Pediatric Neurology Department, Ospedali Riuniti, Ancona, Italy.
Eur J Paediatr Neurol. 2011 Jan;15(1):8-14. doi: 10.1016/j.ejpn.2010.09.003. Epub 2010 Oct 23.
Medical treatment of Dravet syndrome is disappointing. Ketogenic Diet and neurostimulation procedures as Vagus Nerve Stimulation (VNS) and Deep Brain Stimulation are in ongoing evaluation. In the present study, the long-term effectiveness of VNS on seizures, cognition and behavior was retrospectively evaluated in eight young patients with DS and medically refractory epilepsy (mean age at VNS implant: 10.28 years, range: 5-25). The average duration of treatment was 54 months (range: 12-120). Compared to baseline (mean: 55; standard deviation: 83, range: 4-200), the mean number of monthly seizures after VNS implantation was 39 ± 67 at 3 months, 42 ± 67 at 6 months and 38 ± 69 at twelve months (not significant comparisons). In particular, VNS produced a mean seizure rate reduction of 12% at three months, 6% at six months, and 31% at twelve months. All patients but three experienced some reduction in seizure burden (range: 33-61%) at twelve months. Seizure outcome after one year of stimulation was rated as Mc Hugh class II (50-79% reduction in seizure frequency) in four patients, class III (<50% reduction) in one patient and class V (no improvement) in three patients. In this small case series of patients with DS, VNS therapy had a clinically significant effect in reducing seizures at twelve months in four of the eight patients. Even in patients in whom seizure reduction was not dramatic, a slight improvement in alertness and communicative skills was seen. The long-term clinical course of two selected cases is discussed.
Dravet 综合征的治疗效果并不理想。生酮饮食和神经刺激疗法，如迷走神经刺激（Vagus Nerve Stimulation，VNS）和深部脑刺激（Deep Brain Stimulation），正在进行评估。在本研究中，回顾性评估了 8 例患有 Dravet 综合征和药物难治性癫痫的年轻患者（VNS 植入时的平均年龄为 10.28 岁，范围为 5-25 岁）的 VNS 对癫痫发作、认知和行为的长期疗效。治疗的平均持续时间为 54 个月（范围：12-120 个月）。与基线相比（平均值：55；标准差：83，范围：4-200），VNS 植入后 3 个月、6 个月和 12 个月的每月癫痫发作次数平均值分别为 39±67、42±67 和 38±69（无显著差异）。特别是，VNS 在 3 个月、6 个月和 12 个月时分别使癫痫发作率降低了 12%、6%和 31%。所有患者（除了 3 例）在 12 个月时癫痫发作负担均有一定程度的减轻（范围：33%-61%）。1 年后的刺激治疗结果，4 例患者被评定为 McHugh 分级 II（癫痫发作频率降低 50%-79%），1 例患者为 III 级（降低<50%），3 例患者为 V 级（无改善）。在这组 8 例 Dravet 综合征患者的小病例系列中，VNS 治疗在 4 例患者中在 12 个月时对癫痫发作有显著的临床效果。即使在癫痫发作减少不显著的患者中，也观察到警觉性和沟通技能略有改善。讨论了 2 例选定病例的长期临床病程。
