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.

目的 分析肢体抖动型短暂性脑缺血发作(limb-shaking transient ischemic attack,LS-TIA)的临床及血管病变特点.方法 采用回顾性分析方法,收集我院2011年1月～ 2013年10月间以发作性肢体抖动为临床表现的TIA患者,收集患者的临床资料及治疗方案,记录临床和影像学随访结果,记录随访期间有无脑血管事件发生.结果 共收集肢体抖动型TIA患者5例,均存在抖动肢体对侧的颅内外大动脉重度狭窄或闭塞,除1例烟雾病患者,其余4例均接受抗血小板、强化他汀治疗,其中2例接受颈内动脉支架植入术治疗.发病后6个月复查头部MRI均未见新发梗死灶,临床随访6～33个月,均无脑血管事件发生.结论 单侧肢体抖动是TIA的一种发作形式,多存在对侧的颅内外大动脉重度狭窄或闭塞,血管重建治疗及规范的药物治疗对减少脑血管事件可能均有效.

目的 了解神经内科会诊骨科住院患者的科室分布、申请会诊方式以及疾病种类,探讨神经内科会诊骨科疾病的特点.方法 统计该院神经科医师自2011年5月1日-2012年4月30日会诊的骨科住院患者.对神经内科会诊骨科患者的一般情况、疾病诊断、急症会诊及常规会诊的疾病分类等进行统计分析.结果 共会诊患者458例,女性227例(49.3％),男性231例(50.4％),年龄分布16～89岁,中位数年龄62.5岁,平均年龄(60.2±15.6)岁.申请会诊数量最多的科室为脊柱骨科(40.2％)、创伤骨科(29.3％)及矫形骨科(17.0％).会诊患者的主要临床症状包括头晕(21.8％)、头痛(13.3％)及意识改变(12.2％).急症会诊共83例(18.1％),以代谢脑病最常见(31.3％),其次为头晕(30.1％)及脑梗死(12％).常规会诊共375例(81.9％),以术前评估最常见(33.9％),其次为头晕(13.3％),头痛位列第3(8.5％).神经内科会诊后推迟或取消手术19例(4.6％).结论 神经内科会诊骨科患者中,急症会诊最常见的是代谢性脑病,常规会诊最常见的是术前评估,其次为头痛及头晕.神经科会诊医师应充分重视神经内科疾病与骨科疾病的关系,以减少骨科围手术期的致残率及死亡率.

Objective To investigate the evaluation of preoperative neurology consultation in orthopedics inpatients through analysis of the clinical data in general hospital.Methods Preoperative neurology consultation for orthopedics inpatients including general condition,diagnosis,preoperative assessment consultation content and prognosis from May 1,2011 to April 30,2012 were analyzed retrospectively.Results A total of 164 patients were enrolled,with 86 female and 78 male,the age was 24-89 (60.2 ± 12.3) years.The main consultation departments were spinal department (37.8％,62/164),trauma department (31.1％,51/164) and orthopedic department (23.2％,38/164).The main preoperative diseases were cerebral ischemia (61.6％,101/164),old intracranial hemorrhage (9.1％,15/164) and Parkinson disease(8.5％,14/164).The neurology preoperative consultations including:(1)Directing the drug administration in the perioperative period,including vascular disease,Parkinson disease,epilepsy and other neurological disease.(2)Evaluating the cerebral ischemia risk of the orthopedics operation,cerebral vascular stenosis and collateral circulation.(3) The main complementary tests were transcranial doppler [52.4％ (86/164)],extracranial sonography [44.5％(73/164)] and computer tomography [37.8％(62/164)].(4)Altogether 8 operations had been cancelled.Three patients (2 cases of Alzheimer disease,1 case of Parkinson disease) experienced postoperative neurologic complications.Conclusion Neurology preoperative evaluation consultation in orthopedic patients is helpful to reduce their risk of stroke,improve the prognosis of patients,and avoid neurological complications.

目的 分析视神经脊髓炎谱系疾病(NMOSD)脑干损害的临床特点和影像学特征.方法 回顾性分析确诊的79例NMOSD患者,包括53例视神经脊髓炎和26例长节段横贯性脊髓炎脑干损害的临床特点,影像学特征以及与水通道蛋白4(AQP4)抗体的关系.结果 其中17例(17/79,21.5％)经临床或影像学证实有脑干损害.脑干损害组(17例)和无脑干损害组(61例)在性别、病程、单一/复发病程、扩展残疾状态评分(EDSS)无统计学差异,脑干损害组发病年龄小于无脑干损害组(32.29岁和39.39岁,P＜0.05).17例脑干损害的患者,12例有脑干受累的相关症状,包括复视(4/12,33.3％)、恶心和呕吐(9/12,75％)、呃逆(4/12,33.3％)、饮水呛咳(2/12,16.7％)、眩晕(2/12,16.7％).9例(9/12,75％)以脑干症状作为首发症状.受累部位在中脑(2/17,11.8％)、脑桥(6/17,35.3％)、延髓(12/17,70.6％)、颈髓延髓交界区(5/17,29.4％).脑干损害组AQP4抗体阳性率82.4％(14/17),无脑干损害组AQP4抗体阳性率75.8％(47/62),两组无显著性差异.结论 NMOSD脑干损害比较常见,呃逆、呕吐可作为疾病的首发症状,延髓或延髓颈髓延续病灶是较为特异的表现,早期识别有助于诊断和治疗.

Objective To investigate the differences of abnomal MRI and clinical manifestations in different periods of subacute combined degeneration(SCD)in different periods on the rate of abnomal MRI and clinical manifestations.Methods The patients were divided into two groups according to weather more than 6 months or not.The differences of SCD in different periods on the rate of abnomal MRI and clinical manifestations were compared.Results In a total of 30 cases of the SCD patients,11 cases of lesions were found on MRI with the rate of 36.7%.The rate of lesions on MRI was 55.6% when history less than 6 months,but 8.3% when history more than 6 months.The difference was statistically significant(P0.05).There were 16 cases which had numbness of the limbs as the first symptom with the rate of 53.3%.There were 9 cases when as history less than 6 months(50.0%),but 7 cases as historywhen more than 6 months(58.3%).The difference was not statistically significant(P0.05).There were 9 cases which had gait ataxia as the first symptom with the rate of 30.0%.There were 3 cases when as history less than 6 months(16.7%),but 6 cases when as history more than 6 months(50.0%).The difference was not statistically significant(P0.05).There were 11 cases with sphincter disturbances in the course of the disease.The rate was 36.7%.There were 2 cases when history less than 6 months(11.1%),but 9 cases when history more than 6 months(75.0%).The difference was statistically significant(P0.01).Conclusion More lesions on MRI were found in the early period of SCD.The incidentce of sphincter disturbances was higher in late SCD.

Objective Comparing the therapy and safety of on Batroxobin acute cerebral infarction between experimental group (72h~5d after onset) and control group (6~72h after onset). Methods Sixty cases of two groups were intravenous administrated every other day with Batroxobin for 3 times. The first doses was 10BU,the other two was 5BU. We recorded the NIHSS before therapy,after 2h,12h±20min,24h±20min,3d,7d,14d,1 month and 3 month. Mini-mental state examination(MMSE),Barthel Index(BI),Modified Rankin Scales(MRS) and safety evaluation ware collected at 1 and 3 month. Results NIHSS of experimental group was improved at 2h after therapy,that of control group was improved at 12h. Two groups were significantly improved at other points. Evaluation of BI and MMSE was more remarkably improved at 3 month than 1 month in experimental group. Two groups had no adverse effect. Conclusion Batroxobin is effective and safe to acute cerebral infarction after 72h onset.