Vadlamudi L, Andermann E, Lombroso C T, Schachter S C, Milne R L, Hopper J L, Andermann F, Berkovic S F
Epilepsy Research Centre, University of Melbourne, Austin Health, Heidelberg West, Victoria, Australia.
Neurology. 2004 Apr 13;62(7):1127-33. doi: 10.1212/01.wnl.0000118201.89498.48.
To classify the Lennox twin pairs according to modern epilepsy classifications, use the classic twin model to identify which epilepsy syndromes have an inherited component, search for evidence of syndrome-specific genes, and compare concordances from Lennox's series with a contemporary Australian series.
Following review of Lennox's original files describing twins with seizures from 1934 through 1958, the International League Against Epilepsy classifications of seizures and epileptic syndromes were applied to 169 pairs. Monozygous (MZ) and dizygous (DZ) pairs were subdivided into epilepsy syndromes and casewise concordances estimated.
The authors excluded 26 pairs, with 71 MZ and 72 DZ pairs remaining. Seizure analysis demonstrated strong parallels between contemporary seizure classification and Lennox's terminology. Epilepsy syndrome diagnoses were made in 75%. The MZ and DZ casewise concordance estimates gave strong evidence for a major genetic influence in idiopathic generalized epilepsies (0.80 versus 0.00; n = 23). High MZ casewise concordances also supported a genetic etiology in symptomatic generalized epilepsies and febrile seizures. The pairs who were concordant for seizures usually had the same syndromic diagnoses in both twins (86% in MZ, 60% in DZ), suggesting syndrome-specific genes. Apart from partial epilepsies, the MZ casewise concordances were similar to those derived from Australian twin data.
The authors were able to apply contemporary classifications to Lennox's twins. The data confirm genetic bases for common generalized epilepsies as well as febrile seizures and provide further support for syndrome-specific genes. Finally, comparable results to our Australian series were obtained, verifying the value of twin studies.
根据现代癫痫分类对伦诺克斯双胞胎对进行分类，使用经典双胞胎模型确定哪些癫痫综合征具有遗传成分，寻找综合征特异性基因的证据，并将伦诺克斯系列的一致性与当代澳大利亚系列进行比较。
在回顾了伦诺克斯1934年至1958年描述癫痫发作双胞胎的原始档案后，将国际抗癫痫联盟的癫痫发作和癫痫综合征分类应用于169对双胞胎。将同卵（MZ）和异卵（DZ）双胞胎对细分为癫痫综合征，并估计逐例一致性。
作者排除了26对，剩余71对MZ和72对DZ。癫痫发作分析表明当代癫痫发作分类与伦诺克斯的术语之间有很强的相似性。75%的双胞胎做出了癫痫综合征诊断。MZ和DZ逐例一致性估计为特发性全身性癫痫的主要遗传影响提供了有力证据（0.80对0.00；n = 23）。高MZ逐例一致性也支持症状性全身性癫痫和热性惊厥的遗传病因。癫痫发作一致的双胞胎对通常在两个双胞胎中都有相同的综合征诊断（MZ中为86%，DZ中为60%），提示存在综合征特异性基因。除部分性癫痫外，MZ逐例一致性与来自澳大利亚双胞胎数据的结果相似。
作者能够将当代分类应用于伦诺克斯的双胞胎。数据证实了常见全身性癫痫以及热性惊厥的遗传基础，并为综合征特异性基因提供了进一步支持。最后，获得了与我们澳大利亚系列可比的结果，验证了双胞胎研究的价值。
