From the Division of Pediatric Neurology (R.T.V., S.K., S.V.K.), Department of Pediatrics, Cohen Children's Medical Center, New Hyde Park, NY; and Departments of Neurology (A.P.), Boston Children's Hospital and Harvard Medical School, Boston, MA.
Neurology. 2022 Aug 2;99(5):208-211. doi: 10.1212/WNL.0000000000200855. Epub 2022 Jun 3.
-related epilepsy is a developmental and epileptic encephalopathy typically presenting with epilepsy and varying degrees of intellectual disability. Seizures typically present in clusters of focal or generalized seizures, sometimes in the setting of fever. We present the case of a 7-month-old girl presenting with new-onset refractory status epilepticus that followed routine vaccine administration and ensuing cytokine storm. She was diagnosed with a pathogenic variant in The patient required 5 antiseizure medications and pentobarbital-induced burst suppression for control of seizures. She was noted to have elevated serum cytokine levels (interleukin [IL]-2, IL-4, IL-10, IL-13, IL-17, IL-1, IL-1β, and IL-8) and CSF cytokine levels (IL-6 and IL-13). Anakinra was initiated and titrated based on serial cytokine levels, with doses ranging from 5 to 20 mg/kg/d resulting in reduction in cytokine levels and seizure reduction. By age 14 months, she was able to be maintained on 3 active antiseizure medications and ketogenic diet for seizure control.
与疫苗接种相关的癫痫是一种发育性和癫痫性脑病，通常以癫痫和不同程度的智力残疾为特征。发作通常以局灶性或全面性发作群集的形式出现，有时伴有发热。我们报告了一例 7 个月大的女孩，在常规疫苗接种后出现新发性难治性癫痫持续状态，随后出现细胞因子风暴。她被诊断为致病性变异体。患者需要 5 种抗癫痫药物和戊巴比妥诱导的爆发抑制来控制癫痫发作。她的血清细胞因子水平（白细胞介素[IL]-2、IL-4、IL-10、IL-13、IL-17、IL-1、IL-1β 和 IL-8）和 CSF 细胞因子水平（IL-6 和 IL-13）升高。根据细胞因子水平，开始并滴定使用阿那白滞素，剂量范围为 5 至 20mg/kg/d，导致细胞因子水平降低和癫痫发作减少。到 14 个月大时，她能够通过 3 种活性抗癫痫药物和生酮饮食来控制癫痫发作。
