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Early developmental electroencephalography abnormalities, neonatal seizures, and induced spasms in a mouse model of tuberous sclerosis complex
Epilepsia ( IF 5.6 ) Pub Date : 2020-04-10 , DOI: 10.1111/epi.16495
Nicholas Rensing 1 , Kevin J Johnson 1 , Thomas J Foutz 1 , Joseph L Friedman 1 , Rafael Galindo 1 , Michael Wong 1
Affiliation  

Tuberous sclerosis complex (TSC) is one of the most common genetic causes of epilepsy. Seizures in TSC typically first present in infancy or early childhood, including focal seizures and infantile spasms. Infantile spasms in TSC are particularly characteristic in its strong responsiveness to vigabatrin. Although a number of mouse models of epilepsy in TSC have been described, there are very limited electroencephalographic (EEG) or seizure data during the preweanling neonatal and infantile‐equivalent mouse periods. Tsc1GFAPCKO mice are a well‐characterized mouse model of epilepsy in TSC, but whether these mice have seizures during early development has not been documented. The objective of this study was to determine whether preweanling Tsc1GFAPCKO mice have developmental EEG abnormalities or seizures, including spasms.

中文翻译:

结节性硬化症小鼠模型的早期发育性脑电图异常、新生儿癫痫发作和诱发痉挛

结节性硬化症 (TSC) 是癫痫最常见的遗传原因之一。TSC 的癫痫发作通常首先出现在婴儿期或儿童早期,包括局灶性癫痫发作和婴儿痉挛。TSC 中婴儿痉挛的特点是其对氨己烯酸的强烈反应。尽管已经描述了许多 TSC 癫痫小鼠模型,但在断奶前的新生儿和婴儿等效小鼠期间,脑电图 (EEG) 或癫痫发作数据非常有限。Tsc1GFAPCKO 小鼠是 TSC 中具有良好特征的癫痫小鼠模型,但这些小鼠在早期发育过程中是否有癫痫发作尚未被记录。本研究的目的是确定断奶前的 Tsc1GFAPCKO 小鼠是否有发育性脑电图异常或癫痫发作,包括痉挛。
更新日期:2020-04-10
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