J Neurocrit Care.  2020 Jun;13(1):65-68. 10.18700/jnc.190115.

Favorable clinical course after early-intensive immunotherapy for new-onset refractory status epilepticus

• Kim HS
• Kim J
• Hwang BJ
• Woo KN
• Park MG
• Park KP
• Ahn SH

• KMID: 2503388
• DOI: http://doi.org/10.18700/jnc.190115

Abstract

Background
New-onset refractory status epilepticus (NORSE) refers to the newly established concept of a disease characterized by refractory status epilepticus without an identifiable etiology. Recent reports have indicated the importance of immunotherapy for NORSE.
Case Report
A 37-year-old man with no past history of epilepsy was admitted for a presenting complaint of confusion. He was treated with intravenous acyclovir and an antiepileptic drug (AED), following a preliminary diagnosis of herpes encephalitis. He developed generalized tonic-clonic seizures on day 6 of admission that worsened progressively despite treatment with multiple AEDs. NORSE was considered to be a probable diagnosis at this stage and immunotherapy with intravenous methylprednisolone and immunoglobulin was scheduled. However, persistent seizure activity was observed on the electroencephalogram after the completion of initial immunotherapy. Subsequently, rituximab was administered for 4 weeks. He eventually regained consciousness and was able to resume social activity.
Conclusion
Our patient exhibited a favorable outcome with early-intensive immunotherapy and subsequent rituximab treatment for NORSE.

Keyword

New-onset refractory status epilepticus; Immunotherapy; Rituximab

Figure

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  Fig. 1. (A) Follow-up brain magnetic resonance imaging showing disappearance of the diffusion-restricted lesion with remnant fluid-attenuated inversion recovery changes in the right medial temporal lobe (blue lines), which previously showed an increased diffusion restriction and gadolinium enhancement (red lines). (B) Continuous infusion of multiple anesthetics including midazolam and propofol was increased sequentially for the management of refractory status epilepticus, but were tapered during sequential immunotherapy (methylprednisolone 1,000 mg for 5 days and intravenous immunoglobulin [IVIG] 0.4 g/kg for 5 days), followed by the first infusion of rituximab 375 mg/m2 as booster immunotherapy (red arrow), after the maximum possible administration of multiple antiepileptic drugs including levetiracetam 1,500 mg, every 12 hours; valproic acid 800 mg, every 6 hours; phenytoin 300 mg, every 8 hours; phenobarbital 200 mg, every 8 hours; and topiramate 200 mg, every 12 hours. (C) Rituximab was started on day 23 of hospitalization, based on the findings of bilateral periodic lateralized epileptiform discharge on continuous electroencephalography monitoring even 3 days after the completion of initial immunotherapy, with a regimen of once a week for 4 weeks.

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