Steroids for Drug-Resistant Seizures and Prolonged Stroke-Like Episode in a Patient With Sturge-Weber Syndrome: A...

Sturge-Weber syndrome (SWS) brain involvement has been associated with impairments in the blood-brain barrier (BBB) and microglial activation within involved cortical regions. Acute neurological crises, including seizures, stroke-like episodes, and/or significant headaches, are common in these patients. This report describes in detail an adolescent with SWS and acute drug-resistant status epilepticus, headache, and stroke-like episode who improved clinically when treated with high-dose steroids. Review of medical records identified two other patients previously treated with steroids for acute neurological symptoms.

A 13-year-old boy with SWS brain involvement presented with fever, headache, seizures, and right-sided weakness. Brain magnetic resonance imaging (MRI) revealed characteristic findings of SWS. The patient was placed on continuous electroencephalogram that showed findings consistent with electrographic status epilepticus. Seizures were refractory to multiple anti-seizure medications as well as to an intravenous midazolam drip. A repeat MRI of the brain documented a significant increase in leptomeningeal enhancement with associated gyral edema and sulcal effacement. Steroid therapy with methylprednisolone and prednisone improved his status epilepticus and stroke-like symptoms, including resolution of seizures and marked improvement in his hemiparesis.

This patient suggests a potential role for inflammation-targeted therapies in individuals with SWS brain involvement who present with prolonged neurological crises. Two other patients with SWS brain involvement who were treated with high-dose steroids in the context of acute neurological episodes also support the need for future clinical and preclinical research to validate this approach.