一例快速眼动睡眠行为障碍、白塞病、干燥综合征及认知功能障碍的病例报告。
A case report of REM sleep behavior disorder, Behcet's disease, Sjogren's syndrome and cognitive dysfunction.
作者信息
Xiao Fulong, Zhang Jun, Wang Waner, Wang Xuehua, Zhang Wei, Xu Liyue, Han Fang
机构信息
1Sleep Medicine Center, Department of Respiratory and Critical Care Medicine, Peking University People's Hospital, Beijing, 100044 People's Republic of China.
2Department of Neurology, Peking University People's Hospital, Beijing, 100044 People's Republic of China.
出版信息
BMC Rheumatol. 2018 Jul 2;2:19. doi: 10.1186/s41927-018-0022-y. eCollection 2018.
BACKGROUND
Behcet's disease and Sjogren's syndrome is an autoimmune disorder from which many systems of the body can suffer. Here we reported a patient with a history of Behcet's disease and Sjogren's syndrome in which REM sleep behavior disorder (RBD) was then detected by polysomnographic (PSG) monitoring.
CASE PRESENTATION
A 68-year-old male patient with a history of Behcet's disease and Sjogren's syndrome was diagnosed with RBD by clinical examination and video-PSG, and he also underwent a multiple sleep latency test and cerebral magnetic resonance imaging. The patient had a history of Behcet's disease for 20 years and Sjogren's syndrome for 2 years. The cerebral magnetic resonance imaging also suggested cerebral demyelination and mild cortical atrophy, with cognitive dysfunction by a score of 28 on the mini-mental state examination (MMSE) and a score of 22 on the Montreal cognitive assessment (MoCA).
CONCLUSION
RBD is common in the elderly population and is significantly related to α-synucleinopathy. Combining the decline in neuro-cognition and mild cortical atrophy, presentation of RBD in this patient could indicate an underlying α-synucleinopathy neurodegenerative disorder in the future. Considering the role of inflammation in the pathogenesis of α-synucleinopathy and a common shared HLA allelic genes in RBD and Sjogren's syndrome, it is suggested that a physiological process which is related to neuroinflammation may be involved in the pathogenesis of RBD.
背景
白塞病和干燥综合征是一种自身免疫性疾病,身体的许多系统都可能受累。在此,我们报告了一名有白塞病和干燥综合征病史的患者,其快速眼动睡眠行为障碍(RBD)通过多导睡眠图(PSG)监测被检测到。
病例介绍
一名68岁男性患者,有白塞病和干燥综合征病史,经临床检查和视频PSG诊断为RBD,他还接受了多次睡眠潜伏期测试和脑磁共振成像检查。该患者有20年白塞病病史和2年干燥综合征病史。脑磁共振成像还提示脑脱髓鞘和轻度皮质萎缩,简易精神状态检查表(MMSE)评分为28分,蒙特利尔认知评估(MoCA)评分为22分,存在认知功能障碍。
结论
RBD在老年人群中很常见,且与α-突触核蛋白病显著相关。结合神经认知功能下降和轻度皮质萎缩,该患者出现RBD可能预示未来存在潜在的α-突触核蛋白病神经退行性疾病。考虑到炎症在α-突触核蛋白病发病机制中的作用以及RBD和干燥综合征中共同的HLA等位基因,提示与神经炎症相关的生理过程可能参与了RBD的发病机制。