Department of Neurology, Asan Medical Center, Seoul, South Korea.
Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, South Korea.
Epilepsia. 2020 Aug;61(8):1735-1748. doi: 10.1111/epi.16620. Epub 2020 Jul 27.
To identify the timelines of magnetic resonance imaging (MRI) abnormalities and their relationships with the clinical outcomes of patients with new-onset refractory status epilepticus (NORSE).
This retrospective observational study enrolled patients with NORSE who were admitted from March 2008 to July 2018. MRI abnormalities were analyzed visually with the readers blinded to the clinical characteristics of the patients. Poor functional outcome was defined as a Glasgow Outcome Scale score ≤ 3 at discharge. Subsequent pharmacoresistant epilepsy was defined as seizures not controlled by two or more anti-seizure medications 6 months after discharge.
Among 39 patients with NORSE, 32 (82.1%) exhibited an MRI abnormality. The most common abnormalities were persisting mesial temporal lobe signal abnormality (51.3%); initial diffuse leptomeningeal enhancement within 16 days from seizure onset (15/35, 42.9%); and hippocampal atrophy, which started to appear 26 days after seizure onset (15/26, 57.7%). Only three patients had claustrum abnormalities. Patients with insular involvement had longer treatment delay than those without (24.0 vs 5.5 hours, respectively, P = .02). Duration of status epilepticus (SE) tended to have a linear association with hippocampal atrophy (P = .055). Patients with diffuse leptomeningeal enhancement were more likely to have a poor functional outcome and to develop subsequent pharmacoresistant epilepsy than those without this finding (93.3% vs 15.0%, P < .001; 75.0% vs 22.2%, P = .004, respectively); the results were significant even after adjusting for age, sex, and duration of SE. Hippocampal atrophy and diffuse cortical atrophy were also significantly associated with poor functional outcomes (P = .001 and P = .002, respectively), and patients with these conditions were more likely to develop subsequent pharmacoresistant epilepsy than those without these conditions, after adjusting for age and sex (P = .035 and P = .048, respectively), but not after adjusting for duration of SE.
Initial diffuse leptomeningeal enhancement and later hippocampal atrophy were associated with a poor functional outcome and subsequent pharmacoresistant epilepsy.
确定新起难治性癫痫持续状态（NORSE）患者的磁共振成像（MRI）异常时间进程及其与临床结局的关系。
本回顾性观察性研究纳入了 2008 年 3 月至 2018 年 7 月期间收治的 NORSE 患者。MRI 异常由阅读者进行盲法分析，阅读者对患者的临床特征不了解。出院时格拉斯哥预后评分（GOS）≤3 定义为预后不良。出院后 6 个月仍存在癫痫发作且应用两种或两种以上抗癫痫药物治疗无效定义为继发药物难治性癫痫。
在 39 例 NORSE 患者中，32 例（82.1%）存在 MRI 异常。最常见的异常表现为持续性海马旁回信号异常（51.3%）；发病后 16 天内出现初始弥漫性软脑膜强化（15/35，42.9%）；以及发病后 26 天开始出现的海马萎缩（15/26，57.7%）。仅有 3 例患者存在胼胝体异常。存在岛叶受累的患者治疗延迟时间长于无岛叶受累的患者（分别为 24.0 小时和 5.5 小时，P=0.02）。癫痫持续状态（SE）的持续时间与海马萎缩呈线性关联趋势（P=0.055）。与无弥漫性软脑膜强化的患者相比，存在弥漫性软脑膜强化的患者预后不良和继发药物难治性癫痫的风险更高（93.3%比 15.0%，P<0.001；75.0%比 22.2%，P=0.004）；即使在校正年龄、性别和 SE 持续时间后，结果仍然显著。海马萎缩和皮质弥漫性萎缩也与预后不良显著相关（P=0.001 和 P=0.002），在校正年龄和性别后，存在这些情况的患者继发药物难治性癫痫的风险高于无这些情况的患者（P=0.035 和 P=0.048），但在校正 SE 持续时间后，结果不再显著。
初始弥漫性软脑膜强化和随后的海马萎缩与预后不良和继发药物难治性癫痫相关。
