Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, United States.
Neuropediatrics. 2020 Jun;51(3):225-228. doi: 10.1055/s-0039-3402005. Epub 2020 Feb 3.
Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder and characterized by infantile onset hemiplegia involving either side of the body and other paroxysmal spells, including epilepsy. The N-methyl-D-aspartate (NMDA) receptor noncompetitive antagonist ketamine has been proved to be effective terminating status epilepticus (SE) in animal models and human case studies. Less than 50 cases, those use ketamine, have been reported in the management of pediatric SE, but its effectiveness is unknown in patients with AHC. The author reported two patients with molecularly confirmed AHC who presented with super refractory status epilepticus. These two patients failed multiple antiepileptic drugs, including midazolam infusion and propofol but responded promptly to ketamine infusion. This is the first description of the effectiveness of ketamine as an effective third-line agent for super refractory SE associated with AHC. Increased glutamate-mediated neurotoxicity can be present in AHC, not only due to overexpression of glutamate receptors during status epilepticus but also due to unique characteristics of AHC-preferential inactivity of GABAergic interneuron and impaired action of glutamate transporters (EAAT-1). Moreover, as neuroregression is possible after SE in AHC, earlier termination of SE by using ketamine can be an effective option in this population. Further prospective, controlled studies are needed to confirm the finding of this study.
儿童交替性偏瘫（AHC）是一种罕见的神经发育障碍，其特征为婴儿起病的偏瘫累及身体的任一侧，以及其他发作性发作，包括癫痫。N-甲基-D-天冬氨酸（NMDA）受体非竞争性拮抗剂氯胺酮已被证明在动物模型和人类病例研究中可有效终止癫痫持续状态（SE）。在儿科 SE 的治疗中，已有不到 50 例使用氯胺酮的病例报告，但在 AHC 患者中的疗效尚不清楚。作者报告了两例经分子证实的 AHC 患者，他们表现为超难治性 SE。这两例患者对多种抗癫痫药物（包括咪达唑仑输注和丙泊酚）均无反应，但对氯胺酮输注反应迅速。这是首例描述氯胺酮作为治疗与 AHC 相关的超难治性 SE 的有效三线药物的报道。谷氨酸介导的神经毒性增加可能存在于 AHC 中，这不仅是由于 SE 期间谷氨酸受体过度表达，还由于 AHC 的独特特征——GABA 能中间神经元优先失活和谷氨酸转运体（EAAT-1）功能受损。此外，由于 SE 后可能出现神经退行性变，因此在该人群中及早使用氯胺酮终止 SE 可能是一种有效的选择。需要进一步前瞻性、对照研究来证实本研究的发现。
