Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity.

We studied four patients with a focal epilepsy and bilateral occipital corticosubcortical calcifications without any sign of phakomatosis. The clinical course of the disease was similar in all the patients and evolved from a benign onset to a severe encephalopathy with progressive mental impairment. The question of whether these patients have an incomplete and atypical form of Sturge-Weber syndrome or a previously undescribed disorder is addressed.