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

https://doi.org/10.1016/j.jns.2011.11.029Get rights and content

Abstract

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.

Introduction

When vasculitis affects the CNS without systemic involvement, it is called primary angiitis of the CNS (PACNS). PACNS represents 1.2% of vasculitis of all types. Secondary CNS vasculitis is due to drugs, toxins, infections, systemic disease, and connective tissue disorders like systemic lupus erythematosus (SLE) [1], [2]. CNS vasculitis secondary to SLE occurs in less than 7% of all neuropsychiatric SLE cases [3].

CNS vasculitis diagnosis requires clinical presentation, angiography and/or brain biopsy. CNS vasculitis of all types can present with headache, cognitive deficit, stroke, and seizures. CNS involvement is a major cause of morbidity and mortality in patients with SLE. In PACNS, poor prognosis is the usual outcome if patients are not treated. Treatment is mainly with corticosteroids and cyclophosphamide [4]. However there have been case reports of the use of other immunosuppressive agents such as intravenous gamma globulin, methotrexate, mycophenolate mofetil, and azathioprine with varied outcomes [5].

Though seizures can occur in CNS vasculitis, they are rare. Of 149 reported cases of PACNS through 1998, only 10% involved seizures [6]. Therefore, only a few cases of status epilepticus (SE) have been reported, but mortality has been high [6], [7], [11]. A recent study showed among 100 cases of fatal SE in a developing country, only 1 patient developed SE from vasculitis [8]. No clear consensus exists on how to treat these patients. Epilepsy surgery has been employed successfully in focal onset refractory SE when aggressive medical therapy has failed [9], [10]. We compare 2 patients with SE secondary to CNS vasculitis, one treated with medical therapy alone and the other with epilepsy surgery following failed aggressive medical therapy.

Section snippets

Patient 1

A 22 year old woman with SLE presented with vomiting, dizziness, headache, and fever. Prior to lumbar puncture (LP), she was given a dose of vancomycin, ceftriaxone, and dexamethasone. LP revealed a red blood cell count (RBC) of 9/mm3, a white blood cell count (WBC) of 335/mm3 (83% lymphocytes, 8% plasma cells), glucose of 44 mg/dl, and protein of 51 mg/dL. Cerebrospinal fluid (CSF) bacterial culture was negative. Chest X-ray, urinalysis, routine blood chemistries, and complete cell count were

Discussion

Of the limited cases of SE associated with CNS vasculitis reported, more than half died despite treatment [6], [7], [11]. Although guidelines exist for the initial treatment of SE; no consensus exists regarding what defines refractory SE and what is its best treatment. Mortality rates for SE greater than 3 h approach 50 to 65% due to neuronal injury, infection, and systemic complications [12], [13]. Aggressive medical treatment for SE can lead to complications itself such as hypotension,

References (13)

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