Abstracts

Rationale: Central nervous system (CNS) vasculitis is a rare condition that affects vessels of the brain parenchyma and leptomeninges. Patients can present with headaches, cognitive deficits, strokes, and seizures. Intractable status epilepticus (SE) has been reported with CNS vasculitis. Patients are treated with high dose steroids, immunosuppressants, and antiepileptic drugs (AEDs), often with poor outcomes. Epilepsy surgery has been used successfully in the management of intractable partial SE, yet no reports have been made using surgery for patients with CNS vasculitis. Methods: We compare two similar patients with intractable partial SE secondary to CNS vasculitis. A thorough review of these patients’ charts, electroencephalograms (EEG), neuroimaging, and pathology were undertaken. Results: Case 1: 22-year old female with a history of systemic lupus erythematosus (SLE), presented to the hospital with a generalized tonic clonic seizure. Nine days after admission the patient went into convulsive status epilepticus. EEG showed frequent seizures originating from the right temporal region with secondary generalization. Brain MRI showed increased T2 signal in the right temporal and cerebellar hemispheres. After 6 weeks, the patient continued to be in status epilepticus despite treatment with phenytoin, levetiracetam, topiramate, valproic acid, midazolam, pentobarbital, ketamine, methylprednisolone, and cyclophosphamide. Brain biopsy of the right temporal lobe revealed vascular changes consistent with CNS vasculitis associated with SLE. Epilepsy surgery was planned to remove epileptic foci, however the patient expired the night before surgery. Case 2: 20-year old male with a history of hypertension presented to the hospital with complex partial seizures. Within 48 hours, his seizures became continuous. EEG revealed frequent seizures in the right frontal region with occasional secondary generalization. Brain MRI showed multiple areas of increased T2 signal in the right temporal and bilateral frontal lobes. After treatment with methylprednisolone, phenytoin, carbamezapine, phenobarbital, phenytoin, levetiracetam, pentobarbital, and ketamine, the patient continued to be in partial status epilepticus for 8 weeks. A right craniotomy with intraoperative electrocortiography (ECOG) was performed to localize the seizure focus. Continuous epileptiform activity was seen involving the lateral aspect of the right mid frontal lobe extending close to the midline. A partial right frontal resection was performed to control seizures. Histopathology revealed small vessel vasculitis in the cerebral cortex and leptomeninges. One year later, the patient has returned to college. Conclusions: Two cases of intractable partial SE secondary to CNS vasculitis are presented. One patient was treated with conventional therapies and subsequently expired. The second underwent epilepsy surgery to remove epileptic foci with good outcomes. We propose epilepsy surgery should be considered as a therapeutic option in these patients in addition to conventional therapies.