Skarpa D, Barisić N, Bulat M
Klinika za djecje bolesti Rebro Medicinskog fakulteta Sveucilista u Zagrebu.
Lijec Vjesn. 1994 May-Jun;116(5-6):131-5.
Monozygotic twin sisters who had almost identical electroencephalographic abnormalities, but different clinical features and different response of this abnormalities to valproate at the age of 6 years are described. One twin was admitted to the hospital because of numerous brief myotonic seizures of sudden onset with corresponding abnormalities in the electroencephalogram. The seizures disappeared completely and the electroencephalogram became normal within a week of treatment with valproate. The other twin never had any seizures. However, her electroencephalogram repeatedly showed impressive abnormalities and the therapy with valproate had no substantial effect on these abnormalities. We believe this twins to be a suitable model for studying the inheritance of epilepsy and electroencephalographic abnormalities, as well as for studying the action of antiepileptic drugs. At this moment, it is not possible to offer any reasonable explanation for the normalization of the electroencephalographic abnormality during valproate treatment in the clinically affected twin, while the electroencephalogram remained abnormal in the clinically unaffected twin.
本文描述了一对单卵双胞胎姐妹，她们在6岁时脑电图异常几乎相同，但临床特征不同，且这些异常对丙戊酸盐的反应也不同。其中一个双胞胎因突然发作的大量短暂肌阵挛性癫痫发作入院，脑电图有相应异常。使用丙戊酸盐治疗一周内，癫痫发作完全消失，脑电图恢复正常。另一个双胞胎从未有过任何癫痫发作。然而，她的脑电图反复显示出明显异常，丙戊酸盐治疗对这些异常没有实质性影响。我们认为这对双胞胎是研究癫痫和脑电图异常遗传以及抗癫痫药物作用的合适模型。目前，对于临床上受影响的双胞胎在丙戊酸盐治疗期间脑电图异常恢复正常，而临床上未受影响的双胞胎脑电图仍异常这一现象，无法给出任何合理的解释。
