Plasma exchange in cryptogenic new onset refractory status epilepticus

Objective: New onset refractory status epilepticus (NORSE) is a recently described entity and has been difficult to treat because the etiology is often cryptogenic. Our aim in each case was to stop status epilepticus while simultaneously searching for the etiology.

Methods: We describe three patients who presented with NORSE, who were refractory to multiple anticonvulsants and general anesthetics for at least 5 days. All patients had an extensive evaluation including MRI brain, CSF studies, radiologic scans for malignancy and serological autoimmune and infectious investigations.

Results: Each patient responded dramatically to the use of plasma exchange therapy with cessation of status epilepticus by the fourth day of treatment. Although an etiology was sought after, no appropriate cause for NORSE could be found.

Conclusion: We propose early use of plasma exchange therapy (Class IV evidence) in hopes to prevent the complications of status epilepticus and prolonged hospitalization.