Model of cryptogenic infantile spasms after prenatal corticosteroid priming.
Model of cryptogenic infantile spasms after prenatal corticosteroid priming.
复制标题
产前皮质类固醇启动后隐源性婴儿痉挛模型。
DOI：
10.1111/j.1528-1167.2010.02630.x
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发表时间：
2010
期刊：
影响因子：
5.6
通讯作者：
Velísková,Jana
中科院分区：
文献类型：
--
作者：
Velísek,Libor;Chachua,Tamar;Yum,Mi-Sun;Poon,Ka-Lai;Velísková,Jana
婴儿痉挛是一种严重的儿童期癫痫综合征。IS发生在3-12个月大的婴儿身上，以痉挛、发作间期脑电(EEG)节律失常和严重的智力低下为特征。激素疗法[促肾上腺皮质激素(ACTH)，皮质类固醇]是常用的治疗方法，但其疗效受到严重副作用的影响。对于新疗法的研究，需要一个经过验证的IS动物模型。我们建立了胎儿期暴露于倍他米松的幼鼠痉挛发作触发Byn-甲基-d-天冬氨酸(NMDA)的模型。痉挛与人类IS有显著的相似性，包括运动屈曲痉挛、发作期脑电减退和对ACTH的反应性。有趣的是，痉挛并不涉及海马体。放射自显影的代谢图谱以及用c-fos标记的神经元兴奋区域表明，下丘脑结构，如弓状核，与其他下丘脑核团以及脑干有显著的双边联系。
Infantile spasms (IS) is a devastating epilepsy syndrome of childhood. IS occurs in 3–12‐month‐old infants and is characterized by spasms, interictal electroencephalography (EEG) hypsarrhythmia, and profound mental retardation. Hormonal therapy [adrenocorticotropic hormone (ACTH), corticosteroids] is frequently used, but its efficacy is tainted by severe side effects. For research of novel therapies, a validated animal model of IS is required. We propose the model of spastic seizures triggered byN‐methyl‐d‐aspartate (NMDA) in infant rats prenatally exposed to betamethasone. The spasms have remarkable similarity to human IS, including motor flexion spasms, ictal EEG electrodecrement, and responsiveness to ACTH. Interestingly, the spasms do not involve the hippocampus. Autoradiographic metabolic mapping as well as tagging of the areas of neuronal excitation with c‐fos indicates a strong involvement of hypothalamic structures such as the arcuate nucleus, which has significant bilateral connections with other hypothalamic nuclei as well as with the brainstem.