Krahn L E, Black J L, Silber M H
Department of Psychiatry and Psychology, Mayo Clinic, Rochester, Minn. 55905, USA.
Mayo Clin Proc. 2001 Feb;76(2):185-94. doi: 10.1016/S0025-6196(11)63126-1.
Recently, low levels of a newly identified neuropeptide, hypocretin 1, were described in the cerebrospinal fluid of patients with narcolepsy. This neurochemical finding furthers our understanding of this enigmatic sleep disorder typically characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic hallucinations. Narcolepsy appears to be fundamentally related to abnormally regulated rapid eye movement sleep. The diagnosis of this disorder remains challenging because of multiple other conditions that can cause daytime sleepiness and the difficulties in recognizing cataplexy based on patient report. The role of hypocretins in narcolepsy is unclear but intriguing because the cell bodies are restricted to the lateral hypothalamus, a brain region long associated with sleep regulation, with neuronal widespread projections to areas including the locus ceruleus, ventral tegmental area, amygdala, and dorsal raphe. Hypocretins potentially modulate the activity of monoamines and acetylcholine, and therefore their absence leads to the multiple symptoms of narcolepsy. This article reviews the current understanding of the diagnosis and treatment of narcolepsy and discusses the possible implications of the hypocretin discovery.
最近，在发作性睡病患者的脑脊液中发现了一种新确定的神经肽——下丘脑分泌素1的低水平。这一神经化学发现进一步加深了我们对这种神秘睡眠障碍的理解，其典型特征为白天过度嗜睡、猝倒、睡眠麻痹和入睡前幻觉。发作性睡病似乎与快速眼动睡眠的异常调节有着根本联系。由于多种其他病症也会导致白天嗜睡，且基于患者报告识别猝倒存在困难，因此这种疾病的诊断仍然具有挑战性。下丘脑分泌素在发作性睡病中的作用尚不清楚，但很有趣，因为其细胞体局限于下丘脑外侧，这是一个长期以来与睡眠调节相关的脑区，其神经元广泛投射到包括蓝斑、腹侧被盖区、杏仁核和中缝背核等区域。下丘脑分泌素可能调节单胺类和乙酰胆碱的活性，因此其缺失会导致发作性睡病的多种症状。本文综述了目前对发作性睡病诊断和治疗的理解，并讨论了下丘脑分泌素发现的可能意义。
