Neurologic Complications of Sturge-Weber Syndrome: Current Status and Unmet Needs

Abstract Objective To identify the current status and major unmet needs in the management of neurologic complications in Sturge-Weber syndrome (SWS). Methods An expert panel consisting of neurologists convened during the Sturge-Weber Foundation (SWF) Clinical Care Network (CCN) conference in September, 2018. Literature regarding current treatment strategies for neurological complications was reviewed. Results While strong, evidence-based standards are lacking, the implementation of consensus-based standards of care and outcome measures to be shared across all SWF CCN Centers are needed. Each patient with SWS should have an individualized seizure action plan. There is a need to determine the appropriate abortive and preventive treatment of migraine headaches in SWS. Likewise, a better understanding and better diagnostic modalities and treatments are needed for stroke-like episodes. Since behavioral problems are common, the appropriate screening tools for mental illnesses and the timing for screening should be established. Brain magnetic resonance imaging (MRI) preferably done after one year of age is the primary imaging modality of choice to establish the diagnosis, although advances in MRI techniques can improve presymptomatic diagnosis to identify patients eligible for preventive drug trials. Conclusion We identified the unmet needs in the management of neurologic complications in SWS. We define a minimum standard brain MRI protocol to be used by SWS centers. Future multi-center clinical trials on specific treatments of SWS-associated neurologic complications are needed. An improved national clinical database is critically needed to understand its natural course, and for retrospective and prospective measures of treatment efficacy.