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Neuro-inflammation
Original research
Prognosis prediction and immunotherapy optimisation for cryptogenic new-onset refractory status epilepticus
  1. Yoonhyuk Jang1,2,3,
  2. Soo Hyun Ahn1,
  3. Kyung-Il Park1,4,
  4. Bum-Sup Jang5,
  5. Han Sang Lee1,
  6. Jae-Han Bae6,
  7. Yoonkyung Lee7,
  8. Jun-Sang Sunwoo8,
  9. Jin-Sun Jun9,
  10. Keun Tae Kim10,
  11. Su Yee Mon1,
  12. Ji Hye You1,
  13. Tae-Joon Kim11,
  14. Hyunsuk Shin12,
  15. Dohyun Han12,13,
  16. Yong Won Cho10,
  17. Divyanshu Dubey14,15,
  18. Kon Chu1,
  19. Sang Kun Lee1,
  20. Soon-Tae Lee1
  1. 1Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Jongno-gu, Seoul, South Korea
  2. 2Biomedical Research Institute, Seoul National University Hospital, Jongno-gu, Seoul, South Korea
  3. 3The National Strategic Technology Research Institute, Jongno-gu, Seoul, South Korea
  4. 4Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, South Korea
  5. 5Department of Radiation Oncology, Seoul National University Hospital, Jongno-gu, Seoul, South Korea
  6. 6Department of Neurology, Asan Medical Center, Songpa-gu, Seoul, South Korea
  7. 7Department of Neurology, Dong-A University College of Medicine, Busan, South Korea
  8. 8Department of Neurology, Kangbuk Samsung Hospital, Jongno-gu, Seoul, South Korea
  9. 9Department of Neurology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Yeongdeungpo-gu, Seoul, South Korea
  10. 10Department of Neurology, Keimyung University Dongsan Medical Center, Daegu, South Korea
  11. 11Department of Neurology, Ajou University School of Medicine, Suwon, South Korea
  12. 12Proteomics Core Facility, Biomedical Research Institute, Seoul National University Hospital, Jongno-gu, Seoul, South Korea
  13. 13Department of Transdisciplinary Medicine, Seoul National University Hospital, Jongno-gu, South Korea
  14. 14Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  15. 15Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Soon-Tae Lee; staelee{at}snu.ac.kr

Abstract

Background Cryptogenic new-onset refractory status epilepticus (cNORSE) currently lacks comprehensive knowledge regarding its clinical dynamics, prognostic factors and treatment guidance. Here we present the longitudinal clinical profiles, predictive factors for outcomes and the optimal duration of immunotherapy in patients with cNORSE.

Methods This retrospective secondary endpoint analysis investigated patients with cNORSE identified from a prospective autoimmune encephalitis cohort at a national referral centre in Korea. The main outcomes included longitudinal functional scales, seizure frequency and the number of antiseizure medications. Measures encompassed NORSE-related clinical parameters such as the duration of unconsciousness, immunotherapy profiles, cytokine/chemokine analysis, and serial MRI scans.

Results A total of 74 patients with cNORSE were finally analysed (mean age: 38.0±18.2; 36 (48.6%) male). All patients received first-line immunotherapy, and 91.9% (68/74) received second-line immunotherapy. A total of 83.8% (62/74) regained consciousness within a median duration of 30 days (14–56), and 50% (31/62) achieved good outcome (mRS ≤2) at 2 years. Poor 1-year outcomes (mRS ≥3) were predicted by the presence of mesial temporal lobe (mTL) and extra-mTL lesions at 3-month MRI, and prolonged unconsciousness (≥60 days). Those with mTL atrophy exhibited a higher seizure burden post-NORSE. The optimal duration of immunotherapy appeared to be between 18 weeks and 1-year post-NORSE onset.

Conclusions This study elucidates longitudinal clinical dynamics, functional outcomes, prognostic factors and immunotherapy response in patients with cNORSE. These findings might contribute to a more standardised understanding and clinical decision-making for cNORSE.

  • neuroimmunology
  • epilepsy
  • autoimmune encephalitis
  • clinical neurology

Data availability statement

Data are available upon reasonable request. Anonymised data that support the findings of this study are available from the corresponding author upon reasonable request of any qualified investigator for purposes of replicating procedures and results.

https://doi.org/10.1136/jnnp-2024-334285

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    Data availability statement

    Data are available upon reasonable request. Anonymised data that support the findings of this study are available from the corresponding author upon reasonable request of any qualified investigator for purposes of replicating procedures and results.

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    Footnotes

    • KC, SKL and S-TL are joint senior authors.

    • YJ and SHA are joint first authors.

    • YJ and SHA contributed equally.

    • Contributors YJ and S-TL drafted the manuscript. SHA and S-TL revised the manuscript. YJ and SHA prepared tables. YJ and SHA prepared figures. YJ, SHA, S-TL, HSL, J-HB, Y-KL, J-SS, JSJ, JY, T-JK and K-TK reviewed patients’ medical records. YJ, B-SJ and S-TL analysed the clinical data. SHA and K-IP performed volumetric analysis of brain MRI. SYM and S-TL analysed the autoantibody tests. YJ and SKL interpreted continuous EEG. YJ, SHA, S-TL, HS and DH analysed the cytokine/chemokine assays. S-TL, JHY, KC and SKL collected clinical data. YJ, K-IP, DD and S-TL provided study concepts. S-TL, KC and SKL supervised the study. All authors reviewed the manuscript. Guarantor: S-TL.

    • Funding This research was supported by a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, Republic of Korea (grant number: RS-2023-00266044).

    • Competing interests Dr S-TL reports personal fees from Roche/Genentech (steering committee) and Advanced Neural Technologies (advisory board), and grants from GC Pharma and Celltrion, outside the submitted work. Dr DD reports consultant fees (paid to institution) from UCB, Immunovant, Argenx, Arialys and Astellas Pharmaceuticals. Dr DD has patents pending for LUZP4-IgG, cavin-4-IgG and SKOR2 IgG as markers of neurological autoimmunity, and patents licensed for KLHL11 IgG.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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