Zaccara Gaetano, Giannasi Gianfranco, Oggioni Roberto, Rosati Eleonora, Tramacere Luciana, Palumbo Pasquale
Unit of Neurology, Department of Medicine, Uslcentro Toscana Health Authority, Firenze, Italy.
Emergency Department, Uslcentro Toscana Health Authority, Firenze, Italy.
Seizure. 2017 Apr;47:17-24. doi: 10.1016/j.seizure.2017.02.015. Epub 2017 Feb 28.
Convulsive status epilepticus (CSE) is a medical emergency associated with high mortality and morbidity. The most recent definition of CSE is a convulsive seizure lasting more than 5min or consecutive seizures without recovery of consciousness. In adults, for the treatment of the early stages of CSE, diazepam, lorazepam or midazolam are the most common treatments, although the choice of agent seems less important than rapid treatment. Midazolam, when administered intramuscularly (best evidence), buccally, or nasally, is effective and safe in the pre-hospital setting. The antiepileptic drugs, phenytoin, valproate, levetiracetam and, more recently lacosamide, are used in CSE that persists after first-line treatments (established CSE). Phenytoin is more difficult to administer and is less well tolerated. Evidence of the efficacy of lacosamide is scarce. Anaesthetics are the drugs of choice for the treatment of refractory CSE (not responding to second-line drugs). Midazolam seems to be the best tolerated and is the most often used drug, followed by propofol and thiopental (pentobarbital in the USA). A few studies indicate that ketamine is effective with the possible advantage that it can be co-administered with other anaesthetics, such as midazolam or propofol. CSE becomes super-refractory after more than 24h of appropriate treatments and may last weeks. Several anaesthetics have been proposed but evidence is scarce. Autoimmune refractory CSE has been recently identified, and early treatment with immuno-modulatory agents (corticosteroids and IV immunoglobulins and also second-line agents such as cyclophosphamide and rituximab followed by chronic immunosuppressive treatment) is now recommended by many experts.
惊厥性癫痫持续状态（CSE）是一种与高死亡率和高发病率相关的医疗急症。CSE的最新定义是惊厥发作持续超过5分钟或连续发作且意识未恢复。在成人中，对于CSE早期阶段的治疗，地西泮、劳拉西泮或咪达唑仑是最常用的治疗药物，尽管药物的选择似乎不如快速治疗重要。咪达唑仑在肌肉注射（最佳证据）、口腔或鼻腔给药时，在院前环境中是安全有效的。抗癫痫药物苯妥英钠、丙戊酸盐、左乙拉西坦以及最近的拉科酰胺，用于一线治疗后仍持续发作的CSE（确立的CSE）。苯妥英钠给药更困难，耐受性也较差。拉科酰胺疗效的证据较少。麻醉剂是治疗难治性CSE（对二线药物无反应）的首选药物。咪达唑仑似乎耐受性最佳，是最常用的药物，其次是丙泊酚和硫喷妥钠（在美国为戊巴比妥）。一些研究表明氯胺酮有效，其可能的优势在于可以与其他麻醉剂如咪达唑仑或丙泊酚联合使用。经过适当治疗超过24小时后，CSE会变得超级难治，可能持续数周。已经提出了几种麻醉剂，但证据较少。自身免疫性难治性CSE最近已被确认，现在许多专家建议早期使用免疫调节药物（皮质类固醇和静脉注射免疫球蛋白，以及二线药物如环磷酰胺和利妥昔单抗，随后进行慢性免疫抑制治疗）。
