Reimer Dennis, Brandl Ulrich, Vries Heike De
Klinik für Neuropädtiatrie, Jena, Germany.
Neuropediatrics. 2025 Apr;56(2):94-101. doi: 10.1055/a-2524-9195. Epub 2025 Jan 27.
Infantile spasms (IS) are an age-specific epilepsy syndrome associated with poor outcomes. Sustained and early spasm control remains the main goal of therapy. We aimed to evaluate a unique pulsatile dexamethasone therapy regime in children with IS.
Children with IS were treated with oral pulsatile-applied dexamethasone in the Children's Hospital Jena between 2002 and 2021, regardless of duration since IS onset or previous therapy (except ACTH). A prolonged initial pulse was given in case of insufficient response (standard: 5-7 days, prolonged: 10-14 days). We analyzed spasm reduction, electroencephalographic response, adverse reactions, neurodevelopmental status, and epileptic disorders at the last follow-up.
Included were 26 patients with a median age of 5.5 months (interquartile range 4-8) at IS onset and a mean follow-up of 6.2 years (standard deviation [SD] 3.99). Fifty percent had an unknown etiology. Patients received on average 10.8 pulses (SD 6.0); 69.2% achieved initial seizure freedom, however, 38.9% relapsed. Seventeen patients had an initial prolonged pulse, of those, 14 got initially seizure-free (82.4%). Sixty-four percent of the cases had a sustained spasm cessation after the third pulse. At the last follow-up, half of the patients had no persisting epileptic disorder; 22.2% had a favorable neurocognitive development. Patients with unknown etiology were more likely to achieve seizure freedom during therapy ( = 0.025), had a more favorable neurocognitive outcome ( = 0.049), and were less likely to suffer from epileptic disorders ( = 0.037). No serious adverse effects were observed.
Our results show that our treatment is safe and leads to outcomes comparable to usually applied hormonal therapy regimes. Etiology remains the most influential factor.
婴儿痉挛症（IS）是一种与不良预后相关的特定年龄癫痫综合征。持续且早期控制痉挛仍是治疗的主要目标。我们旨在评估一种独特的脉冲式地塞米松治疗方案用于IS患儿的效果。
2002年至2021年期间，耶拿儿童医院对IS患儿采用口服脉冲式应用地塞米松进行治疗，无论自IS发作以来的持续时间或先前的治疗情况（促肾上腺皮质激素除外）。若反应不足则给予延长的初始脉冲（标准：5 - 7天，延长：10 - 14天）。我们在最后一次随访时分析了痉挛减少情况、脑电图反应、不良反应、神经发育状况和癫痫疾病。
纳入26例患者，IS发作时的中位年龄为5.5个月（四分位间距4 - 8），平均随访6.2年（标准差[SD] 3.99）。50%的病因不明。患者平均接受10.8次脉冲治疗（SD 6.0）；69.2%的患者最初实现了癫痫发作自由，但38.9%复发。17例患者有初始延长脉冲，其中14例最初无癫痫发作（82.4%）。64%的病例在第三次脉冲后实现了持续的痉挛停止。在最后一次随访时，一半的患者没有持续的癫痫疾病；22.2%的患者神经认知发育良好。病因不明的患者在治疗期间更有可能实现癫痫发作自由（P = 0.025），神经认知结局更有利（P = 0.049），且患癫痫疾病的可能性更小（P = 0.037）。未观察到严重不良反应。
我们的结果表明，我们的治疗是安全的，且导致的结果与通常应用的激素治疗方案相当。病因仍然是最有影响的因素。
