Doose H, Gerken H, Kiefer R, Völzke E
Neuropadiatrie. 1977 Feb;8(1):10-20. doi: 10.1055/s-0028-1091500.
Electroencephalographical investigations were performed in 203 epileptic children exhibiting focal sharp waves in the EEG and in 312 siblings. As controls served 685 brain-healthy children (Gerken 1971) as well as 252 children with spike wave absences and their 242 siblings (Doose et al. 1973). According to the EEG findings in probands so-called "centrencephalic" EEG-criteria (theta rhythms, spikes and waves, photosensitivity) as a symptom of a genetically determined seizure susceptibility can be expected in at least 60% of the probands. 18% of the siblings showed at least once one definite "centrencephalic" EEG pattern. These results must be regarded as minimal values, since in siblings only one electro-encephalographic study could be performed. The results make it evident, that in the pathogenesis of epilepsy with focal sharp waves a genetically determined seizure susceptibility plays a very important role. After comparison with the results of genetic investigations in patients with spike wave absences (Doose et al. 1973) the difference as for the genetic basis between "centrencephalic" and focal epilepsies apparently is only due to a different gene density.
对203名脑电图显示局灶性锐波的癫痫患儿及其312名兄弟姐妹进行了脑电图检查。对照组包括685名脑健康儿童(格肯,1971年)以及252名无棘波的儿童及其242名兄弟姐妹(多泽等人,1973年)。根据先证者的脑电图结果,至少60%的先证者可预期出现所谓的“中央脑性”脑电图标准(θ节律、棘波和慢波、光敏感性),作为遗传决定的癫痫易感性的症状。18%的兄弟姐妹至少有一次出现明确的“中央脑性”脑电图模式。这些结果必须被视为最小值,因为对兄弟姐妹仅进行了一次脑电图研究。结果表明,在伴有局灶性锐波的癫痫发病机制中,遗传决定的癫痫易感性起着非常重要的作用。与无棘波患者的遗传研究结果(多泽等人,1973年)进行比较后,“中央脑性”癫痫和局灶性癫痫在遗传基础上的差异显然仅归因于不同的基因密度。