Journal of Neurocritical Care (대한신경집중치료학회지)

The Korean Neurocritical Care Society (대한신경집중치료학회)
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Consciousness Recovery by Rituximab after Seizure Control in Cryptogenic New-Onset Refractory Status Epilepticus

잠복신발현난치뇌전증지속상태에서 발작이 조절된 후에 투여한 리툭시맙에 의한 의식 회복

  • Yang, Tae-Won (Department of Neurology, Gyeongsang National University Changwon Hospital, Gyeongsang National University College of Medicine) ;
  • Jo, Jeong Won (Department of Dermatology, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine) ;
  • Kim, Do-Hyung (Department of Neurology and Institute of Health Science, Gyeongsang National University College of Medicine) ;
  • Kim, Young-Soo (Department of Neurology and Institute of Health Science, Gyeongsang National University College of Medicine) ;
  • Kwon, Oh-Young (Department of Neurology and Institute of Health Science, Gyeongsang National University College of Medicine)
  • 양태원 (경상대학교 의과대학 창원경상대학교병원 신경과) ;
  • 조정원 (경상대학교 의과대학 경상대학교병원 피부과) ;
  • 김도형 (경상대학교 의과대학 신경과학교실 및 건강과학연구원) ;
  • 김영수 (경상대학교 의과대학 신경과학교실 및 건강과학연구원) ;
  • 권오영 (경상대학교 의과대학 신경과학교실 및 건강과학연구원)
  • Received : 2018.10.31
  • Accepted : 2018.12.07
  • Published : 2018.12.30
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Abstract

Background: New-onset refractory status epilepticus (NORSE) occurs in people without a history of seizures. In these cases, the seizure causes are unclear, and the seizures are not controlled by standard treatment. Autoimmune encephalitis (AIE) can be a cause of NORSE. Cryptogenic NORSE may be associated with AIE, but antibodies associated with the condition have not yet been identified. Primary immunotherapy may not be effective for AIE. Rituximab has improved the prognosis in some cases. Case Report: We treated a cryptogenic NORSE patient with a combination of antiepileptic drugs and immunotherapy. On the 13th hospital day, the seizures were controlled, but the patient remained in a coma. The patient rapidly recovered after administration of rituximab started on the 26th hospital day. Conclusion: Rituximab may be helpful for cryptogenic NORSE patients in whom primary immunotherapy controls seizures, but fails to improve consciousness.

Keywords

References

  1. Gaspard N, Foreman BP, Alvarez V, Cabrera Kang C, Probasco JC, Jongeling AC, et al. New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology 2015;85:1604-13. https://doi.org/10.1212/WNL.0000000000001940
  2. Gall CR, Jumma O, Mohanraj R. Five cases of new onset refractory status epilepticus (NORSE) syndrome: outcomes with early immunotherapy. Seizure 2013;22:217-20. https://doi.org/10.1016/j.seizure.2012.12.016
  3. Li J, Saldivar C, Maganti RK. Plasma exchange in cryptogenic new onset refractory status epilepticus. Seizure 2013;22:70-3. https://doi.org/10.1016/j.seizure.2012.09.011
  4. Shorvon S, Ferlisi M. The treatment of super-refractory status epilepticus: a critical review of available therapies and a clinical treatment protocol. Brain 2011;134(Pt 10):2802-18. https://doi.org/10.1093/brain/awr215
  5. Wilder-Smith EP, Lim EC, Teoh HL, Sharma VK, Tan JJ, Chan BP, et al. The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. Ann Acad Med Singapore 2005;34:417-20.
  6. Gaspard N, Hirsch LJ, Sculier C, Loddenkemper T, van Baalen A, Lancrenon J, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia 2018;59:745-52. https://doi.org/10.1111/epi.14022
  7. Taylor RP, Lindorfer MA. Drug insight: the mechanism of action of rituximab in autoimmune disease--the immune complex decoy hypothesis. Nat Clin Pract Rheumatol 2007;3:86-95.
  8. Lee WJ, Lee ST, Byun JI, Sunwoo JS, Kim TJ, Lim JA, et al. Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort. Neurology 2016;86:1683-91. https://doi.org/10.1212/WNL.0000000000002635
  9. Shin JW, Koo YS, Kim YS, Kim DW, Kim KK, Lee SY, et al. Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis: a multicenter cohort study. J Neuroimmunol 2018;315:1-8. https://doi.org/10.1016/j.jneuroim.2017.12.004
  10. Iizuka T, Kanazawa N, Kaneko J, Tominaga N, Nonoda Y, Hara A, et al. Cryptogenic NORSE: its distinctive clinical features and response to immunotherapy. Neurol Neuroimmunol Neuroinflamm 2017;4:e396. https://doi.org/10.1212/NXI.0000000000000396
  11. Spatola M, Sabater L, Martinez-Hernandez E, Rosenfeld M, Graus F, Dalmau J. Novel findings in autoimmune encephalitis with antibodies against the metabotropic glutamate receptor 5 (mGluR5) (P1.333). Neurology 2017;88(16 Supplement):P1-333.