Objective To analyze the changes in biomarkers associated with sleep disorders in anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis, and to preliminarily explore the mechanism of sleep disorders. Methods Fifty patients with anti-LGI1 antibody-associated encephalitis who admitted He'nan Provincial People's Hospital from April 2015 to September 2022 were selected. Serum and cerebrospinal fluid (CSF) samples were collected for biomarkers detection, and Pittsburgh Sleep Quality Index (PSQI) was used to evaluate the presence of sleep disorders. Results The 50 patients were divided into the sleep disorders (>7) group (n=28) and the no sleep disorders (≤7) group (n=22) based on PSQI score. Compared with the no sleep disorders group, the sleep disorders group in serum and CSF showed higher levels of neurofilament light chain (NfL; t=6.690, P=0.000; t=2.356, P=0.023), glial fibrillary acidic protein (GFAP; t=3.713, P=0.000; t=2.768, P = 0.008), ionized calcium-binding adapter molecule 1 (Iba1; t = 5.042, P = 0.000; t = 3.472, P = 0.001), orexin A (t = 3.250, P = 0.002; t = 5.376, P = 0.000), cortisol (t = 2.487, P = 0.016; t=5.779, P=0.000), tumor necrosis factor-α (TNF-α; t=3.832, P=0.000; t=5.122, P=0.000) and chemokine (C-X-C motif) ligand 13 (CXCL13; t=2.483, P=0.017; t=4.116, P=0.000), while only interleukin-1β (IL-1β) increased in CSF (t=2.526, P=0.015). Conclusions Sleep disorders associated with anti-LGI1 antibody-associated encephalitis is elevated in levels of neuroinjury markers, neuroendocrine markers and neuroimmune markers, suggesting that sleep disorders associated with anti-LGI1 antibody-associated encephalitis is not a single mechanism and may be related to the extensive involvement of the neuro-endocrine-immune network.

Objective To quantitatively analyze the difference in the content of plasma exosome α-synuclein(α-syn)be-tween Parkinson disease(PD)and idiopathic rapid eye movement sleep behavior disorder(iRBD),and to identify predictable biological markers.Methods A total of 20 patients with iRBD(iRBD group),21 PD patients without RBD(PD-nRBD group),and 20 healthy controls matched for age and sex(HC group)were enrolled.Rapid-Eye-Movement Sleep Behavior Dis-order Questionnaire-Hong Kong(RBDQ-HK)was used to evaluate the nocturnal symptoms of all subjects,and the motor section of Unified Parkinson Disease Rating Scale Ⅲ(UPDRS Ⅲ)was used to evaluate motor impairment.ELISA was used to measure the content of plasma exosome α-syn,and the three groups were analyzed in terms of the content of plasma exosome α-syn and its correlation with RBDQ-HK score and UPDRS Ⅲ score.Results There was a significant difference in UPDRS Ⅲ score be-tween the iRBD group,the PD-nRBD group,and the HC group(P=0.000 1),with a significant difference between any two groups(P<0.05);there was also a significant difference in RBDQ-HK score between the three groups(P=0.000 1),and the iRBD group had a significantly higher score than the other two groups(P=0.000 1).The iRBD group and the PD-nRBD group had a significantly higher content of plasma exosome α-syn than the HC group(P=0.001),and the iRBD group had a lower con-tent of plasma exosome α-syn than the PD-nRBD group(P>0.05).In the iRBD group,plasma exosome α-syn was positively correlated with RBDQ-HK score(r=0.842,P=0.000 1),and in the PD-nRBD group,plasma exosome α-syn was positively cor-related with UPDRS Ⅲ score(r=0.817,P=0.000 1)and H-Y staging(r=0.592,P=0.005).Conclusion The presence of plasma exosome α-syn is observed in iRBD patients,which is similar to that in PD-nRBD patients,and the content of plasma exosome α-syn is associated with the motor score of PD-nRBD and the nocturnal symptom score of iRBD.Therefore,plasma exo-some α-syn is expected to become an early biomarker for predicting the conversion of iRBD to PD.

Purpose Idiopathic rapid eye movement Sleep Behavior Disorder (iRBD) is considered as a prodromal and most valuable warning symptom for Parkinson’s disease (PD). Although iRBD and PD without RBD (nRBD-PD) are both α-synucleinopathies, whether they share the same neurodegeneration process is not clear enough. In this study, the pattern and extent of neurodegeneration were investigated and compared between early-stage nRBD-PD and iRBD from the perspective of whole-brain functional network changes. Methods Twenty-one patients with iRBD, 23 patients with early-stage nRBD-PD, and 22 matched healthy controls (HCs) were enrolled. Functional networks were constructed using resting-state functional MRI (fMRI) data. Network topological properties were analyzed and compared among groups by graph theory approaches. Correlation analyses were performed between network topological properties and cognition in the iRBD and nRBD-PD groups. Results Both patients with iRBD and patients with early-stage nRBD-PD had attention, executive function, and some memory deficits. On global topological organization, iRBD and nRBD-PD groups still presented small-worldness, but both groups exhibited decreased global/local efficiency and increased characteristic path length. On regional topological organization, compared with HC, nRBD-PD presented decreased nodal efficiency, decreased degree centrality, and increased nodal shortest path length, while iRBD presented decreased nodal efficiency and nodal shortest path. For iRBD, brain regions with decreased nodal efficiency were included in the corresponding regions of nRBD-PD. Nodal shortest path changes were significantly different in terms of brain regions and directions between nRBD-PD and iRBD. Attention deficits were correlated with local topological properties of the occipital lobe in both iRBD and nRBD-PD groups. Conclusion Both global and local efficiency of functional networks declined in nRBD-PD and iRBD groups. The overlaps and differences in local topological properties between nRBD-PD and iRBD indicate that iRBD not only shares functional changes of PD but also presents distinct features.

Purpose:This study investigates the topological properties of brain functional networks in patients with isolated rapid eye movement sleep behavior disorder (iRBD). Participants and Methods:A total of 21 patients with iRBD (iRBD group) and 22 healthy controls (HCs) were evaluated using resting-state functional MRI (rs-fMRI) and neuropsychological measures in cognitive and motor function. Data from rs-fMRI were analyzed using graph theory, which included small-world properties, network efficiency, network local efficiency, nodal shortest path, node efficiency, and network connectivity, as well as the relationship between behavioral characteristics and altered brain topological features. Results:Rey-Osterrieth complex figure test (ROCFT-copy), symbol digital modalities test (SDMT), auditory verbal learning test (AVLT)-N1, AVLT-N2, AVLT-N3, and AVLT-N1-3 scores were significantly lower in patients with iRBD than in HC (P < 0.05), while trail making test A (TMT-A), TMT-B, and Unified Parkinson's Disease Rating Scale Part-III (UPDRS-III) scores were higher in patients with iRBD (P < 0.05). Compared with the HCs, patients with iRBD had no difference in the small-world attributes (P > 0.05). However, there was a significant decrease in network global efficiency (P = 0.0052) and network local efficiency (P = 0.0146), while an increase in characteristic path length (P = 0.0071). There was lower nodal efficiency in occipital gyrus and nodal shortest path in frontal, parietal, temporal lobe, and cingulate gyrus. Functional connectivities were decreased between the nodes of occipital with the regions where they had declined nodal shortest path. There was a positive correlation between TMT-A scores and the nodal efficiency of the right middle occipital gyrus (R = 0.602, P = 0.014). Conclusion:These results suggest that abnormal behaviors may be associated with disrupted brain network topology and functional connectivity in patients with iRBD and also provide novel insights to understand pathophysiological mechanisms in iRBD.

Objective:To investigate the correlations of melanin concentration hormone (MCH) in cerebrospinal fluid (CSF) and serum with sleep disorder, memory dysfunction and prognoses in patients with cerebral ischemic stroke (CIS).Methods:One hundred elderly CIS patients, admitted to Department of Neurology, He'nan Provincial People's Hospital from June 2021 to January 2022 were enrolled as CIS group, and 50 subjects collected from Physical Examination of the same hospital during the same period were enrolled as control group. MCH levels in the CSF and serum were detected by ELISA. Sleep quality was assessed by polysomnography and Pittsburgh Sleep Quality Index (PSQI). Memory function was assessed by Rivermead Behavioral Memory Test 2 nd Edition (BMT-II). Prognoses were assessed by modified Rankin Scale (mRS) 3 months after discharge. The clinical data and MCH levels of the two groups were compared; the differences in MCH levels among CIS patients with different degrees of sleep disorder, and different memory functions and prognoses were compared. Correlations of MCH level and sleep parameters with RBMT-II scores in these CIS patients were analyzed. Results:Compared with that in the control group, the proportion of patients with hypertension in CIS group was significantly higher ( P<0.05). Compared with the control group ([42.39±16.11] pg/mL), the serum MCH level in CIS group ([36.89±15.19] pg/mL) was statistically lower ( P<0.05). In CIS patients, patients with mild or severe sleep disorder had significantly decreased CSF MCH level compared with patients without sleep disorder ( P<0.05), patients with severe sleep disorder had significantly decreased CSF MCH level compared with patients with mild sleep disorder ( P<0.05); patients with severe sleep disorder had significantly decreased serum MCH level compared with patients without sleep disorder ( P<0.05); CSF MCH level was negatively correlated with PSQI scores, sleep latency and wake frequency ( P<0.05), and positively correlated with percentage of rapid eye movement ( P<0.05); serum MCH level in CIS patients was negatively correlated with PSQI scores and wake frequency ( P<0.05). In CIS patients, the CSF and serum MCH levels in patients with memory dysfunction was significantly lower compared with those with normal memory function ( P<0.05); a positive correlation was noted between RBMT-II scores and CSF MCH level ( P<0.05). In CIS patients, patients with poor prognosis had statistically lower CSF and serum MCH levels compared with those with good prognosis ( P<0.05). Conclusion:The serum MCH level in CIS patients is significantly decreased, which is closely related to the occurrence of sleep disorder and memory dysfunction after stroke; and they further affects the prognoses.

目的 特发性快眼动睡眠行为障碍(idopathic rapid eye movement sleep behavior disorder,iRBD)又称帕金森病临床前期,为寻找其早期损害的证据,利用功能核磁共振探查其早期大脑自发活动性的改变,以提供其脑损害的影像证据.方法 选取符合诊断标准的iRBD 21例,性别、年龄匹配的健康对照组22例,进行认知量表及统一帕金森病等级评分-运动部分(Unified Parkinson's Disease Rating Scale motor section,UPDRSⅢ)测评,并进行功能磁振扫描,利用局部一致性(region homogeneity,ReHo)方法进行数据分析;结果 与正常对照组相比,iRBD组Rey-Osterrieth复杂图形测验(ROCFT)、听词语学习测验(AVLT)N1、N2、N3、符号数字模式测验(SDMT)、数字排序测验-注意力部分(DOT-A)得分均下降(U=133.5、t=-2.416、t=-2.873、U=134.5、U=81.0、U=144.5,P<0.05),连线测验B(TMT-B)及UPDRSⅢ得分明显增加(U=138.0、U=121.0,P<0.05);与对照组比较,iRBD组基底节区右尾状核、左壳核、右壳核及右苍白球局部一致性明显下降(t=-4.611、t=-4.360、t=-4.298、t=-3.422,P<0.05).结论 iRBD患者存在视空间、记忆、执行、注意及运动方面损害,其基底节区神经团块内部自发活动一致性明显下降,提示基底节区功能活动异常,可能是iRBD早期基底节损害的标记.

Introduction The structural and functional damages of the striatum were evident in idiopathic REM sleep behavior disorder (iRBD). With the research on iRBD deepens, cognitive impairment in iRBD is getting increasing attention. However, the mechanism of cognitive impairment in iRBD was poorly understood. Methods Neuropsychological assessment was carried out in 21 polysomnographies (PSGs) confirmed iRBD patients and 22 normal controls. Both regional homogeneity (ReHo) and seed-based functional connectivity (FC) rs-fMRI analyses were applied to explore the FC abnormalities and its association with cognition in iRBD patients. Positive ReHo clusters were set as seeds for further FC analysis. Results Idiopathic REM sleep behavior disorder patients presented cognitive deficits in attention/working memory, executive function, immediate memory, and visuo-spatial ability. ReHo analysis revealed abnormal spontaneous brain activities in the striatum (right caudate, left pallidum and bilateral putamen) in iRBD. FC analysis showed decreased striatum-related FCs in the frontal, temporal, occipital lobes, thalamus, anterior cingulate gyrus, as well as decreased intrinsic FCs between bilateral putamen and between caudate and pallidum. Deficits in attention/working memory, executive function, and immediate memory were associated with abnormal striatal-cortical FCs including frontal, temporal, and anterior cingulate cortices. Conclusion Functional changes of striatum and cognitive impairment in iRBD were reconfirmed in the present study. Abnormal striatal-cortical networks, especially the striatal-frontal network, contribute to the working memory/executive function deficits in iRBDs. These findings supported the role of striatum not only in motor but also in cognition impairment in iRBD.

Purpose To explore the characteristics of gut microbiota and its relationship between clinical manifestations in patients with type 1 narcolepsy (NT1). Patients and Methods Scale and polysomnography were performed in 20 NT1 patients and 16 healthy controls (HC group) to evaluate the clinical characteristics of NT1. Illumina sequencing was performed on bacterial 16S ribosomal RNA gene using V3-V4 regions to compare the fecal microbiota in all subjects. Associations between clinical characteristics and gut microbiota were analyzed using partial correlation analysis. Results Compared with the HC group, the NT1 group had a significantly higher ESS score, longer total sleep time, increased wakefulness, decreased sleep efficiency, disturbance of sleep structure, shorter mean sleep latency, and increased sleep-onset REM periods (all P < 0.05). No differences in alpha and beta diversity were observed between the two groups. In contrast, there were significant differences at the level of class, order, family, and genus (all P < 0.05). LEfSe analysis showed that the relative abundance of Klebsiella in the NT1 group was higher than that in the HC group (P < 0.05), while the relative abundance of Blautia, Barnesiellaceae, Barnesiella, Phocea, Lactococcus, Coriobacteriia, Coriobacteriales, Ruminiclostridium_5, and Bilophila were lower (all P < 0.05). Partial correlation analysis revealed that partial differential bacteria in the NT1 group were correlated with total sleep time, sleep efficiency, stage 1 sleep, arousal index, and sleep latency (all P < 0.05). Conclusion Our data revealed differences in intestinal flora structure between NT1 patients and the normal population, thus providing a theoretical basis for future microecological therapy for narcolepsy. However, future larger sample size studies and different study designs are needed to further clarify the possible pathogenesis and potential causality of intestinal flora in NT1 patients and explore the new treatment strategies.

DOI：10.3969/j.issn.1672⁃6731.2020.07.018

OBJECTIVE To investigate the changes in behaviors and brain structural network in patients with idiopathic rapid eye movement sleep behavior disorder (iRBD). METHODS Twenty patients with iRBD (iRBD group) and 22 healthy control subjects were evaluated using the Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn-Yahr staging. Diffusion tensor imaging and graph- theoretical analysis were performed for analyzing the topological characteristics of brain structural networks of the patients, and the correlation between the behavioral changes and the changes in the topological characteristics of the brain networks was analyzed. RESULTS The UPDRS score was significantly higher in iRBD group than in the healthy control group (P < 0.05). No significant difference was found in small-world attributes between the patients with iRBD and the control subjects (P>0.05). The patients with iRBD exhibited significantly shortened characteristic shortest path length Lp (P < 0.05) and significantly increased global efficiency, local efficiency and assortativity (P < 0.05). Examination of regional topological properties of the brain network revealed abnormal node properties in the frontal, temporal, parietal, occipital, and striatal and limbic lobes in patients with iRBD. The patients also had significantly increased degree centrality of the left pallidum and enhanced nodal efficiency in the left thalamus, superior temporal gyrus, temporal pole and bilateral superior occipital gyrus, bilateral putamens as well as the right gyrus rectus, amygdala, supramarginal gyrus, and middle temporal gyrus. The nodal local efficiency was significantly increased in the left superior frontal gyrus, middle cingulate gyrus, superior parietal gyrus, bilateral fusiform gyrus, right superior motor area, postcentral gyrus and angular gyrus of the patients with iRBD. The nodal shortest path was significantly shortened in the left superior motor area, pallidum, thalamus, superior temporal gyrus, temporal pole, bilateral putamens, bilateral superior occipital gyrus, right rectus gyrus, amygdala, supramarginal gyrus and middle temporal gyrus, and the nodal clustering coefficient was significantly lowered in the left superior occipital gyrus of the patients. In patients with iRBD, the UPDRS I score was positively correlated with the nodal efficiency in the right supramarginal gyrus (r=0.50, P < 0.05) and local nodal efficiency in the right fusiform gyrus (r=0.53, P < 0.05), and negatively correlated with the nodal clustering coefficient in the left superior occipital gyrus (r=-0.552, P < 0.05). CONCLUSIONS Patients with iRBD present with abnormal changes in mental condition, behaviors, emotions, activities of daily living and motor functions. The brain structural network of patient with iRBD still has a small-world property with abnormal global topological property and abnormal distribution of local topological property in the cortex, striatum and limbic system.

目的 探讨伴快速眼动睡眠期行为障碍的帕金森(Parkinson disease with REM sleep behavior disorder,PD-RBD)患者认知功能以及其蓝斑下核与各脑区的功能连接.方法 纳入PD-RBD患者20例,不伴快速眼动睡眠期行为障碍的帕金森(Parkinson disease with no REM sleep behavior disorder,PD-nRBD)患者23例和正常对照者(normal control,NC)21名进行认知功能评估,并对三组行静息态功能磁共振扫描,比较双侧蓝斑下核与各脑区的功能连接.结果 PD-RBD组DOT-A(P=0.004)、SDMT(P<0.001)、SCWTB(P=0.001)、SCWT C(P=0.013)、AVLT N1(P<0.001),N2(P<0.001),N3(P<0.001),N4(P<0.001),N5(P=0.003),N6(P=0.004)和PD-nRBD组DOT-A(P=0.011)、SDMT(P<0.001)、SCWTB(P=0.007)、SCWT C(P=0.011)、AVLT N1(P<0.001),N2(P<0.001),N3(P<0.001),N4(P=0.003),N5(P=0.011),N6(P=0.002)评分低于对照组且有统计学差异.与NC组相比,PD-RBD组双侧蓝斑下核分别与双侧楔前叶、 双侧小脑的功能连接均减弱,PD-nRBD组右侧蓝斑下核与双侧小脑连接增强;PD-RBD组左侧蓝斑下核与左侧楔前叶功能连接强度与ROCFT临摹部分得分呈显著正相关(r=0.507,P=0.038),右侧蓝斑下核与右侧楔前叶功能连接强度与SCWTC、ROCFT临摹部分得分呈显著正相关(r=0.501,P=0.041;r=0.628,P=0.007).结论 PD-RBD和PD-nRBD患者均存在注意力/执行力及记忆力损害,但仅PD-RBD患者蓝斑下核与认知相关脑区出现功能连接异常,并参与引起PD-RBD患者注意力和视空间能力的下降.

Objectives To explored the clinical features of patients with recurrent isolated sleep paralysis (RISP), providing clues for the pathogenesis and treatment of the disease. Methods The clinical features and auxiliary examina-tions of 2 patients with recurrent isolated sleep paralysis diagnosed at the People’s Hospital of Zhengzhou University in 2017 were retrospectively analyzed. Result Both cases were excluded from narcolepsy,which was consistent with the clas-sic performance of sleep paralysis. At the time of attack,the consciousness of Example 1 was not completely clear,the neck could be slightly twisted and accompanied by auditory hallucinations;Example 2 consciousness was completely clear and there was no hallucination. Both cases were accompanied by obvious symptoms of anxiety,but the causal relationship was not clear. Example 1 received trazodone treatment with good results. Conclusions RISP attacks are in a state of separa-tion,but in a variety of forms;Its recurrent attacks may be associated with impaired sleep homeostasis;A variety of antide-pressant drugs are effective for RISP.

Objective To investigate the abnormal functional connectivity (FC) between the cores (including sublaterodorsal nucleus (SLD) and ventrolateral periaqueductal gray matter (vlPAG)) and the whole brain in rapid eye movement sleep behavior disorder (RBD) by resting state functional magnetic resonance imaging (rfMRI).Methods A total of 41 subjects recruited in the Department of Neurology,the People's Hospital of Zhengzhou University were enrolled in this study according to international diagnosis criteria,including 20 with idiopathic RBD (iRBD group) and 21 age,sex-matched normal controls (control group).All subjects were examined by Hoehn-Yahr Staging,cognitive tests and rfMRI.Resluts HoehnYahr staging score was 0(0,0) in the iRBD group,which showed no significant difference from that in the control group (0 (0,0),Z =-1.820,P =0.069).The scores of Rey Auditory Verbal Learning Test (AVLT) N1,AVLT N2,Symbol Digital Modalities Test,Rey-Osterrieth Complex Figure Test-Copy were 3.80 ± 1.67,5.10 ± 1.77,33.00(31.25,34.00) and 22.00(20.25,26.00) respectively in the iRBD group,which were significantly lower than that in the control group (4.95 ± 1.28,t =2.482,P =0.017;6.43±1.16,t =2.848,P=0.007;33.00(29.50,35.50),Z=-3.792,P=0.000;35.00(33.00,36.00),Z =-2.351,P =0.019) respectively.The scores of Trail Making Test 1 (86.5 (70.0,100.0))and Trail Making Test 2 (197.0(180.5,211.5)) in the iRBD group were significantly higher than that in the control group (66.0(49.0,91.5),112.0(99.5,173.0) respectively,Z=-2.373,P=0.018;Z =-3.105,P =0.002).Compared with the control group,the FC analysis showed reduced connections from the right SLD to the bilateral cingnlate gyrus (t =-4.173) and bilateral frontal gyrus (t =-2.965(left),-3.662(right)),from the vlPAG to the left precentral-postcentral gyrus(t =3.930),and from the vlPAG to the right frontal gyrus (t =4.141) in the iRBD.There was no statistically significant difference from the left SLD to the whole brain.Conclusion There were abnormal FCs from the SLD and vlPAG to cognitive and motor areas in RBD patients,perhaps leading to clinical RBD symptoms such as cognitive deterioration and movement disorder.

Objective To investigate the characteristics of sleep structures in patients with multiple dreams through the retrospective analysis of polysomnography in patients with multiple dreams,and to provide a theoretical basis for the treatment of multiple dreams.Methods Twenty-two cases with multiple dreams in Department of Neurology,the People's Hospital of Zhengzhou University from July 2015 to Ferbuary 2018 were included in multi-dream group and 12 healthy people in control group.The sleep parameters related to polysomnography during the visit were collected and recorded,and the differences between the two groups were compared.Resluts There was no statistically significant difference in apneahypopnea index,sleep latency,rapid eye movement (REM) sleep latency,slow-wave sleep ratio,and REM-arousal index between the two groups.Compared with the control group,sleep efficiency (73.46％ ± 12.41％ vs 90.43％ ± 4.42％,t=-4.555,P=0.000),REM period ratio (16.28％ ± 5.59％ vs 21.59％ ± 2.70％,t =-3.727,P =0.001) decreased in the multi-dream group;whereas ratio of light sleep (66.49％ ±9.97％ vs 59.85％ ±3.01％,t =2.966,P =0.006),awakening numbers (13.4 ±6.98 vs 6.08 ± 3.34,t =3.411,P =0.002),arousal index (20.11 ± 10.69 vs 11.82 ± 8.09,t =2.338,P =0.026),non-REM arousal index (20.22 ± 10.53 vs 12.08 ± 8.69,t =2.283,P =0.029) increased.Conclusion The sleep efficiency of patients with multiple dreams is reduced,and their perceived dreams may originate from light sleep periods.

快速眼动睡眠行为障碍( rapid eye movement sleep behavoir disorder,RBD) 分为继发性和自发性,继发性RBD 常与服用药物(如抗抑郁药)?神经变性病?免疫性疾病以及脑干损伤相关[1-5] .研究发现,IgLON5 抗体相关睡眠障碍常伴有RBD[4] .自发性RBD 经历数十年,有90% 患者发展为帕金森病( Parkinson disease,PD)?伴认知障碍的PD?路易体痴呆或多系统萎缩[6. 7] ,因此自发性RBD 不单单是异态睡眠,而是α-突触核蛋白病的临床前期,所以定义为隐源性RBD 更为合适[8] ,但又不能涵盖没有转变为α-突触核蛋白病的RBD,因此有学者认为将其称为孤立RBD(isolated RBD) 更为合适[9] .除此,孤立RBD 临床表现是运动症状,将其定义为神经变性病的非运动症状是不恰当的,定义为α-突触核蛋白病的睡眠症状更为合适[9] .孤立RBD 可以预测α-突触核蛋白病,推测孤立RBD 早期可能存在维持睡眠肌张力的脑网络功能失调.

目的 通过对特发性快速眼球运动睡眠行为障碍(iRBD)患者认知脑区改变的探索,寻找其与帕金森病(PD)患者大脑形态改变的异同,为两者的联系提供依据.方法 对14例iRBD患者、12例PD患者及8名健康人(CON组)进行认知心理测评(复杂图形测试、临摹及回忆、符号-数字转换测试、连线测试、画钟测试、数字广度测验)及磁共振检查,采用基于体素形态学的研究技术进行分析.结果 iRBD组及PD组患者的数字符号转换测试、连线测验测试2与CON组之间的差异均有统计学意义(P<0.05),iRBD组与PD组间的差异无统计学意义(P>0.05);与CON组比较,iRBD组与PD组患者的右顶叶及左枕叶体积均缩小(P<0.05),但iRBD组与PD组间的差异无统计学意义(P>0.05);PD组右枕叶较CON组体积减小(P<0.05).结论 iRBD患者及PD患者均出现注意力及执行功能等认知能力下降,并且都出现右顶叶和枕叶萎缩,提示两者的认知损害存在相似的病理基础.

Objective To investigate the structure of corpus striatum and the integrity of white matter fiber in patients with Parkinson's disease (PD) and idiopathic rapid eye movement sleep behavior disorder (iRBD).Methods Twelve patients with iRBD,12 patients with PD and 10 healthy subjects that were well matched in gender,age and education were enrolled in this study.Head MRI examination was performed to all subjects to observe the changes of corpus striatum structure (the gray matter volume) and the integrity of white matter fiber [fractional anisotropy (FA)] by combining voxel-based morphometry (VBM) and diffusion tensor imaging (DTI).Results Compared with healhy subjects,the gray matter volume of left caudate nucleus was significantly decreased (P ＜ 0.005),and FA values of left caudate nucleus (P ＜ 0.005),right caudate nucleus (P ＜ 0.001) and right putamen (P ＜ 0.05) were all significantly reduced in iRBD patients;FA value of right putamen was significantly decreased in PD patients (P ＜ 0.05).Compared with PD patients,the gray matter volume of left caudate nucleus of iRBD patients was significantly reduced (P ＜ 0.001),FA values of left caudate nucleus (P ＜ 0.01) and right caudate nucleus (P ＜ 0.005) of iRBD patients were significantly reduced.Conclusions There is atrophy of gray matter volume and extensive white matter fiber impairment in corpus striatum of patients with iRBD,and the white matter fiber impairment was similar to PD,which provides an anatomical evidence for iRBD being presymptom of PD.

Objective To explore the relation between abnormal functional connectivity of substantia nigra and impairment of movement and cognition in patients with rapid eye movement sleep behavior disorder (RBD). Methods A total of 22 subjects, including 14 patients with RBD and 8 sex, age, education-matched healthy controls, were enrolled in this study according to international diagnostic criteria. Unified Parkinson's Disease Rating Scale Ⅲ (UPDRS Ⅲ) and Hoehn-Yahr Stage were used to evaluate motor function. Digit Ordering Test - Attention (DOT - A), Symbol Digit Modalities Test (SDMT), Stroop Color-Word Test (SCWT), Trail Making Test (TMT), Rey-Osterrieth Complex Figure Test (ROCFT), Clock Drawing Test (CDT), Boston Naming Test (BNT) and Auditory Verbal Learning Test (AVLT) were used to evaluate cognitive function. The functional connectivity from left and right substantia nigra to brain region were examined. Results There were no statistical differences of UPDRSⅢ and Hoehn?Yahr Stage between 2 groups (P > 0.05, for all). In comparison with control group, SDMT (P = 0.001), ROCFT-copy (P = 0.013) and AVLT-N2 (P = 0.032) were significantly lower, while TMT-B test was significantly higher (P =0.005) in RBD group. Compared with control group, the functional connectivity of right substantia nigra to left precentral gyrus (P < 0.005) and right angular gyrus (P < 0.005) were all decreased in RBD group. Conclusions The results suggest that cognitive impairment occurs earlier than movement disorders in RBD, and there are abnormal functional connectivity from right substantia nigra to left precentral gyrus and right angular gyrus, proving that abnormal functional connectivity is the base of behavior disorders in RBD. DOI: 10.3969/j.issn.1672-6731.2017.09.005

Objective To investigate the abnormal functional connectivity (FC) between the substantia nigra (SN) and the brain motor area in rapid eye movement sleep behavior disorder (RBD) by Unified Parkinson′s Disease Rating Scale (UPDRS), Hoehn-Yahr Scale and resting state functional magnetic resonance imaging (rfMRI).Methods A total of 34 subjects (14 with RBD (RBD group), 12 with Parkinson′s disease (PD group), and 8 age, sex-matched normal controls (control group)) recruited in the Department of Neurology, Henan Provincial People′s Hospital from 2014 to 2015 were enrolled in this study according to international diagnosis criteria.All subjects were examined by UPDRS, Hoehn-Yahr Scale and rfMRI.Results UPDRS scores and Hoehn-Yahr staging were 0.00 (0.00, 3.75) and 0.00 (0.00, 0.50) respectively in the RBD group, which were significantly different from that in the PD group (30.5 (18.75, 33.00) and 1.75 (1.50, 2.50), respectively;Z=-3.782, P<0.05 and Z=-2.963, P<0.05), whereas not significantly different from that in the control group (0.00 (0.00, 0.00),0.00 (0.00, 0.00), respectively;Z=1.16, P>0.05 and Z=1.10, P>0.05).The FC analysis showed alterations from the right SN to bilateral cerebellum respectively among the three groups (Fright cerebellum=12.975, Fleft cerebellum=6.144;P<0.05);furthermore, there were significant increases in the RBD group compared with the PD group (t=-4.602,-5.080, P<0.005).The FC analysis showed reduced correlation from the right SN to the left prefrontal cortex in the RBD and the PD groups compared with the control group (t=-4.899,-3.500, P<0.005), but there was no statistically significant difference between the RBD and the PD groups (t=2.035, P>0.05).Conclusions There was abnormal FC from the SN to motor areas in RBD patients, and partial alterations were similar as PD patients.rfMRI provided an evidence that RBD might be presymptom of PD.

目的：总结抗NMDA受体脑炎的临床表现、诊断、治疗及预后等特点，以期提高对该病的认识。方法分析3例抗NMDA受体脑炎患者的临床表现、实验室检查、影像学检查、对免疫治疗的反应及预后。结果3例患者均表现有显著的精神症状，难治性癫痫和自主神经功能障碍，部分患者伴有运动障碍；脑脊液及血清抗 NMDA 受体阳性；免疫治疗有效。结论无精神病史的患者出现不明原因的精神症状伴痫性发作、记忆丧失、意识水平降低、运动障碍甚至出现中枢性通气不足，特别是伴有卵巢畸胎瘤者应警惕抗NMDA受体脑炎，及时诊断和治疗有助于抗NMDA受体脑炎患者的恢复。