Department of Neurological Sciences, University of Bologna, Bologna, Italy.
Clin Neurophysiol. 2010 Feb;121(2):153-62. doi: 10.1016/j.clinph.2009.10.012. Epub 2009 Dec 1.
To investigate the behavioural and neurophysiological pattern of cataplexy.
Seven narcolepsy with cataplexy patients underwent daytime videopolygraphy using humorous movies or/and jokes to trigger cataplectic attacks.
During segmental cataplectic attacks, EMG showed brief and irregular periods of silencing focally involving facial, neck, axial or limb muscles, sometimes coinciding with bursts of rapid eye movements. All patients enacted intentional movements in response to these segmental postural lapses. During global cataplectic attacks, EMG showed suppression of activity alternated with patterned enhancement, enhanced EMG activity in neck muscles preceding that of other cranial, axial and lower limb muscles. This waxing and waning EMG pattern ended with a complete body collapse and persistent muscle atonia. Breathing irregularities, heart rate (HR) instability and EEG desynchronization were observed during global cataplectic attacks without any appreciable blood pressure changes, but with HR deceleration and silencing of sympathetic skin response while in complete atonia. Patients subjectively perceived the involuntary postural lapses as startling and alarming.
Cataplexy in our patients showed many of the features of tonic REM sleep.
Cataplexy can be construed as a "freezing-like" perturbation of the orienting response with transient impairment of posture and movements resulting in a "patchwork-compromise-behaviour".
研究猝倒的行为和神经生理模式。
7 名猝倒性发作的嗜睡症患者接受日间录像检查,使用幽默电影或/和笑话来引发猝倒发作。
在节段性猝倒发作期间,肌电图显示短暂且不规则的沉默期,主要涉及面部、颈部、轴性或肢体肌肉,有时与快速眼球运动爆发同时发生。所有患者在这些节段性姿势丧失时都会做出有意的动作。在全身性猝倒发作期间,肌电图显示活动抑制与模式增强交替出现,颈部肌肉的肌电图活动增强先于其他颅、轴性和下肢肌肉。这种肌电图的起伏模式以完全的身体瘫痪和持续的肌肉弛缓结束。在全身性猝倒发作期间观察到呼吸不规则、心率(HR)不稳定和脑电图去同步化,没有明显的血压变化,但在完全弛缓时 HR 减速和交感皮肤反应沉默。患者主观上认为无意识的姿势丧失令人吃惊和惊恐。
我们患者的猝倒发作具有 REM 睡眠强直的许多特征。
猝倒可以被理解为定向反应的“冻结样”扰动,导致姿势和运动的短暂障碍,从而导致“拼凑妥协行为”。