Child Neurology Division, Department of Pediatrics.
Division of Neuroradiology and Pediatric Radiology, University of Arkansas for Medical Sciences, Little Rock, AR.
Clin Neuropharmacol. 2021;44(1):23-26. doi: 10.1097/WNF.0000000000000420.
Alternating hemiplegia of childhood (AHC) is a neurological disorder with early-onset alternating hemiplegia and other paroxysmal events such as epilepsy and dystonia due to de novo pathogenic mutations in the ATP1A3. Physicians and scientists investigated several agents in the treatment without strong evidence of definitive long-term benefit. Knowledge regarding utility of anti-inflammatory agents in the treatment is scarce except the anecdotal report of corticosteroid use. We described 2 patients with AHC who were exposed to intravenous immunoglobulin (IVIG) treatment because of an alternative diagnosis. An 8-year-old girl received 4 years of periodic IVIG infusion and was free of paroxysmal events during the first 16 months of therapy. A 2-year-old boy received IVIG infusion for 10 months and remained seizure-free for 2 years since the beginning of the treatment, but without a definite change in hemiplegic episodes. Our report is the first description of IVIG use in patients with AHC. Although these patients did not achieve complete remission, partial responsiveness was noted. Although the definite role of IVIG in the treatment of AHC cannot be extrapolated from our study, further prospective controlled studies should be done because of the recent discovery of the connection between an anti-inflammatory hormone (Oubain) and Na+/K+ ATPase.
儿童交替性偏瘫（AHC）是一种神经系统疾病，具有早期发作的交替性偏瘫和其他阵发性事件，如癫痫和由于 ATP1A3 中新发致病性突变引起的肌张力障碍。医生和科学家研究了几种治疗药物，但没有确凿的长期获益证据。除了皮质类固醇使用的传闻报告外，关于抗炎药物在治疗中的应用的知识很少。我们描述了 2 名因其他诊断而接受静脉注射免疫球蛋白（IVIG）治疗的 AHC 患者。一名 8 岁女孩接受了 4 年的周期性 IVIG 输注，在治疗的前 16 个月没有阵发性事件。一名 2 岁男孩接受了 10 个月的 IVIG 输注，自治疗开始以来 2 年内无癫痫发作，但偏瘫发作无明显变化。我们的报告是首例 AHC 患者使用 IVIG 的描述。尽管这些患者没有达到完全缓解，但观察到部分反应。尽管不能从我们的研究中推断出 IVIG 在 AHC 治疗中的明确作用，但由于最近发现抗炎激素（Oubain）和 Na+/K+ATP 酶之间存在联系，应该进行进一步的前瞻性对照研究。
