Cornwall Camilla Dyremose, Krøigård Thomas, Kristensen Joachim Sejr Skovbo, Callesen Henriette Edemann, Beier Christoph Patrick
Department of Neurology, Odense University Hospital, Odense, Denmark.
Department of Clinical Research, University of Southern Denmark, Odense, Denmark.
JAMA Neurol. 2023 Jul 31;80(9):959-68. doi: 10.1001/jamaneurol.2023.2407.
Super-refractory status epilepticus (SRSE) is defined as status epilepticus (SE) that continues or recurs 24 hours or more after the onset of anesthetic therapy or recurs on the reduction/withdrawal of anesthesia. Current clinical knowledge of the disease and optimal treatment approach is sparse.
To systematically assess clinical characteristics, causes, outcomes, prognostic factors, and treatment approaches for patients with SRSE.
DESIGN, SETTING, AND PARTICIPANTS: In this systematic review and meta-analysis, all studies reporting adult patients (18 years or older) diagnosed with nonanoxic SRSE were considered for inclusion, irrespective of study design. The databases used were MEDLINE, Cochrane Library, EMBASE, and ClinicalTrials.org (database inception through May 5, 2022).
The study complied with the PRISMA guidelines for reporting, data extraction, and data synthesis. Different tools were used to assess risk of bias. All available data were extracted and missing data were neither imputed nor completed by contacting the study authors.
Successful treatment of SRSE, in-hospital mortality, and disability at discharge (estimated modified Rankin Scale).
The study team identified a total of 95 articles and 30 conference abstracts reporting 1200 patients with nonanoxic SRSE (266 individual patients were available for meta-analysis). They had a mean SRSE duration of 36.3 days, mean age of 40.8 years, and equal sex distribution. Patients with SRSE had a distinct pattern of etiologies where acute cerebral events and unknown etiologies accounted for 41.6% and 22.3% of all etiologies, respectively. Reports of SRSE caused by, eg, alcohol, drugs, or tumors were rare. At discharge, only 26.8% had none to slight disability (none, 16 [8.4%]; nonsignificant and slight disability, 35 [18.4%]). In-hospital mortality was 24.1%. Mortality stabilized after long-term treatment (more than 28 days) but with increased rates of seizure cessation and moderate to severe disability. Established prognostic factors, such as age and etiology, were not associated with in-hospital mortality. Reported treatment with ketamine, phenobarbital, other barbiturates, vagus nerve stimulator, and ketogenic diet were not associated with outcome.
Patients with SRSE are distinct due to their pattern of care (eg, long-term treatment to younger patients without negative prognostic factors and unknown/nonmalignant etiologies) and their natural course of SE. Very long-term treatment was associated with lower mortality and high odds of cessation of SRSE but increased risk of moderate to severe disability.
超难治性癫痫持续状态（SRSE）被定义为在麻醉治疗开始后持续或复发24小时及以上的癫痫持续状态（SE），或在麻醉减量/停用后复发的癫痫持续状态。目前关于该疾病的临床知识和最佳治疗方法较为匮乏。
系统评估SRSE患者的临床特征、病因、结局、预后因素和治疗方法。
设计、设置和参与者：在这项系统评价和荟萃分析中，纳入所有报告诊断为非缺氧性SRSE的成年患者（18岁及以上）的研究，无论研究设计如何。使用的数据库包括MEDLINE、Cochrane图书馆、EMBASE和ClinicalTrials.org（数据库起始至2022年5月5日）。
该研究遵循PRISMA报告、数据提取和数据综合指南。使用不同工具评估偏倚风险。提取所有可用数据，未通过联系研究作者来估算或补充缺失数据。
SRSE的成功治疗、住院死亡率和出院时的残疾情况（估计改良Rankin量表）。
研究团队共识别出95篇文章和30篇会议摘要，报告了1200例非缺氧性SRSE患者（266例个体患者可用于荟萃分析）。他们的SRSE平均持续时间为36.3天，平均年龄为40.8岁，性别分布均衡。SRSE患者有独特病因模式，急性脑事件和病因不明分别占所有病因的41.6%和22.3%。例如，由酒精、药物或肿瘤引起的SRSE报告很少。出院时，只有26.8%的患者无至轻度残疾（无残疾，16例[8.4%]；无显著残疾和轻度残疾，35例[18.4%]）。住院死亡率为24.1%。长期治疗（超过28天）后死亡率稳定，但癫痫发作停止率和中度至重度残疾率增加。既定的预后因素，如年龄和病因，与住院死亡率无关。报告的氯胺酮、苯巴比妥、其他巴比妥类药物、迷走神经刺激器和生酮饮食治疗与结局无关。
SRSE患者因其护理模式（例如，对无不良预后因素且病因不明/非恶性的年轻患者进行长期治疗）和SE的自然病程而具有独特性。极长期治疗与较低死亡率和SRSE停止的高几率相关，但中度至重度残疾风险增加。
