Cerebrospinal fluid ACTH and cortisol in opsoclonus-myoclonus: Effect of therapy
Cerebrospinal fluid ACTH and cortisol in opsoclonus-myoclonus: Effect of therapy
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DOI：
10.1016/j.pediatrneurol.2005.03.004
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发表时间：
2005-08-01
影响因子：
3.8
通讯作者：
Allison, TJ
中科院分区：
文献类型：
--
作者：
Pranzatelli, MR;Chun, KY;Allison, TJ
眼阵挛-肌阵挛综合征是少数几种儿童促肾上腺皮质激素（ACTH）反应性中枢神经系统疾病之一。我们测量了69名眼阵挛-肌阵挛儿童和25名年龄和性别匹配的对照组的脑脊液ACTH和皮质醇，以确定内源性水平，并寻找ACTH和皮质类固醇治疗的假设差异激素效应。脑脊液皮质醇是10倍高ACTH治疗（n = 26），但与口服类固醇治疗（n = 18）或无治疗（n = 25）不变。每天接受高剂量ACTH的儿童比隔日接受ACTH的儿童显著更高。在ACTH治疗的儿童中，脑脊液和血清皮质醇高度相关（r = 0.96，P = 0.0001），脑脊液与血清皮质醇的平均比值约为1：10。脑脊液ACTH浓度在未治疗的眼阵挛-肌阵挛和对照受试者之间没有显著差异，但ACTH（-29%）或类固醇治疗（-36%）较低，表明ACTH释放的反馈抑制。这些数据描述了ACTH和皮质类固醇治疗的中枢效应的差异，以及ACTH高剂量和低剂量之间的差异，并支持儿童眼阵挛-肌阵挛中脑肾上腺轴的完整性。(c)2005年，Elsevier Inc. All rights reserved.
Opsoclonus-myoclonus syndrome is one of a few corticotropin (ACTH) -responsive central nervous system disorders of childhood. We measured cerebrospinal fluid ACTH and cortisol in 69 children with opsoclonus-myoclonus and 25 age- and sex-matched control subjects to determine endogenous levels and look for hypothesized differential hormonal effects of ACTH and corticosteroid treatment. Cerebrospinal fluid cortisol was 10-fold higher with ACTH treatment (n = 26), but was unchanged with oral steroid treatment (n = 18) or no treatment (n = 25). It was significantly higher in children receiving daily high-dose ACTH than alternate day ACTH. In ACTH-treated children, cerebrospinal fluid and serum cortisol were highly correlated (r = 0.96, P = 0.0001), with a mean ratio of cerebrospinal fluid to serum cortisol of approximately 1:10. Cerebrospinal fluid ACTH concentration did not differ significantly between untreated opsoclonus-myoclonus and control subjects but was lower with ACTH (-29%) or steroid treatment (-36%), suggesting feedback inhibition of ACTH release. These data delineate differences in the central effects of ACTH and corticosteroid therapy, as well as between high and low ACTH doses, and support the integrity of the brain-adrenal axis in pediatric opsoclonus-myoclonus. (c) 2005 by Elsevier Inc. All rights reserved.