BILATERAL OCCIPITAL CALCIFICATION, EPILEPSY AND CELIAC-DISEASE - CLINICAL AND NEUROIMAGING FEATURES OF A NEW SYNDROME

Twenty patients affected by bilateral occipital cortical-subcortical c alcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 ca ses studied, intestinal biopsy revealed coeliac disease. Fourteen pati ents had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with menta l deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was n ormal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MR I was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. Th e study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified.