Department of Paediatrics and Paediatric Hematology and Oncology, Klinikum Oldenburg, Rahel-Straus-Straße 10, 26133 Oldenburg, Germany.
Int J Endocrinol. 2010;2010:519607. doi: 10.1155/2010/519607. Epub 2010 Dec 16.
Childhood craniopharyngiomas are rare embryogenic malformations of the sellar region, presumably derived from Rathke cleft epithelium. The overall survival rates after neurosurgical intervention and/or irradiation are high (92%). However, the quality of survival is frequently impaired due to endocrine deficiencies, sleep disturbances, daytime sleepiness, and severe obesity caused by hypothalamic lesions. Based on self-assessment using nutritional diaries, caloric intake was similar in patients and BMI-matched controls. Analyses of physical activity by accelerometric measurements showed a markedly lower level of physical activity. Significant daytime sleepiness and disturbances of circadian rhythms have been demonstrated in obese childhood craniopharyngioma patients. Daytime sleepiness and obesity in these patients were both correlated with low nocturnal and early morning melatonin levels. Polysomnographic studies in patients with severe daytime sleepiness revealed sleeping patterns typical for secondary narcolepsy. Reports on a beneficial effect of treatment with central stimulating agents supported the hypothesis that secondary narcolepsy should be considered as a rare cause for severe daytime sleepiness in patients with childhood craniopharyngioma.
儿童颅咽管瘤是鞍区罕见的胚胎发育畸形,推测来源于 Rathke 裂隙上皮。神经外科干预和/或放疗后的总生存率很高(92%)。然而,由于下丘脑病变引起的内分泌缺乏、睡眠障碍、白天嗜睡和严重肥胖,生存质量常常受损。基于营养日记的自我评估,患者的热量摄入与 BMI 匹配的对照组相似。通过加速度计测量分析的身体活动表明,身体活动水平明显较低。肥胖的儿童颅咽管瘤患者表现出明显的日间嗜睡和昼夜节律紊乱。这些患者的日间嗜睡和肥胖与夜间和清晨褪黑素水平低有关。对严重日间嗜睡患者的多导睡眠图研究显示出典型的继发性嗜睡症睡眠模式。关于中枢兴奋剂治疗有疗效的报告支持了这样一种假设,即继发性嗜睡症应被视为儿童颅咽管瘤患者严重日间嗜睡的罕见原因。