Renier W O, Le Coultre R
Interdisciplinair Kinderneurologisch Centrum, Sint Radboudziekenhuis, Nijmegen.
Tijdschr Kindergeneeskd. 1989 Jun;57(3):81-6.
The authors have evaluated critically the results of ACTH and ketogenic diet in 122 cases of 'malignant' childhood epilepsies, especially West-syndrome (WS) and Lennox-Gastaut-syndrome (LGS). In agreement with data from the literature about 10% of the idiopathic forms recovered with or without ACTH. In the other cases ACTH caused a transient amelioration of the EEG and/or the spasms; a high-dosage regimen of ACTH was not significantly better than a low-dosage regimen. Fatal complications only occurred in the high-dosage ACTH group. Ketogenic diet in 10 children with LGS seems to have an influence on the LGS-specific seizures but not on the other signs and symptoms of the syndrome. The diet is difficult to maintain and is not free of side-effects.
作者对122例儿童“恶性”癫痫,尤其是韦斯特综合征(WS)和伦诺克斯 - 加斯东综合征(LGS)应用促肾上腺皮质激素(ACTH)和生酮饮食的结果进行了严格评估。与文献数据一致,约10%的特发性癫痫形式无论是否使用ACTH均有恢复。在其他病例中,ACTH使脑电图和/或痉挛得到短暂改善;高剂量ACTH方案并不比低剂量方案显著更好。致命并发症仅发生在高剂量ACTH组。10例LGS患儿采用生酮饮食似乎对LGS特异性癫痫发作有影响,但对该综合征的其他体征和症状无影响。这种饮食难以维持且并非没有副作用。