Abstract：
Alternating hemiplegia of childhood (AHC) is characterized by recurrent episodes of hemiplegia which may alternate sides between attacks. The condition is associated with severe neurodevelopmental disorder presenting in early infancy, and may encompass a wide range of other paroxysmal manifestations (e.g., dystonia, nystagmus, dysautonomia) and pervasive neurological disabilities (e.g., developmental delay, learning disabilities, choreoathetosis, and ataxia). Epileptic seizures are particularly common among patients with AHC. Diagnosis is usually based on history and clinical grounds using the Aicardi criteria. Mutations in the ATP1A3 gene are implicated in the disease pathology of the condition, as well as several other neurodevelopmental disorders, suggesting AHC forms part of a spectrum of overlapping clinical syndromes rather than a distinct clinical entity per se. Management of patients with AHC includes the rapid induction of sleep during paroxysmal attacks and the avoidance of identified triggers. Pharmacotherapeutic treatments have a role in managing epileptic seizures, as well as in the prevention of paroxysmal attacks wherein flunarizine remains the treatment of choice.
摘要：
儿童交替性偏瘫（AHC）的特征是偏瘫反复发作，可能在两次发作之间交替发作。这种情况与婴儿期早期出现的严重神经发育障碍有关，并可能涵盖广泛的其他阵发性表现(例如，肌张力障碍,眼球震颤,自主神经功能障碍)和广泛性神经残疾(例如，发育迟缓,学习障碍,卵巢狭窄症,和共济失调）。癫痫发作在AHC患者中特别常见。诊断通常基于使用Aicardi标准的病史和临床依据。ATP1A3基因的突变与疾病的病理有关，以及其他几种神经发育障碍，提示AHC构成重叠临床综合征谱的一部分，而不是一个独特的临床实体本身。AHC患者的管理包括在阵发性发作期间快速诱导睡眠和避免确定的触发因素。药物治疗在控制癫痫发作中起作用，以及预防阵发性发作，其中氟桂利嗪仍然是首选治疗方法。
