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Disputes & Debates: Editors' Choice
March 30, 2020

Author response: New onset refractory status epilepticus research: What is on the horizon?

March 31, 2020 issue
94 (13) 596

Abstract

We thank Dr. Sethi for the comment on our Contemporary Issues article.1 There are currently no guidelines and early immunotherapy may be indicated for patients with biomarkers or risk factors for autoimmunity (e.g., extreme delta with or without brushes, suggestive of anti-NMDA receptor antibody encephalitis,2 a personal or family history of autoimmunity), or tumors that are known to be associated with autoimmune encephalitis.3 There are limited data to date, and the decision to treat with immunomodulatory treatments is ultimately a clinical one pending further research. Any patient without a clear etiology for NORSE within the first 72 hours of presentation and without a clear contraindication to immunosuppression warrants careful consideration for treatment with immunotherapy. Current evidence suggests that earlier treatment is associated with better clinical outcomes.4 This comment may also draw further attention to the current autoantibody testing limitations; we hope that improved and faster testing may become available.

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References

1.
Gofton TE, Gaspard N, Hocker SE, Loddenkemper T, Hirsch LJ. New onset refractory status epilepticus research: what is on the horizon? Neurology 2019;92:802–810.
2.
Steriade C, Hantus S, Moosa ANV, Rae-Grant AD. Extreme delta - with or without brushes: a potential surrogate marker of disease activity in anti-NMDA-receptor encephalitis. Clin Neurophysiol 2018;129:2197–2204.
3.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63–74.
4.
Khawaja AM, DeWolfe JL, Miller DW, Szaflarski JP. New-onset refractory status epilepticus (NORSE)--The potential role for immunotherapy. Epilepsy Bhav 2015;47:17–23.
5.
Rosenthal ES, Claassen J, Wainwright MS, et al. Brexanolone as adjunctive therapy in super‐refractory status epilepticus. Ann Neurol 2017;82:342–352.
6.
Sage therapeutics reports top-line results from phase 3 STATUS trial of brexanolone in super-refractory status epilepticus. In: sage therapeutics [online]. Available at: investor.sagerx.com/news-releases/news-release-details/sage-therapeutics-reports-top-line-results-phase-3-status-trial. Accessed May 18, 2019.

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Published In

Neurology®
Volume 94Number 13March 31, 2020
Pages: 596
PubMed: 32229639

Publication History

Published online: March 30, 2020
Published in print: March 31, 2020

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Lawrence J. Hirsch

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