From the Departments of Neurology (K.T.T., J.C.P., E.H.K., R.G.G., A.L.H., A.V., M.C.C.) and Pediatrics (E.H.K., A.L.H.), Johns Hopkins Hospital, Baltimore, MD; Department of Neurology (S.E.H.), Mayo Clinic, Rochester, MN; Department of Food and Nutrition Services (K.R.), Rush University Medical Center, Chicago, IL; Institute for Clinical and Translational Research (B.H.), Johns Hopkins University, Baltimore; Department of Neurology (P.W.K.), Johns Hopkins Bayview Medical Center, Baltimore; and Departments of Anesthesiology-Critical Care Medicine, Neurosurgery, and Medicine (R.G.G.), Johns Hopkins University School of Medicine, Baltimore, MD.
Neurology. 2014 Feb 25;82(8):665-70. doi: 10.1212/WNL.0000000000000151. Epub 2014 Jan 22.
To describe a case series of adult patients in the intensive care unit in super-refractory status epilepticus (SRSE; refractory status lasting 24 hours or more despite appropriate anesthetic treatment) who received treatment with the ketogenic diet (KD).
We performed a retrospective case review at 4 medical centers of adult patients with SRSE treated with the KD. Data collected included demographic features, clinical presentation, diagnosis, EEG data, anticonvulsant treatment, and timing and duration of the KD. Primary outcome measures were resolution of status epilepticus (SE) after initiation of KD and ability to wean from anesthetic agents.
Ten adult patients at 4 medical centers were started on the KD for SRSE. The median age was 33 years (interquartile range [IQR] 21), 4 patients (40%) were male, and 7 (70%) had encephalitis. The median duration of SE before initiation of KD was 21.5 days (IQR 28) and the median number of antiepileptic medications used before initiation of KD was 7 (IQR 7). Ninety percent of patients achieved ketosis, and SE ceased in all patients achieving ketosis in a median of 3 days (IQR 8). Three patients had minor complications of the KD including transient acidosis and hypertriglyceridemia and 2 patients ultimately died of causes unrelated to the KD.
We describe treatment of critically ill adult patients with SRSE with the KD, with 90% of patients achieving resolution of SE. Prospective trials are warranted to examine the efficacy of the KD in adults with refractory SE.
This study provides Class IV evidence that for intensive care unit patients with refractory SE, a KD leads to resolution of the SE.
描述重症监护病房中超级难治性癫痫持续状态（SRSE；尽管给予适当的麻醉治疗，但持续状态仍持续 24 小时或更长时间）成年患者的病例系列，这些患者接受了生酮饮食（KD）治疗。
我们在 4 家医疗中心对接受 KD 治疗的 SRSE 成年患者进行了回顾性病例回顾。收集的数据包括人口统计学特征、临床表现、诊断、脑电图数据、抗惊厥治疗以及 KD 的开始时间和持续时间。主要结局指标是 KD 开始后癫痫持续状态（SE）的缓解和麻醉剂的脱除能力。
4 家医疗中心的 10 名成年患者因 SRSE 开始接受 KD。中位年龄为 33 岁（四分位距 [IQR] 21），4 名患者（40%）为男性，7 名（70%）患有脑炎。开始 KD 前 SE 的中位持续时间为 21.5 天（IQR 28），开始 KD 前使用的抗癫痫药物中位数为 7（IQR 7）。90%的患者出现酮症，在中位 3 天（IQR 8）内，所有达到酮症的患者 SE 均停止。3 名患者出现 KD 的轻微并发症，包括短暂酸中毒和高三酰甘油血症，2 名患者最终因与 KD 无关的原因死亡。
我们描述了对患有 SRSE 的重症成年患者使用 KD 的治疗方法，90%的患者 SE 得到缓解。需要进行前瞻性试验来检查 KD 在难治性 SE 成年患者中的疗效。
这项研究提供了 IV 级证据，表明对于难治性 SE 的重症监护病房患者，KD 可导致 SE 缓解。
