Valencia Garcia Sara, Luppi Pierre-Hervé, Fort Patrice
SLEEP Team, CNRS UMR 5292, INSERM U1028, Centre de Recherche en Neurosciences de Lyon (CRNL), Université Claude Bernard Lyon 1, Lyon, France.
J Exp Neurosci. 2018 Nov 2;12:1179069518808744. doi: 10.1177/1179069518808744. eCollection 2018.
Muscle atonia is a major pathognomonic sign of paradoxical sleep (PS; coined REM Sleep), during which dreams mainly occur. In the 1980s, an idiopathic syndrome called REM sleep behavior disorder (RBD) was described in patients endowed with loss of PS paralysis concomitant to abnormal movements, suggesting a dysfunction of PS networks. Another major clinical RBD feature is its prevalent phenoconversion into synucleinopathies as Parkinson's disease in a delay of 10-15 years after diagnosis. Thus, we undertook experiments in rats to disentangle brainstem networks involved in PS, including atonia. We first identified a contingent of pontine glutamate neurons recruited during PS with inputs to the ventromedial medulla (vmM) where they contact γ-aminobutyric acid (GABA)/glycine inhibitory neurons also activated during PS. Here, we further show that these vmM inhibitory neurons send efferents to somatic spinal motoneurons until lumbar levels. As reported for the pontine generator, the genetic inactivation of the vmM inhibitory neurons abolishes atonia during PS without effects on waking locomotion and is sufficient to recapitulate major RBD symptoms. These original data suggest that RBD may reflect a severe dysfunction and/or degeneration linked to a developing synucleinopathic attack targeting specifically neurons that generate PS-specific atonia.
肌肉张力缺失是异相睡眠(PS;现称快速眼动睡眠)的主要特征性体征,梦境主要在这一阶段出现。在20世纪80年代,一种名为快速眼动睡眠行为障碍(RBD)的特发性综合征在一些患者中被描述出来,这些患者在出现异常运动的同时伴有异相睡眠麻痹的丧失,提示异相睡眠网络功能失调。RBD的另一个主要临床特征是,在诊断后10至15年,它普遍会转变为帕金森病等突触核蛋白病。因此,我们在大鼠身上进行了实验,以厘清参与异相睡眠(包括肌肉张力缺失)的脑干网络。我们首先确定了一组脑桥谷氨酸能神经元,它们在异相睡眠期间被募集,并向延髓腹内侧(vmM)投射,在那里它们与同样在异相睡眠期间被激活的γ-氨基丁酸(GABA)/甘氨酸抑制性神经元接触。在此我们进一步表明,这些vmM抑制性神经元向脊髓躯体运动神经元发出传出纤维直至腰段。正如对脑桥发生器的报道,vmM抑制性神经元的基因失活消除了异相睡眠期间的肌肉张力缺失,而对清醒时的运动没有影响,并且足以重现RBD的主要症状。这些原始数据表明,RBD可能反映了一种严重的功能失调和/或退化,与一种正在发展的突触核蛋白病攻击有关联,这种攻击专门针对产生异相睡眠特异性肌肉张力缺失的神经元。