Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone
Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone
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DOI：
10.1111/j.0013-9580.2004.30503.x
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发表时间：
2004-03-01
期刊：
影响因子：
5.6
通讯作者：
Shinnar, S
中科院分区：
文献类型：
--
作者：
Kivity, S;Lerman, T;Shinnar, S
目的：评价隐源性婴儿痉挛症患儿应用高密合成促肾上腺皮质激素（ACTH）治疗的疗效，以及发病1个月内早期治疗与疗效的关系。方法：我们评估了37名隐源性婴儿痉挛患者的长期认知和癫痫发作结果（发病，a-e 3至9个月）接受高剂量二十四碳丙交酯贮库的标准化治疗方案，每48小时1 mg IM，持续2周。随后8- 10周缓慢减量，然后口服泼尼松。10 mg/天，持续1个月，随后缓慢减量，持续5个月或直至婴儿满1岁，以较晚者为准。治疗前评估发育情况。前瞻性随访癫痫发作结局。认知结果确定后6至2 - 1年，并在治疗滞后和治疗前regulation.Results：22名婴儿进行了治疗婴儿痉挛症发病后1个月内，15后1至6.5个月。在早期治疗组的所有22例（100%）患者中发现了正常的认知结果，在晚期治疗组中发现了40%的患者。正常的认知结果被发现在所有25（100%）的患者没有或只有轻度的精神恶化在介绍，包括4个在后期治疗组，但在12例患者中只有3个谁有显着或严重恶化前treatment.Conclusions：早期治疗隐源性婴儿痉挛症与高剂量ACTH协议是有利的长期认知结果。一旦严重的发育退化持续一个月或更长时间。正常认知结果的预后很差。需要进一步的研究油的最佳治疗方案，这种疾病。
Purpose: To evaluate the outcome of children with cryptogenic infantile spasms treated with high-close synthetic adrenocorticotropic hormone (ACTH) and the relation between early treatment, within 1 month of onset, and outcome.Methods: We assessed the long-term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, a-e 3 to 9 months) receiving standardized treatment regimen of hi-h-dose tetracosactide depot, 1 mg IM every 48 h for 2 weeks. with a subsequent 8- to 10-week slow taper and followed by oral prednisone. 10 mg/day for a month, with a subsequent slow taper for 5 Months Or until the infant reached the age of 1 year, whichever came later. Development was assessed before treatment. Seizure outcomes were followed up prospectively. Cognitive outcomes were determined after 6 to 2 1 years and analyzed in relation to treatment lag and pretreatment regression.Results: Twenty-two infants were treated within 1 month of onset of infantile spasms, and 15 after 1 to 6.5 months. Normal cognitive outcome was found in all 22 (100%) patients of the early-treatment group, and in 40% of the late-treatment group. Normal cognitive outcome was found in all 25 (100%) patients who had no or only mild mental deterioration at presentation, including four in the late-treatment group but in only three of the 12 patients who had had marked or severe deterioration before treatment.Conclusions: Early treatment of cryptogenic infantile spasms with a high-dose ACTH protocol is associated with favorable long-term cognitive outcomes. Once major developmental regression lasts for a month or more. the prognosis for normal cognitive outcome is poor. Further studies are needed oil the optimal treatment regimen for this disorder.