New-Onset Refractory Status Epilepticus with Underlying Autoimmune Etiology: a Case Report.

Lucille Brunker, Priscilla Hirst, Joseph J Schlesinger
Author Information
  1. Lucille Brunker: 1Department of Anesthesiology, Vanderbilt University Medical Center, Nashville, TN USA.
  2. Priscilla Hirst: 2Department of Medicine, Montefiore Health System, New Rochelle, United States. ORCID
  3. Joseph J Schlesinger: 3Department of Anesthesiology, Department of Hearing and Speech Sciences, Department of Biomedical Engineering, Vanderbilt University Medical Center, Nashville, TN USA.

Abstract

Management of new-onset refractory status epilepticus and the approach to burst suppression variable is often challenging. We present the unusual case of a previously healthy 18-year-old male with new-onset status epilepticus admitted to the neurologic intensive care unit for 70 days. Despite treatment with multiple anti-epileptic drugs in addition to IV anesthetics, burst suppression was initially unsustainable and the patient remained in super-refractory status epilepticus. Extensive evaluation revealed an underlying autoimmune-mediated etiology with positivity for glutamic acid decarboxylase-65 antibody. Clinical response with a goal of 1-2 bursts per screen on EEG monitor was eventually achieved after a course of rituximab and plasma exchange therapy as well as a 7-day barbiturate coma with a regimen of clobazam, lacosamide, Keppra, and oxcarbazepine followed by a slow taper of phenobarbital and the addition of fosphenytoin. Remarkably, the patient was subsequently discharged to a rehabilitation facility with complete neurologic recovery. We discuss treatment strategies for new-onset refractory status epilepticus and highlight the role of rapid initiation of burst suppression with high-dose IV anesthetics to ensure neuroprotection while the underlying etiology is addressed with immune-modulating therapy.

Keywords

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