1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-2-40 10.15844/pedneurbriefs-2-5-14 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Epilepsy with Occipital Calcifications http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 05 1988 01 08 2016 2 5 40 40 Copyright: © 1988 The Author(s) 1988 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity

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.

 Bilateral Occipital Corticosubcortical Calcifications Progressive Mental Impairment Double-Contoured Curvilinear

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.

 Gobbi G Sorrenti G Santucci M Rossi PG Ambrosetto P Michelucci R Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity Neurology 1988 Jun 38 6 913 20 10.1212/WNL.38.6.913 3368073 Taly AB Nagaraja D Das S Shankar SK Pratibha NG Sturge-Weber-Dimitri disease without facial nevus Neurology 1987 Jun 37 6 1063 4 10.1212/WNL.37.6.1063 3587631