Division of Radiological Sciences, Department of Medical and Health Sciences (IMH), Linköping University , Linköping , Sweden ; Center for Medical Image Science and Visualization (CMIV), Linköping University , Linköping , Sweden.
Center for Medical Image Science and Visualization (CMIV), Linköping University , Linköping , Sweden ; Division of Disability Research and Linnaeus Centre HEAD, Department of Behavioral Science and Learning, Linköping University , Linköping , Sweden.
Front Neurol. 2014 Apr 2;5:42. doi: 10.3389/fneur.2014.00042. eCollection 2014.
The Kleine-Levin syndrome (KLS) is a rare sleep disorder, characterized by exceptionally long sleep episodes. The neuropathology of the syndrome is unknown and treatment is often inadequate. The aim of the study was to improve understanding of the underlying neuropathology, related to cerebral networks, in KLS during sleep episodes. One patient with KLS and congenital nystagmus was investigated by resting state functional magnetic resonance imaging during both asymptomatic and hypersomnic periods. Fourteen healthy subjects were also investigated as control samples. Functional connectivity was assessed from seed regions of interest in the thalamus and the dorsal pons. Thalamic connectivity was normal in the asymptomatic patient whereas the connectivity between the brain stem, including dorsal pons, and the thalamus was diminished during hypersomnia. These results suggest that the patient's nystagmus and hypersomnia might have their pathological origin in adjacent dorsal pontine regions. This finding provides additional knowledge of the cerebral networks involved in the neuropathology of this disabling disorder. Furthermore, these findings regarding a rare syndrome have broad implications, and results could be of interest to researchers and clinicians in the whole field of sleep medicine.
克莱恩-莱文综合征（KLS）是一种罕见的睡眠障碍，其特征是异常长的睡眠发作。该综合征的神经病理学尚不清楚，治疗往往不充分。本研究旨在通过在睡眠发作期间对 KLS 进行静息状态功能磁共振成像来提高对相关脑网络的潜在神经病理学的认识。对一名患有 KLS 和先天性眼球震颤的患者进行了研究，在无症状和过度睡眠期间均进行了研究。还对 14 名健康受试者作为对照样本进行了研究。通过丘脑和脑桥背侧的种子感兴趣区评估功能连接。在无症状患者中，丘脑的连接正常，而在过度睡眠期间，脑桥背侧与丘脑之间的连接减弱。这些结果表明，患者的眼球震颤和过度嗜睡可能源于邻近的脑桥背侧区域。这一发现为该致残性疾病的神经病理学中涉及的脑网络提供了更多的知识。此外，这些关于罕见综合征的发现具有广泛的意义，结果可能对睡眠医学领域的研究人员和临床医生都有兴趣。
