Jang Yoonhyuk, Ahn Soo Hyun, Park Kyung-Il, Jang Bum-Sup, Lee Han Sang, Bae Jae-Han, Lee Yoonkyung, Sunwoo Jun-Sang, Jun Jin-Sun, Kim Keun Tae, Mon Su Yee, You Ji Hye, Kim Tae-Joon, Shin Hyunsuk, Han Dohyun, Cho Yong Won, Dubey Divyanshu, Chu Kon, Lee Sang Kun, Lee Soon-Tae
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Jongno-gu, Seoul, South Korea.
Biomedical Research Institute, Seoul National University Hospital, Jongno-gu, Seoul, South Korea.
J Neurol Neurosurg Psychiatry. 2024 Dec 16;96(1):26-37. doi: 10.1136/jnnp-2024-334285.
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.
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.
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.
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.
目前，对于隐源性新发难治性癫痫持续状态（cNORSE）的临床动态、预后因素及治疗指导尚缺乏全面认识。在此，我们展示了cNORSE患者的纵向临床特征、预后预测因素及免疫治疗的最佳持续时间。
这项回顾性次要终点分析研究了在韩国一家国家转诊中心从一个前瞻性自身免疫性脑炎队列中识别出的cNORSE患者。主要结局包括纵向功能量表、癫痫发作频率及抗癫痫药物数量。测量指标涵盖与NORSE相关的临床参数，如昏迷持续时间、免疫治疗情况、细胞因子/趋化因子分析及系列磁共振成像扫描。
最终分析了74例cNORSE患者（平均年龄：38.0±18.2岁；36例（48.6%）为男性）。所有患者均接受了一线免疫治疗，91.9%（68/74）接受了二线免疫治疗。共有83.8%（62/74）的患者在中位30天（14 - 56天）内恢复意识，50%（31/62）在2年时获得良好结局（改良Rankin量表≤2分）。3个月磁共振成像显示存在内侧颞叶（mTL）和mTL以外病变以及昏迷时间延长（≥60天）可预测1年预后不良（改良Rankin量表≥3分）。mTL萎缩的患者在NORSE后癫痫发作负担更高。免疫治疗的最佳持续时间似乎在NORSE发作后18周和1年之间。
本研究阐明了cNORSE患者的纵向临床动态、功能结局、预后因素及免疫治疗反应。这些发现可能有助于对cNORSE有更标准化的理解及临床决策。
