[Clinical and CT-scan presentations in tuberous sclerosis complex: report of eight pediatric cases revealed by epilepsy].

OBJECTIVE

To describe the clinical and CT-scan features of the Tuberous sclerosis complex (TSC) revealed in children by epilepsy.

PATIENTS AND METHOD: It was a cross sectional descriptive study carried out in the Yaounde Gyneco-Obstetric and Pediatric Hospital (YGOPH). Eight consecutive patients consulting for epilepsy between January 2005 and December 20010 with clinical and CT-scan diagnosis criteria of TSC were included in this study.

RESULTS

There were two girls and six boys (aged 25months to 14years) with drug-resistant epilepsy and characteristics skin lesions of TSC (hypomelanotic macules, angiofibroma of Pringle, shagreen patches). The delay between the onset of epileptic crisis and the diagnosis of TSC varied from 10 months to 6years. One case of renal involvement and another of ophthalmic involvement were found. Cerebral CT-scan was abnormal for each patient with calcified sub-ependymal nodules in 100%, cortical tubers in 5 patients (62.5%) and one suspicion of giant sub-ependymal atrocytoma.

CONCLUSION

Features of TSC are ruled by epilepsy, skin lesions, calcified sub-ependymal nodules and cortical tubers. Thus children presenting with epilepsy, should have a careful dermatological examination to search for TSC lesions. Cerebral CT-scan will afterwards seek for characteristic brain lesions.