Forty-six children with refractory epilepsy (12 with symptomatic generalized epilepsy, 14 with symptomatic partial epilepsy, and 20 with undetermined epilepsy) were treated by high-dose (serum level above 100 micrograms/ml) valproate (VPA) therapy. Monotherapy was used with 34 patients and two drugs with 12. Serum VPA concentrations ranged from 105.1 to 198.4 micrograms/ml. Assessment of initial response to treatment, after the serum level had reached the appropriate level, showed seizures to be completely controlled in 15 (32.6%) of 46 patients and improved in 12 (26.1%) (50% or more). Follow-up of more than 6 months after the time of initial response showed control of seizures in 14 (30.4%) and improvement in 11 (23.9%). The initial effect on EEG was the disappearance of epileptic discharges in 3 (6.5%) of 46 patients and marked improvement in 15 (32.6%). Follow-up revealed the disappearance of epileptic discharges in 7 (15.2%) and marked improvement in 9 patients (19.6%). High-dose VPA therapy was especially effective for West syndrome and for epilepsy with continuous spike-waves during slow-wave sleep. Control of atypical absences and myoclonic seizures was relatively good. Hypofibrinogenemia and thrombocytopenia were sometimes encountered but these side effects were reversible with reduction of dosage.

译文

46例难治性癫痫患儿 (12例症状性全身性癫痫,14例症状性部分性癫痫,20例未确定癫痫) 接受大剂量 (血清水平高于100微克/毫升) 丙戊酸钠 (VPA) 治疗。单药治疗34例,两种药物12例。血清VPA浓度范围为105.1至198.4微克/毫升。在血清水平达到适当水平后,对治疗的初始反应的评估显示,46例患者中有15例 (32.6% 例) 的癫痫发作得到完全控制,而12例 (26.1% 例) (50% 例或以上) 的癫痫发作得到改善。初始反应时间后超过6个月的随访显示14例 (30.4%) 癫痫发作得到控制,11例 (23.9%) 改善。对EEG的最初影响是46例患者中有3例 (6.5% 例) 癫痫放电消失,而15例 (32.6% 例) 明显改善。随访显示7例 (15.2% 例) 癫痫放电消失,9例 (19.6% 例) 明显改善。大剂量VPA治疗对West综合征和慢波睡眠期间持续尖峰波的癫痫特别有效。非典型缺勤和肌阵挛发作的控制相对较好。有时会遇到低纤维蛋白原血症和血小板减少症,但随着剂量的减少,这些副作用是可逆的。

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