an d amygdalar atrophy demonstrated by MRIV. Conclusions: In one patient, SEEG confirmed that seizures originated in the occipital lobe. The pr esence of dual pathology was demonstrated in another, raising the poss ibility of both occipital and temporal seizure onset. The presence of extraoccipital lesions or of mesial temporal atrophy requires SEEG for clarification of seizure onset. In the absence of confounding factors and when laterality can be demonstrated, surgical treatment may be co nsidered.