发作性睡病1型与自主神经功能紊乱

Autonomic disturbances in narcolepsy type 1

发作性睡病1型的特征性病理改变是下丘脑外侧区Hypocretin神经元大量丢失，由于Hypocretin的信号通路与脑干和脊髓的自主神经中枢有广泛的相互作用，发作性睡病1型患者常伴有自主神经功能紊乱，如昼夜节律／睡眠异常、能量代谢和体温调控障碍、瞳孔调节障碍、性功能障碍以及猝倒过程中的自主神经波动等。我们拟对发作性睡病1型患者在一般状态和猝倒状态下的自主神经功能失调进行综述，以期加强对发作性睡病1型患者自主神经功能障碍的重视。

The pathogenesis of narcolepsy type 1 is characterized by massive loss of hypocretin neurons in the lateral hypothalamus. Due to the extensive interaction between the hypocretin signalling pathway and the autonomic nerve centre of brain stem and spinal cord, narcolepsy type 1 patients often showed autonomic nervous dysfunctions, such as circadian rhythms/sleep abnormalities, energy metabolism and body temperature regulation disorder, pupil adjustment disorder, sexual dysfunction and autonomic fluctuations during eataplexy. We reviewed the autonomic dysfunction of the narcolepsy type 1 patients in general and during eataplexy, in order to strengthen the attention to autonomic nervous dysfunction in narcolepsy type 1.