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遗传性癫痫:突变小鼠蹒跚症中的棘波和局灶性运动性癫痫发作

Inherited epilepsy: spike-wave and focal motor seizures in the mutant mouse tottering.

作者信息

Noebels J L, Sidman R L

出版信息

Science. 1979 Jun 22;204(4399):1334-6. doi: 10.1126/science.572084.

DOI:10.1126/science.572084
PMID:572084
Abstract

Mice with the mutant gene tottering (tg, chromosome 8, autosomal recessive) show, in adolescence, abnormal bursts of bilaterally synchronous spike waves as revealed in electrocorticograms recorded over long periods. The spike waves are accompanied by behavioral "absence" attacks and intermittent focal motor seizures showing somatotopic progression. Cerebral metabolic activity during seizures was assayed by autoradiography of brain sections from mice injected intravenously with 14C-labeled 2-deoxyglucose. Metabolic activity was increased bilaterally in selected brainstem structures. Spontaneous electrocorticographic and clinical seizures of this general pattern were recognized hitherto only in humans.

摘要

携带突变基因蹒跚(tg,位于8号染色体,常染色体隐性遗传)的小鼠在青春期会出现异常的双侧同步尖波爆发,这在长时间记录的脑电图中得以揭示。这些尖波伴随着行为性“失神”发作和间歇性局灶性运动性癫痫发作,并呈现躯体定位进展。通过对静脉注射14C标记的2-脱氧葡萄糖的小鼠脑切片进行放射自显影,测定癫痫发作期间的脑代谢活性。在选定的脑干结构中,双侧代谢活性增加。这种一般模式的自发性脑电图和临床癫痫发作此前仅在人类中被识别。

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Inherited epilepsy: spike-wave and focal motor seizures in the mutant mouse tottering.遗传性癫痫:突变小鼠蹒跚症中的棘波和局灶性运动性癫痫发作
Science. 1979 Jun 22;204(4399):1334-6. doi: 10.1126/science.572084.
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