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
心理生理性失眠是临床上极为常见的失眠症，是一种因躯体紧张和预料妨碍睡眠的联想引起的失眠，这种预料联想可为内部感受或外部刺激引起，易受睡眠环境因素的影响而改变，在睡眠脑电图检查中存在明显的首夜效应（first night effect，...心理生理性失眠是临床上极为常见的失眠症，是一种因躯体紧张和预料妨碍睡眠的联想引起的失眠，这种预料联想可为内部感受或外部刺激引起，易受睡眠环境因素的影响而改变，在睡眠脑电图检查中存在明显的首夜效应（first night effect，FNE），国内外临床和多导睡眠图资料较少，有关多导睡眠图检查中首夜效应，争议较多。本研究采用连续2夜多导睡眠图记录和结合心理-行为干预方法，对心理生理性失眠患者的首夜效应及相关因素进行探讨，现报告如下。展开更多
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.
文摘心理生理性失眠是临床上极为常见的失眠症，是一种因躯体紧张和预料妨碍睡眠的联想引起的失眠，这种预料联想可为内部感受或外部刺激引起，易受睡眠环境因素的影响而改变，在睡眠脑电图检查中存在明显的首夜效应（first night effect，FNE），国内外临床和多导睡眠图资料较少，有关多导睡眠图检查中首夜效应，争议较多。本研究采用连续2夜多导睡眠图记录和结合心理-行为干预方法，对心理生理性失眠患者的首夜效应及相关因素进行探讨，现报告如下。
