Abstract

Introduction: Sturge Weber syndrome (SWS) is a rare, non-hereditary, congenital developmental condition. Abnormal embryological development would be the cause of this disease. The intensity of this process and its extension to adjacent tissues are responsible for the variety of expressions of the syndrome. The main characteristics are the presence of a facial spot at birth, known as port wine, and alterations in the central nervous system, like seizures and mental retardation. Objective: o evaluate the available surgical treatments for SWS epilepsy. Methods: The PubMed and LILACS databases were used, using “treatment” as selection criteria. A total of 18 papers selected were organized in a table for statistical analysis. Results: The majority of studies concluded that both clinical and surgical treatments could be effective in the management of SWS epilepsy, although its effectiveness depends on the disease progression. Conclusion: Clinical and surgical methods should be considered in the treatment of SWS epilepsy. An unanswered question that still remains is whether suppression of seizures could prevent future neurological symptoms and cognitive decline.

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