Marescaux C, Vergnes M
INSERM U 398, Hôpitaux Universitaires, Strasbourg, France.
Ital J Neurol Sci. 1995 Feb-Mar;16(1-2):113-8. doi: 10.1007/BF02229083.
In Genetic Absence Epilepsy Rats from Strasbourg (GAERS), 100% of the animals present recurrent generalized non-convulsive seizures characterized by bilateral and synchronous spike-and-wave discharges (SWD) accompanied with behavioural arrest, staring and sometimes twitching of the vibrissae. Spontaneous SWD (7-11 cps) start and end abruptly on a normal background EEG at a mean frequency of 1.5 per min. Drugs effective against absence seizures in humans suppress the SWD dose-dependently, whereas drugs specific for convulsive or focal seizures are ineffective. Depth EEG recordings and lesion experiments show that SWD in GAERS depend on cortical and thalamic structures with a possible rhythmic triggering by the lateral thalamus; GABAA and GABAB receptors seem to play a critical role. SWD are genetically determined with an autosomal dominant inheritance. The variable expression of SWD in offspring from GAERS and control reciprocal crosses may be due to the existence of multiple genes.
在来自斯特拉斯堡的遗传性失神癫痫大鼠（GAERS）中，100%的动物会出现反复的全身性非惊厥性癫痫发作，其特征为双侧同步的棘波-慢波放电（SWD），伴有行为停止、凝视，有时还有触须抽搐。自发性SWD（7 - 11赫兹）在正常背景脑电图上突然开始和结束，平均频率为每分钟1.5次。对人类失神性癫痫有效的药物可剂量依赖性地抑制SWD，而对惊厥性或局灶性癫痫特效的药物则无效。深度脑电图记录和损伤实验表明，GAERS中的SWD依赖于皮质和丘脑结构，外侧丘脑可能存在节律性触发作用；GABAA和GABAB受体似乎起着关键作用。SWD由遗传决定，呈常染色体显性遗传。GAERS与对照反向杂交后代中SWD的可变表达可能是由于多个基因的存在。
