Refractory status epilepticus secondary to CNS vasculitis, a role for epilepsy surgery

Central nervous system (CNS) vasculitis is a rare group of disorders that affect vessels of the brain parenchyma and meninges. It presents with headache, cognitive changes, or seizures, yet without aggressive management, it carries a high degree of morbidity and mortality. Refractory status epilepticus (SE) has been reported with CNS vasculitis. Patients are treated with immunosuppression, antiepileptic drugs (AED), and anesthetic agents. Outcomes are usually poor. Epilepsy surgery for refractory partial SE has succeeded in patients. We present a comparison of two patients with refractory partial SE due to CNS vasculitis. One patient was treated medically and died, while the other underwent epilepsy surgery to remove the epileptic focus along with medical therapy and the patient had substantial recovery. We describe clinical, electrophysiological, pathological, and treatment features of both patients and discuss rationale for surgical intervention. This is the first case report of the use of epilepsy surgery for the treatment of refractory SE associated with CNS vasculitis.