Four patients, aged 13-22 yrs, with focal epilepsy, and bilateral occipital corticosubcortical calcifications without facial cutaneous angioma were followed at the Neurological Institute, University of Bologna Medical School, Via Ugo Foscolo, Bologna, Italy, and were found to develop a severe encephalopathy with progressive mental impairment. The age at onset of seizures was 3-8 years and psychomotor function was normal while seizures remained controlled from 1-2 years. Unexpectedly, the seizures recurred and were refractory to medication. Concommitantly, all patients had progressively severe mental impairment, and the EEG’s showed progressive slowing of the background activity. During non-REM sleep, fast polyspike bursts, diffuse and with greater prominence in both occipital regions, were observed. CT’s showed occipital calcifications and skull X-ray in one patient showed double-contoured curvilinear calcifications. The authors regarded a diagnosis of atypical Sturge-Weber syndrome as questionable. [1]

COMMENT: A case of Sturge-Weber-Dimitri disease without facial nevus [2], published after submission of this paper and noted by the authors as an addendum, was found to have bilateral leptomeningeal angioma. Bilateral calcification and bilateral ectodermal angioma in Sturge-Weber syndrome may not have been reported often but they occur in my experience. A progressive epileptic encephalopathy also may occur, particularly as a sequel to status epilepticus with Sturge-Weber disease. In the present cases the cause for the deterioration was unclear. This experience should prompt consideration of early neurosurgical excision in similar cases with unilateral calcified lesions, despite initial responsiveness to anticonvulsant medication.