Department of Neurological Sciences and Molecular, Federico II University, Naples, Italy.
Eur J Neurol. 2010 May;17(5):666-71. doi: 10.1111/j.1468-1331.2009.02896.x. Epub 2009 Dec 7.
The growth hormone (GH) and insulin-like growth factor (IGF) system may be involved in neurodegenerative processes, and some abnormalities have been reported in amyotrophic lateral sclerosis (ALS). Our aim was to investigate the GH-IGF axis in patients with ALS and evaluate correlations between this endocrine system and clinical features.
Serum levels of GH, IGF-I, IGF-II, insulin, IGF-binding protein 1 (IGF-BP1), and IGF-binding protein 3 (IGF-BP3) were measured in 25 patients with ALS and 25 age-, gender-, and BMI-matched healthy controls. A GHRH plus arginine test was performed in patients and controls. Clinical status of patients was evaluated with the ALS Functional Rating Scale - Revised (ALSFRS-R) and upper motor neuron (UMN) score.
GHRH plus arginine test showed GH deficiency (GHD) in 13 (52%) patients with ALS; severe GHD was found in 6 (24%) and partial GHD in 7 (28%) patients. IGF-I levels were significantly higher in patients with ALS than in healthy controls (182.9 +/- 90.8 vs. 139.4 +/- 58.1 ng/ml; P = 0.015). IGF-I levels were higher in patients with ALS with UMN score >10 than those with UMN score <10 (217.8 +/- 100.8 vs. 155.5 +/- 74.6 ng/ml, P = 0.05). IGF-II levels were significantly lower in patients with ALS than in healthy controls (720.9 +/- 215 vs. 1001.9 +/- 475.4 ng/ml; P = 0.03).
The results demonstrate an impairment of the GH-IGFs system in ALS. The degenerative process in ALS might lead to a compensatory increase in IGF-I in an attempt to provide additional support to motor neurons or degenerating muscle fibers. The decrease in IGF-II levels may also be of pathological significance.
生长激素（GH）和胰岛素样生长因子（IGF）系统可能参与神经退行性过程，一些异常已在肌萎缩侧索硬化症（ALS）中报道。我们的目的是研究 ALS 患者的 GH-IGF 轴，并评估该内分泌系统与临床特征之间的相关性。
测量了 25 例 ALS 患者和 25 例年龄、性别和 BMI 匹配的健康对照者的血清 GH、IGF-I、IGF-II、胰岛素、IGF 结合蛋白 1（IGF-BP1）和 IGF 结合蛋白 3（IGF-BP3）水平。对患者和对照者进行了 GHRH 加精氨酸试验。使用肌萎缩侧索硬化功能评定量表修订版（ALSFRS-R）和上运动神经元（UMN）评分评估患者的临床状况。
GHRH 加精氨酸试验显示 13 例（52%）ALS 患者存在 GH 缺乏（GHD）；6 例（24%）为严重 GHD，7 例（28%）为部分 GHD。ALS 患者的 IGF-I 水平明显高于健康对照组（182.9±90.8 与 139.4±58.1ng/ml；P=0.015）。UMN 评分>10 的 ALS 患者的 IGF-I 水平高于 UMN 评分<10 的患者（217.8±100.8 与 155.5±74.6ng/ml，P=0.05）。ALS 患者的 IGF-II 水平明显低于健康对照组（720.9±215 与 1001.9±475.4ng/ml；P=0.03）。
结果表明 ALS 患者的 GH-IGF 系统受损。ALS 中的退行性过程可能导致 IGF-I 的代偿性增加，以向运动神经元或退化的肌纤维提供额外的支持。IGF-II 水平的降低也可能具有病理意义。
