Short communicationRefractory status epilepticus secondary to CNS vasculitis, a role for epilepsy surgery
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,
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Dietary, immunological, surgical, and other emerging treatments for pediatric refractory status epilepticus
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2022, Zeitschrift fur Epileptologie