Abstracts

Rationale: Sturge-Weber Syndrome (SWS) is a rare cause of catastrophic focal epilepsy associated with progressive neurologic and cognitive decline that is ameanable to early surgical treatment. This study aims to characterize longitudinal patterns of development in individual SWS cases that were treated surgically.Methods: Pre and postoperative clinical parameters describing the severity and extent of the epileptogenic leptangiomatous disease by MRI and EEG, postsurgical seizure control and cognitive developmental profiles of 15 consecutive patients with SWS who had a surgical procedure to treat refractory epilepsy were studied.Results: All 15 patients had a high seizure and disease burden with daily frequent seizures (3>20/day), status epilepticus (2). The localization was hemispheric in 7, multilobar in 6, bilateral in 2. Nine of 15 (60%) remitted immediately postoperatively and remained seizure free at 2 years (Engel 1A), two became seizure free after 2nd surgery and the remaining 4 (2 bilateral) cases had a significant seizure reduction (Engel 3b). Presurgical and post surgical DQs were below 2SD in all cases and varied considerably. Pre-post developmental quotients reflected: no surgery related loss of DQ, stable but delayed longitudinal DQ curves in 11 patients. Bilaterality, poor seizure outcome and additional comorbidities (deafness) were associated with DQ stagnation in 2 cases and loss od DQ in 1 patient. None of the trajectories documented a dramatic restart or catch up of development after successful surgery. There were no patients with a normal general cognitive ability.Conclusions: Longitudinal development after epilepsy surgery in children with SWS progresses at a stable velocity but is significantly delayed. There is no evidence for catch up. Early restart of development following surgery may lead to an improved longterm developmental outcome.