快动眼睡眠行为障碍（rapid eye movement sleep behavior disorder,RBD）是一种异态睡眠的形式,主要特征是在快动眼睡眠（rapid eye movement,REM）期间,出现骨骼肌迟缓现象消失（rapid eye movement sleep without atonia,RWA）及梦...快动眼睡眠行为障碍（rapid eye movement sleep behavior disorder,RBD）是一种异态睡眠的形式,主要特征是在快动眼睡眠（rapid eye movement,REM）期间,出现骨骼肌迟缓现象消失（rapid eye movement sleep without atonia,RWA）及梦境演绎行为（dream enactment behavior,DEB）。展开更多
快动眼睡眠行为障碍(rapid eye movement sleep behavior disorder,RBD)是以快动眼睡眠(rapid eye movement sleep,REM)期骨骼肌失迟缓缺失引起的临床表现,它可作为单一疾病发生,也可作为α-突触核蛋白病中的先驱症状而存在,同时影响疾...快动眼睡眠行为障碍(rapid eye movement sleep behavior disorder,RBD)是以快动眼睡眠(rapid eye movement sleep,REM)期骨骼肌失迟缓缺失引起的临床表现,它可作为单一疾病发生,也可作为α-突触核蛋白病中的先驱症状而存在,同时影响疾病的进展和程度。但关于RBD的发病机制仍不清楚,随着影像学技术的发展,脑干及黑质纹状体功能在RBD中的作用日益受到关注,脑干作为睡眠管理中心在RBD发病机制中尤显重要。本文就最近几年国外的主要神经影像学进展进行综述。展开更多
Background: Rapid eye movement sleep behavior disorder (RBD) is a parasomnia that is manifested by dream enactment behavior. The electrophysiologic substrate for RBD on polysomnography is rapid eye movement sleep with...Background: Rapid eye movement sleep behavior disorder (RBD) is a parasomnia that is manifested by dream enactment behavior. The electrophysiologic substrate for RBD on polysomnography is rapid eye movement sleep without atonia. Rapid eye movement sleep behavior disorder likely stems from neuronal network dysfunction in the brainstem, although it is not yet clear which specific networks are involved. Rapid eye movement sleep behavior disorder is often associated with the sporadic synucleinopathies but rarely associated with the sporadic tauopathies. There are no reports on the possible association of rapid eye movement sleep without atonia and RBD with any familial tauopathy. Objective: To characterize the clinical sleep and polysomnography features in a kindred with a familial tauopathy. Methods: We performed standard polysomnography in 11 members of the pallidopontonigral degeneration kindred irrespective of any sleep-related complaints. Neuropathologic findings were analyzed in those who subsequently underwent autopsy. Results: Six affected and 5 genealogically at-risk family members were studied. None of the 11 had a history of dream enactment behavior. Nine of the 11 members attained sufficient rapid eye movement sleep on polysomnography, and the electrophysiologic features of rapid eye movement sleep without atonia and behavioral manifestations of RBD were absent in all subjects. Neuropathologic examination of 4 affected individuals revealed marked nigral degeneration in 3 along with mild degenerative changes in the locus coeruleus, pontine nuclei and tegmentum, and medullary tegmentum. Conclusions: These findings argue against nigral degeneration being the primary cause of RBD. The absence of the historical, electrophysiologic, and behavioral manifestations of RBD in this kindred provides further evidence that RBD is rare in the sporadic and familial tauopathies. The difference in frequencies of RBD associated with the synucleinopathies compared with the tauopathies suggests differences in the selective vulnerability of brainstem circuits between the synucle inopathies and tauopathies.展开更多
文摘快动眼睡眠行为障碍（rapid eye movement sleep behavior disorder,RBD）是一种异态睡眠的形式,主要特征是在快动眼睡眠（rapid eye movement,REM）期间,出现骨骼肌迟缓现象消失（rapid eye movement sleep without atonia,RWA）及梦境演绎行为（dream enactment behavior,DEB）。
文摘快动眼睡眠行为障碍(rapid eye movement sleep behavior disorder,RBD)是以快动眼睡眠(rapid eye movement sleep,REM)期骨骼肌失迟缓缺失引起的临床表现,它可作为单一疾病发生,也可作为α-突触核蛋白病中的先驱症状而存在,同时影响疾病的进展和程度。但关于RBD的发病机制仍不清楚,随着影像学技术的发展,脑干及黑质纹状体功能在RBD中的作用日益受到关注,脑干作为睡眠管理中心在RBD发病机制中尤显重要。本文就最近几年国外的主要神经影像学进展进行综述。
文摘Background: Rapid eye movement sleep behavior disorder (RBD) is a parasomnia that is manifested by dream enactment behavior. The electrophysiologic substrate for RBD on polysomnography is rapid eye movement sleep without atonia. Rapid eye movement sleep behavior disorder likely stems from neuronal network dysfunction in the brainstem, although it is not yet clear which specific networks are involved. Rapid eye movement sleep behavior disorder is often associated with the sporadic synucleinopathies but rarely associated with the sporadic tauopathies. There are no reports on the possible association of rapid eye movement sleep without atonia and RBD with any familial tauopathy. Objective: To characterize the clinical sleep and polysomnography features in a kindred with a familial tauopathy. Methods: We performed standard polysomnography in 11 members of the pallidopontonigral degeneration kindred irrespective of any sleep-related complaints. Neuropathologic findings were analyzed in those who subsequently underwent autopsy. Results: Six affected and 5 genealogically at-risk family members were studied. None of the 11 had a history of dream enactment behavior. Nine of the 11 members attained sufficient rapid eye movement sleep on polysomnography, and the electrophysiologic features of rapid eye movement sleep without atonia and behavioral manifestations of RBD were absent in all subjects. Neuropathologic examination of 4 affected individuals revealed marked nigral degeneration in 3 along with mild degenerative changes in the locus coeruleus, pontine nuclei and tegmentum, and medullary tegmentum. Conclusions: These findings argue against nigral degeneration being the primary cause of RBD. The absence of the historical, electrophysiologic, and behavioral manifestations of RBD in this kindred provides further evidence that RBD is rare in the sporadic and familial tauopathies. The difference in frequencies of RBD associated with the synucleinopathies compared with the tauopathies suggests differences in the selective vulnerability of brainstem circuits between the synucle inopathies and tauopathies.
文摘快动眼睡眠行为障碍（rapid eye movement sleep behavior disorder,RBD）是一种反复伴随快速眼球运动（rapid eye movement,REM）期的梦境演绎而表现出复杂行为和（或）发声的异态睡眠.其特征性的临床表现及多导睡眠监测（polysomnography,PSG）中出现REM期肌肉失迟缓现象（rapid eye movement sleep without atonia,RWA）,是诊断RBD的必要条件[1].该病在中老年人群并不少见,中国人群中,基于RBD量表的大规模社区流行病学调查发现可疑RBD的患病率在4％ ～ 5％[2].RBD的患病率随着年龄的增大而升高,男性更为多见,约为女性的4倍甚至更多[3].
