Scammell Thomas E, Willie Jon T, Guilleminault Christian, Siegel Jerome M
Department of Neurology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA.
Sleep. 2009 Jan;32(1):111-6.
People with narcolepsy often have episodes of cataplexy, brief periods of muscle weakness triggered by strong emotions. Many researchers are now studying mouse models of narcolepsy, but definitions of cataplexy-like behavior in mice differ across labs. To establish a common language, the International Working Group on Rodent Models of Narcolepsy reviewed the literature on cataplexy in people with narcolepsy and in dog and mouse models of narcolepsy and then developed a consensus definition of murine cataplexy. The group concluded that murine cataplexy is an abrupt episode of nuchal atonia lasting at least 10 seconds. In addition, theta activity dominates the EEG during the episode, and video recordings document immobility. To distinguish a cataplexy episode from REM sleep after a brief awakening, at least 40 seconds of wakefulness must precede the episode. Bouts of cataplexy fitting this definition are common in mice with disrupted orexin/hypocretin signaling, but these events almost never occur in wild type mice. It remains unclear whether murine cataplexy is triggered by strong emotions or whether mice remain conscious during the episodes as in people with narcolepsy. This working definition provides helpful insights into murine cataplexy and should allow objective and accurate comparisons of cataplexy in future studies using mouse models of narcolepsy.
发作性睡病患者常出现猝倒发作，即由强烈情绪引发的短暂肌肉无力。目前许多研究人员正在研究发作性睡病的小鼠模型，但不同实验室对小鼠猝倒样行为的定义有所不同。为了建立一种通用的表述，发作性睡病啮齿动物模型国际工作组回顾了有关发作性睡病患者以及发作性睡病犬类和小鼠模型中猝倒的文献，随后制定了小鼠猝倒的共识定义。该小组得出结论，小鼠猝倒为颈部张力缺失的突发发作，持续至少10秒。此外，发作期间脑电图中θ波活动占主导，且视频记录显示有不动状态。为了将猝倒发作与短暂觉醒后的快速眼动睡眠区分开来，发作前必须至少有40秒的清醒时间。符合这一定义的猝倒发作在食欲素/下丘脑泌素信号传导中断的小鼠中很常见，但在野生型小鼠中几乎从未发生过。目前尚不清楚小鼠猝倒是否由强烈情绪引发，以及小鼠在发作期间是否像发作性睡病患者一样保持清醒。这个工作定义为小鼠猝倒提供了有益的见解，并应有助于在未来使用发作性睡病小鼠模型的研究中对猝倒进行客观准确的比较。
