克莱恩-莱文综合征睡眠期间的间歇性激素分泌：下丘脑功能障碍的证据。

Episodic hormone secretion during sleep in Kleine-Levin syndrome: evidence for hypothalamic dysfunction.

作者信息

Gadoth N, Dickerman Z, Bechar M, Laron Z, Lavie P

机构信息

Department of Neurology, Beilinson Medical Center, Petah Tiqva, Israel.

出版信息

Brain Dev. 1987;9(3):309-15. doi: 10.1016/s0387-7604(87)80051-7.

DOI:10.1016/s0387-7604(87)80051-7

PMID:3477962

Abstract

"Acute" hypothalamic-pituitary function tests including insulin tolerance test, LRH, ACTH and TRH stimulation tests and nocturnal secretory pattern of human growth hormone, 11-OHCS, prolactin, FSH, LH and TSH were studied in a 23-year-old male with Kleine-Levin syndrome during the course of a typical hypersomnic attack. The "acute" tests revealed paradoxical growth-hormone response to TRH stimulation, borderline high basal plasma prolactin levels with normal response to TRH. The hormonal secretory pattern during sleep revealed abnormalities in LH, 11-OHCS and prolactin secretion. These together with the results of the "acute" tests are indicative of an abnormality in the hypothalamic regulation of various pituitary hormones. This observation may indeed be the first laboratory demonstration confirming a long-standing hypothesis that Kleine-Levin syndrome is related to hypothalamic dysfunction.

摘要

对一名患有克莱恩-莱文综合征的23岁男性，在一次典型的嗜睡发作过程中，研究了“急性”下丘脑-垂体功能测试，包括胰岛素耐量试验、促黄体生成素释放激素（LRH）、促肾上腺皮质激素（ACTH）和促甲状腺激素释放激素（TRH）刺激试验，以及人生长激素、11-羟皮质类固醇（11-OHCS）、催乳素、促卵泡生成素（FSH）、促黄体生成素（LH）和促甲状腺激素（TSH）的夜间分泌模式。“急性”试验显示，对TRH刺激的生长激素反应异常，基础血浆催乳素水平临界升高，但对TRH反应正常。睡眠期间的激素分泌模式显示LH、11-OHCS和催乳素分泌异常。这些结果与“急性”试验结果共同表明，下丘脑对各种垂体激素的调节存在异常。这一观察结果可能确实是首次通过实验室证实了一个长期存在的假设，即克莱恩-莱文综合征与下丘脑功能障碍有关。