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The paper addresses the relatively rare inherited neurodermal disorder – Sturge-Weber syndrome that can manifest in epileptic seizures. We describe updated concepts, epidemiology, etiology, pathogenesis, clinical manifestations, and surgical treatment of the disease. We examined medical records of 21 patients (aged from 1 to 11 years) with Sturge-Weber syndrome treated over the period of 1996-2016. After surgical treatment of 10 patients (five cases with hemispheretomy and five – with multifocal resection), positive outcomes (Engel class I, II) were found in 70% of cases, and negative (Engel class III, IV) – in 20% of cases. Оne child suddenly died during epileptic seizures. In non-operated children (age from 2 to 5 years) under our observation, an improvement was noted in six cases, no changes – in three cases, and a further progression of the disease – in three cases. In this article, we analyze two of these cases in detail.

About 30% of patients with temporal epilepsy are resistant to antiepileptic drugs; in such cases, surgical treatment is considered to be the best option. Existence of an extra-temporal epileptogenic focus in the insula is a possible cause of seizures after temporal lobe surgery in about 20% of patients. Because of its localization, the anatomical features of the insular lobe, as well as the entire concept of insular epilepsy have not been well documented. Yet, it has been established that the seizures developing from the insular cortex can mimic the paroxysms arising from the temporal lobe. When the insula is involved in the epileptic process, resection of the insular cortex improves the outcome of surgical treatment. Rational and well-based selection of candidates for surgical treatment is of paramount importance for patients with drug-resistant epilepsy. The article presents a review of the up-to-date literature on this matter.