Objective To investigate the features of visual and visuospatial dysfunction in patients with Parkinson’s disease (PD) ,and to explore the possible risk factors. Methods From July 2010 to July 2012 ,87 PD patients were enrolled in the Neurological Clinic of Beijing Hospital. An improved questionnaire first designed by Mc Dowell & Harris with 35 items about visual and visuospatial symptoms was applied to investigate the characteristics of visual and visuospatial disorders in PD patients. Additionally ,the relationship between visual‐spatial symptoms and clinical characteristics of the patients ,including gender ,age ,course of disease ,onset side , LED (levodopa equivalent dose ) ,unified Parkinson’s disease rating scale part 3 (U PDRS‐Ⅲ) score , mini‐mental state examination score (M M SE ) , Hamilton rating scale for depression score (HAMD ) and activity of daily living scale score (ADL ) were explored. Results 97.7% of the PD patients had one or more symptoms of visual‐spatial disorder , in w hich walking deviation／access deviation was the highest (55.2%) , followed by bumping (43.7%) . After removing symptoms due to PD motor symptoms ,the portion of PD patients with pure visual‐spatial disorders was still as high as 42.5% . T here were statistically significant differences in male ratio (P＝0.036) ,age (P＝0.012 ) ,left onset ( P＝0.026 ) between the two groups with and without spatial hypoxia. Significant differences also existed in age ( P＝0.041 ) and U PDRS‐Ⅲ score ( P＝0.041 ) between the two groups with and without sense of diminished field of vision. Significant differences were found in male ratio ( P＝0.026) and left onset (P＝0.044 ) between the two groups with and without difficulty in estimating depth／length. Statistically significant differences were also observed in age ( P＝ 0.040 ) and left onset ( P＝ 0.007 ) between the two groups with and without walking／fetching deviation. Significant differences were also found in left onset (P＝0.047) and U PDRS‐Ⅲ score (P＝0.024) between the two groups with and without difficulty of movement in narrow areas. T he ADL scores of PD patients with impaired spatial hierarchy ,walking／fetching deviation ,and difficulty in moving in narrow areas were higher than those without these symptoms (P＜0.05) . Conclusions Visual and visuospatial disorders are common in PD patients and may cause lower life quality. Age , gender ,side of onset ,UPDRS‐Ⅲ score maybe the possible risk factors.

Objective To analysis correlation of quality of social relationships a nd emotional symptoms in stroke hemiplegia patients.Methods 72 cases of stroke hemiplegia patients were selected as the observation object in the Department of Neurology of our hospital's from January 2016 ～ December 2016.Social relationship quality scale (SRQS)assessment of patients with quality of social relations.17 item Hamilton Depression Rating Scale (HAM D-17),14Hamilton Anxiety Scale (HAMA) and Beck-rafaelsdn Mania Rating Scale (BRMS) were used to measure the emotional symptoms.Pearson correlation analysis between social relationship and emotion symptoms.Results In these stroke hemiplegia patients,the total score of Social Relational Quality Scale (SRQS) is 32.8 ± 7.4.For the subscale analysis,Family Intimacy score is 13.8 ± 4.9;Family Commitment score is 10.2 ± 3.8,and Friendships score is 9.7 ± 2.9.For the emotion symptoms,the HAMD score is 54.8 ± 6.6,HAMA score is 48.1 ± 5.2,and BRMS score is 38.4 ± 4.5.Pearson correlation analysis showed that the total SRQS is negative associated depression,anxiety and mania symptoms in stroke hemiplegia patients (P＜ 0.01).The three subscale score of SRQS (Family Intimacy,Family Commitment and Friendships) were also negative associated depression,anxiety and mania symptoms in stroke hemiplegia patients (P ＜ 0.01).Conclusion There is a negative correlation between the quality of social relationship and emotion symptoms in stroke hemiplegia patients.Increasing the quality of social relationship may weaken the emotion symptoms.

Objective To analyze the clinical features of unilateral oculomotor nerve palsy caused by multiple myeloma(MM).Methods A 79-year-old female diagnosed MM for 3 years was admitted due to the disease relapse.The patient presented left oculomotor nerve palsy,bone marrow biopsy showed 4.5％ of myeloma cells,the serum level of λ light chain was up to 18 g/L,and brain CT/MRI scan revealed left parasphenoidsinus lesion.A diagnosis of relapsing intramedullary and extramedullary MM,IgGλ type,stage ⅢA was made.Ten cases were retrieved from literature since 1990,the clinical features of 11 cases were analyzed as follows.Results Among 11 cases of unilateral oculomotor nerve palsy caused by MM,7 were males and 4 females with a mean age of (60 ± 12) years (40-79 years).The sub-types of MM were IgG type (7 cases),IgA type (2 cases),biclonal of IgG and IgA type (1 case),and IgD type (1 case).Oculomotor nerve palsy was presented as the initial manifestation of MM in 9 cases,as a sign of relapse of MM in 1 case,and during MM treatment in 1 case.In 7 cases,oculomotor nerve was the only cranial nerve involved,while in other 4 cases,the Ⅳ,Ⅴ,Ⅵ cranial nerves were also involved.Neuroimaging revealed parasphenoid sinus lesions in 9 cases,and myeloma meningitis in 2 cases.Most of the reviewed cases achieved significant clinical improvement after chemotherapy and/or radiotherapy,except 2 cases with myeloma meningitis.The case in our department was improved significantly and rapidly after receiving chemotherapy with bortezomib.Conclusion MM should be considered when unilateral oculomotor nerve palsy is presented with or without the history of MM,the disease is sensitive to chemotherapy in most circumstances.

目的 总结抗LGi1抗体阳性边缘系统脑炎的临床与影像学特点,加深对于本病的认识.方法 报告于2013年11月25日就诊于北京积水潭医院神经内科门诊后收住院确诊的1例抗LGi1抗体阳性边缘系统脑炎患者的整个临床发病及诊治转归过程,对于患者的临床资料、影像学、电生理资料进行分析总结.结果 患者为中年女性,亚急性起病,主要临床表现包括极其频繁发作的面部肩部不适及面部抽动、频率稍低的发作性幻嗅、逐渐出现的记忆力下降、精神淡漠等认知及精神障碍;头部MRI在T2及Flair序列上见到了双侧颞叶内侧、海马区的高信号病灶,脑电图见到多导广泛的高幅慢波及棘慢或尖慢综合波等癫痫样放电;血及脑脊液LGi1抗体均阳性,同时血甲状腺抗过氧化物酶抗体及甲状腺抗球蛋白抗体均明显增高,提示叠加了Hashimoto脑炎,此外尚合并部分其他自身抗体的阳性;在接受了大剂量甲基强的松龙冲击并逐渐减量的治疗方案后,患者病情迅速缓解好转,随访1y未再复发.结论 抗LGi1抗体阳性边缘系统脑炎作为一种罕见的以损伤边缘系统为主的自身免疫性脑炎,在边缘系统脑炎基本表现的基础上,有其特殊的临床变现.同时还可以合并其他自身免疫相关脑炎及自身抗体阳性,尽早明确诊断并给予一线的免疫治疗可明显改善患者预后.

目的 分析探讨梅毒性脊髓炎临床和影像学特点.方法 回顾性研究我们确诊的2例梅毒性脊髓炎患者的临床及影像学资料,并结合相关文献复习,对上述资料进行分析总结.结果 共有11例梅毒性脊髓炎患者纳入分析.梅毒性脊髓炎临床罕见,归于脊膜血管梅毒一类.临床表现没有特异性,主要是横贯性脊髓脊膜损害.临床上大多数病例为急性或亚急性起病,进行性加重,仅有1例是缓慢起病,病程中突然恶化.影像学多表现为长节段胸段或颈胸段脊髓髓内病变,只有1例腰骶髓受累病例,还有1例同时累及颅内大动脉狭窄.梅毒性脊髓炎在T2加权像上呈现脊髓实质内长节段高信号病灶,增强扫描时可见脊髓表浅部位的局灶性强化,也称为脊髓“烛光征”;影像学虽有一定特征性,但同样也缺乏特异性.脑脊液检查全部表现为蛋白增高,细胞数增多,脑脊液细胞学为淋巴细胞为主的炎症,本病的确诊只能依据血清及脑脊液梅毒特异性抗体检测.梅毒性脊髓炎绝大部分病例治疗效果较好,但患者恢复的速度和程度却可以存在明显差异.绝大多数起病急、病程短、进展快的患者治疗效果既快又好,而病程相对较长、病史过程中有突发恶化特点的患者治疗效果可能不好.合并脑膜血管梅毒的1例患者也遗留了神经功能缺损.结论 梅毒性脊髓炎临床罕见,临床和影像学都不具有特异性,很容易误诊漏诊.当遇到长节段脊髓病变时要想到本病可能,应当尽快行血清及脑脊液梅毒相关抗体检测.在明确诊断后要尽快治疗,不同病程及病史特点的梅毒性脊髓炎患者对青霉素等抗生素治疗的反应可能会有所不同.

目的 总结分析硬脊膜动静脉瘘的临床及影像学特点,加深认识,提高诊治水平.方法 回顾性分析2004年6月～ 2014年9月在北京积水潭医院确诊的一组9例硬脊膜动静脉瘘患者的临床、影像学及治疗转归资料,总结其临床及影像学特点.结果 9例患者中男8例,女1例.病变部位:2例累及颈段,3例累及胸段,4例累及腰骶段及马尾神经根.缓慢进展4例,病程中有波动3例,误诊为其他疾病并在应用激素后病情突然恶化2例.临床上表现为不对称的横贯性脊髓和(或)神经根症状体征,二便和性功能全部受累.脊髓MRI多表现为长节段脊髓病变,T2像矢状位上见到"铅笔征",还可以在髓周见到串珠样、虫蚀样、或管样的多发血管流空影.受累节段越靠近腰骶段及马尾区,髓周见到异常血管流空影比例更高,病程中症状波动机会也更多.脊髓DSA检查见到动静脉瘘口和粗大迂曲扩张的引流静脉确诊.瘘口阻断手术和介入栓塞治疗都是可以选择的治疗办法.本病可以有复发,本组介入栓塞治疗组复发率更高.激素治疗后病情恶化的2例患者在明确诊断、尽快行手术治疗后,仍然可以逐渐缓慢恢复.结论 SDAVF是一组在临床和影像学上有其特点的疾病,脊髓MRI检查有助于诊断,脊髓DSA检查是确诊的手段,当怀疑或者临床不排除本病可能时,避免首先使用激素治疗,而应当先尽早明确诊断.瘘口阻断手术和介入栓塞治疗都是治疗SDAVF的有效方法,即使是激素应用后病情恶化的患者,及早进行手术或栓塞治疗也是有帮助的.