[Epilepsy surgery in children with tuberous sclerosis].

Abstract

Most children with tuberous sclerosis (TS) present with intractable seizures. Various factors including demography, clinical data and surgery option are mentioned to affect the outcome after epilepsy surgery in these cases.

Objective: To evaluate some demographic and clinical variables probably related to seizure outcome.

Material and methods: Thirty-three children, median age 4.2 ys (7.5 mths-16 ys), with TS and DR-epilepsy underwent surgery. Within overall 38 procedures (redo surgery was needed in 5 cases), tuberectomy (with or without perituberal cortectomy) was performed in 21 cases, lobectomy - 8, callosotomy - 3, various disconnections (anterior frontal, TPO and hemispherotomy) - 6 patients. Standard preoperative evaluation included MRI and video-EEG. Invasive recordings were used in 8 cases, coupled by MEG and SISCOM SPECT in some cases. ECOG and neuronavigation were used routinely during tuberectomies, and stimulation and mapping were employed in cases with lesions overlapping or near to eloquent cortex. Surgical complications: wound CSF leak (n=1) and hydrocephalus (n=2) were noted in 7.5% of cases. Postoperative neurological deficit (most frequently hemiparesis) developed in 12 patients, being temporary in majority of them. At the last FU (med 5.4 ys) favorable outcome (Engel I) has been achieved in 18 cases (54%), while 7 patients (15%) with persisting seizures reported less common attacks and their milder form (Engel Ib-III). Six patients were able to discontinue AED-treatment and 15 children resumed development and markedly improved in cognition and behavior.

Results and conclusion: Among different variables potentially influencing the outcome after epilepsy surgery in cases with TS, the most important one is seizure type. If prevalent, focal type may be a biomarker of favorable outcomes and probability to become free of seizures.