EPILEPSY WITH BILATERAL OCCIPITAL CALCIFICATIONS - STURGE-WEBER VARIANT OR A DIFFERENT ENCEPHALOPATHY

A series of cases of epilepsy with associated bilateral occipital calc ifications (EBOC) without signs of phakomatosis and without any disord ers known to produce cerebral calcifications have been reported. It is unclear whether EBOC is an incomplete variant of Sturge-Weber disease (SWD) or if it is a different, as yet undefined encephalopathy. We de scribe four new cases of EBOC that are different clinically by age of onset, type, course, severity of epilepsy, and associated cognitive de ficits but that are linked by similar neuroradiologic findings. Simila r to cases described in the literature, there is convincing evidence i n favor of the hypothesis that these cases belong to an encephalopathy different from SWD and frequently associated with celiac disease.