目的 探讨全膝关节置换术围手术期脑梗死的发病率、危险因素以及预后,以指导围手术期风险管理.方法 选取我院2008-2018年行全膝关节置换术的患者,记录患者的人口学资料、日常生活能力评分、术前合并症、手术类型、麻醉方式、是否进入重症监护室、输入红细胞、血小板以及血浆的数量.使用χ2检验和t检验分析患者预后,使用logistic回归模型分析全膝关节置换术围手术期急性梗死的危险因素.结果 共16490例患者行全膝关节置换手术,男性3453例,年龄18～90岁,平均年龄64.24岁.11例患者出现全膝关节置换手术后脑梗死,男性3例,女性8例,年龄55～80岁,平均年龄68.82岁,围手术期脑梗死的发病率为0.07％.术后脑梗死发生的时间1～3 d,中位数为1 d.围手术期脑梗死患者出院时日常生活能力显著下降(40.50 vs.56.35,P<0.001),进入监护室比例明显增加(1.10％vs.27.30％,P<0.001).单因素分析显示,高血压、糖尿病、心房纤颤、高脂血症、脑梗死病史、贫血以及双侧手术方式是围手术期脑梗死的危险因素(P<0.05).多因素logistic回归分析显示,脑梗死病史(OR=88.586,95％CI:22.076～355.561,P<0.001)、心房纤颤(OR=18.230,95％CI:1.637～203.026,P=0.018)和双侧手术方式(OR=7.226,95％CI:1.985～26.304,P=0.003)是全膝关节置换术围手术期脑梗死的危险因素.结论 全膝关节置换术围手术期脑梗死患者预后差,对具有脑梗死病史、心房纤颤及双侧膝关节置换术等危险因素的患者进行识别预警,以减少围手术期脑梗死的发生.

Objective To discuss the relationship between emergency biochemical indicators before intravenous thrombolysis and the early clinical improvement and prognosis after intravenous thrombolysis of acute ischemic stroke. Methods From June 2014 to May 2019,67 patients with acute ischemic stroke were treated by intravenous thrombolysis with ateplase in Beijing Jishuitan Hospital. The NIHSS score before thrombolysis and 24 hours after thrombolysis were re-corded,and the early clinical improvement was assessed by the difference between them(ΔNIHSS). ΔNIHSS≥4 was di-vided into excellent group,1-3 into effective group and ≤0 into ineffective group. Early prognosis was recorded by Modi-fied Rankin score(mRS)on 7 days after thrombolysis:mRS score(≤2)was divided into good prognosis group and 3-4 into general prognosis group and 5-6 into poor prognosis group. To compare the differences of biochemical indicators before thrombolysis among the above groups. The measurement data were expressed as mean ± standard deviation(χ ± SD). Inde-pendent sample t test was used to compare the two samples. Results 1. Compare the emergency biochemical indicators be-tween the excellent group(n=10)and the ineffective group(n=30)at 24hours after thrombolysis,the indicators which have significant difference include:T-Bil(14. 28 ± 4. 77 mmol/L VS 11. 22 ± 3. 86 mmol/L,P=0. 048),blood glucose(6. 82 ± 1. 18 mmol/L VS 8. 99 ± 4. 31 mmol/L,P=0. 017). Compare the emergency biochemical indicators between the effective group(n=10)and the ineffective group(n=30)after thrombolysis 24hours,the indicators which have significant difference include:T-Bil(14. 89 ± 5. 88 mmol/L VS 11. 22 ± 3. 86 mmol/L,P=0. 007),D-Bil(4. 63 ± 1. 89mmol/L VS 3. 62 ± 1. 22mmol/L,P=0. 019),blood glucose(6. 86 ± 1. 50 mmol/L VS 8. 99 ± 4. 31 mmol/L,P=0. 016). 2. Compare the three different prognosis group at 7 days after thrombolysis. There was significant difference in uric acid level before throm-bolysis between the good prognosis group(n=56)and the poor prognosis group(n=9)(397 ± 96. 62 mmol/L VS 344. 33 ± 59. 46 mmol/L,P=0. 041). Conclusion Higher level of bilirubin and lower level of blood glucose before thrombolysis were correlated with the better early clinical improvement at 24 hours after thrombolysis,while higher level of uric acid be-fore thrombolysis were correlated with better prognosis at 7 days after thrombolysis.

目的 分析糖尿病周围神经病变(DPN)合并腕管综合征(CTS)的电生理特点,探讨电生理检查对于鉴别DPN合并CTS的诊断价值.方法 将2013年5月至2017年4月于我院门诊和病房收治的DPN、CTS及DPN合并CTS患者共122例进行回顾性分析,分为CTS组51例 、DPN组30例 、DPN合并CTS组(DPN+CTS)41例,另选健康对照(NC)组32名,行神经电生理检查包括正中神经运动末端潜伏期(DML)、正中神经复合肌肉动作电位(CMAP)、正中神经感觉传导速度(SCV)、正中神经分段SCV(指-掌SCV及掌-腕SCV)、正中-桡神经潜伏期差(MRLD)及正中-尺神经潜伏期差(M ULD).结果DPN+CTS组掌-腕SCV低于DPN组[(37.66±5.78)vs(45.57±3.89)m/s,P<0.05],MRLD、MULD大于DPN组[(0.56±0.19)vs(0.19±0.08)ms,(0.57±0.18)vs(0.20±0.09)ms,P<0.05];DPN组 、DPN+CTS组与NC组比较,正中神经DML均延长[(3.99±0.57)vs(4.68±0.88)vs(3.24±0.37)ms,P<0.05],SCV均降低[(46.22±5.46)vs(41.39±5.25)vs(52.60±3.36)m/s,P<0.05].结论常规电生理检查鉴别DPN合并CTS有一定困难,分段感觉传导速度SCV及MRLD、MULD对鉴别DPN合并CTS有诊断价值.

Objective To investigate the value of serum uric acid level in the differential diagnosis of epilepsy and syncope.Methods A total of 208 patients with epilepsy (106 patients) and syncope (102 patients) who visited the neurology emergency department of Beijing Jishuitan Hospital from November 2012 to April 2017 were enrolled into this study.Venous blood was collected within 12 hours of onset to measure the serum uric acid level by uricolase method.Results The serum uric acid level of the epilepsy group (529.28 ± 202.35) μmol/L was significantly higher than that of the syncope group (306.84 ±76.02) μmol/L(t =10.417,P ＜0.01).The proportion of elevated serum uric acid level in epilepsy group (71.70％) was significantly higher than that (8.82％) in syncope group (x2 =85.035,P ＜0.01).Serum uric acid levels were negatively correlated with age in the epilepsy group (r =-0.302,P =0.002).Serum uric acid levels were not associated with age in the syncope group (P =0.653).25 cases of epilepsy with elevated uric acid were followed up:serum uric acid levels dropped to normal within 3 days after the onset of epilepsy in 88％ of the patients (22 patients),serum uric acid levels of the other 3 cases (12％) were significantly lower than before.Conclusion Serum uric acid level has certain clinical value for the differentiation of epilepsy and syncope.

Objective..To investigate the effects of different doses of atorvastatin combined with clopidogrel on coagulation function and serum high-sensitivity C-reactive protein (hs-CRP) level in cerebral infarction patients.Methods:Clinical data of 650 cerebral infarction patients treated in our hospital from January 2009 to December 2015 were retrospectively analyzed.Patients were divided into an observation group (n =225) and a control group (n =425),according to different treatment methods.The patients in the control group received low-dose atorvastatin (20 mg/d) and clopidogrel (75 mg/d),while those in the observation group received high-dose atorvastatin (40 mg/d) and clopidogrel (75 mg/d).The platelet (PLT) count,erythrocyte sedimentation rate (ESR),prothrombin time (PT) and activated partial thromboplastin time (APTT) as well as the serum levels of fibrinogen (FIB),D-dimer and hs-CRP were compared between the two groups.Results:The response rate in the observation group was higher than that in the control group (97.41％ vs.92.0％,P ＜0.05).Before treatment,there were no significant differences in the PLT,ESR,APTT,PT and FIB level between the two groups (P ＞0.05).After treatment,the ESR and FIB level in the observation group were lower than those in the control group (P ＜0.05),whereas the PT,APTT and PLT count were similar in the two groups (P ＞0.05).No significant difference was found between the two groups in the serum D-Dimer and hs-CRP levels at pretreatment (P ＞0.05).After treatment,the serum levels of D-Dimer and hs-CRP in both groups were decreased (P ＜0.05),but they were more significantly decreased in the observation group (P ＜ 0.05).Conclusion:Atorvastatin combined with clopidogrel achieved a good curative effect for the treatment of cerebral infarction,with few adverse reactions and high safety.High-dose (40 mg/L) of atorvastatin was more effective in improving the coagulation function and inhibiting the inflammatory response than using low-dose (20 mg/L).

Objective:To investigate the value of serum myoglobin (Mb) and creatine kinase (CK) levels in the differential diagnosis of epilepsy and syncope.Method:A total of 163 patients with generalized tonic clonic seizure (80 patients) and syncope (83 patients) who visited the neurology emergency department of our hospital from November 2012 to May 2017 were enrolled into this study.Venous blood was collected within 12 hours of onset to measure the serum Mb level by Chemiluminescence method and the serum CK level by rate method.Result:The serum Mb level of the epilepsy group [111.65(65.88～193.80) ng/ml] was significantly higher than that of the syncope group [26.40(18.80～45.30) ng/ml](P＜0.01);The serum CK level of the epilepsy group [122.00(77.50 ～193.50) IU/L] was significantly higher than that of the syncope group [79.00(59.00～110.00) IU/L](P＜0.01).The proportion of elevated serum Mb level in epilepsy group (75.00％) was significantly higher than that in syncope group (8.43 ％)(P＜0.01);The proportion of elevated serum CK level in epilepsy group (30.00％) was significantly higher than that in syncope group (7.23％)(P＜0.01).For patients with transient cognitive impairment who were diagnosed with epilepsy or syncope,elevated serum Mb level had a sensitivity of 75 ％ and a specificity of 91.57％ for diagnosis of epilepsy,while elevated serum CK level with a sensitivity of 30％ and a specificity of 92.77％.Conclusion:Serum Mb level has certain clinical value for the differentiation of generalized tonic clonic seizure and syncope.Serum CK level is less sensitive to differentiate epilepsy from syncope.

Objective: To investigate the value of split hand in the differential diagnosis of amyotrophic lateral sclerosis(ALS) and cervical spondylotic amyotrophy (CSA). Methods: A total of 62 ALS patients, 57 CSA patients and 65 normal controls who visited the Neurology and Spine Department of Beijing Jishuitan Hospital from May 2013 to June 2017 were enrolled into this study.The amplitudes of compound muscle action potentials (CMAP) were recorded from abductor digiti minimi (ADM) and abductor pollicis brevis (APB). Moreover, the ratio of CMAP amplitude between ADM and APB (ADM/APB) was calculated. Results: The ADM/APB of the ALS group (1.93±1.97)was significantly higher than that of the normal control group (0.92±0.22)(P<0.05); the ADM/APB of the CSA group (0.74±0.32)was significantly lower than that of the normal control group (0.92±0.22)(P<0.05); the ADM/APB of the ALS group (1.93±1.97)was significantly higher than that of the CSA group(0.74±0.32)(P<0.05); the areas under receiver operator characteristic curve in patients with ALS was 0.843, the diagnostic sensitivity and specificity for ALS were 75.8% and 83.1% with the cutoff value of ADM/APB=1.077; the areas under receiver operator characteristic curve in patients with CSA was 0.737, the diagnostic sensitivity and specificity for CSA were 64.9% and 87.1% with the cutoff value of ADM/APB=0.739. Conclusion: The ADM/APB has certain clinical value for the differentiation of ALS and CSA.

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.

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 effectiveness and safety of thrombolytic therapy for cardiogenic cerebral embolism by intravenous recombinant tissue plasminogen activator(rt-PA). Methods Retrospective analysis was used in this study. Forty five patients who was hospitalized between January 2007 and December 2011 in Beijing Jishuitan Hospital and suffered from cardiogenic cerebral embolism were selected. Twenty two patients were treated with rt-PA within 4.5～12 hours from the onset(4.5 hours for anterior circulation infarct and 12 hours for posterior circulation infarct) as thrombolytic group and 23 patients who accepted regular secondary prevention of acute ischemic stroke were selected as controls. The Oxfordshire Community Stroke Project(OCSP) classification, risk factors, past history and drug history and neurological deficits were collected. The neurological deficits, and the incidence of favorable outcome on the base line, 24th hour and 90th day were compared. The incidence of hemorrhagic transformation(HT) and mortality within 90 days were analyzed.Results There was no significant difference in the National Institutes of Health Stroke Scale(NIHSS) score and OCSP classification in the onset between both groups. On the time of 24 hours after the onset, the NIHSS score improved significantly in the thrombolytic group(4.5[-1.5, 8]) compared with the control group(0[0, 3])(P=0.04). The incidence of early improvement in the thrombolytic group(54.55%) increased compared with the control group(34.78%). But there was no significant difference in it between both groups. On the 90 days after onset, the NIHSS score improved significantly in the thrombolytic group(9[7,11]) compared with the control group(3.5[2, 4.75])(P <0.01). The incidence of favorable outcome in the thrombolytic group(22.7%～36.4%) increased compared with the control group(17.4%～26.1%). But there was no significant difference in it between both groups. There were 4 HT cases and 3 symptomatic intracranial hemorrhage(sICH) cases in each group. In the control group, all the 4 HT cases had diabetes history and the 4 cases in the thrombolytic group did not. The systolic blood pressure in the 6 sICH cases were over 140 mmHg. There were 3 death cases in each group within 90 days from the onset. All of the 6 cases had high score of NIHSS. The OCSP classification of most of the deaths was total anterior circulation infarct(TACI).Conclusion Giving rt-PA to the patients who suffered from cardiogenic cerebral embolism could improve the neurological deficits on the time of 24 hours and 90 days after the onset. Intravenous thrombolysis with rt-PA is relatively safe.

目的 探讨硬脊膜动静脉瘘(spinal dural arteriovenous fistula,SDAVF)的临床和影像学特点,提高对于SDAVF的认识.方法 对6例确诊SDAVF患者的临床、影像学资料以及治疗与转归等进行回顾性分析.结果 6例患者中5例为中老年男性,1例为青年女性.6例患者的病灶部位均在胸腰段脊髓,其中5例有脊髓圆锥及马尾神经根受累.双下肢麻木症状发病5例,骶尾部或双下肢疼痛发病2例,下肢无力发病2例,6例患者均有尿便和性功能障碍,1例急性起病,5例亚急性或慢性起病.2例呈进行性病程,无明显波动,4例病程中病情有明显波动.脊髓磁共振检查(magnetic resonance imaging,MRI)全部病例均见到脊髓轻度增粗和髓内弥漫性长T<,2>异常信号灶,4例脊髓表面尤其是背侧可见到迂曲血管流空影,腰骶段受累病例更加明显.6例均行脊髓数字减影血管造影(digital subtraction angiography,DSA)检查,见到供血动脉形成的动静脉瘘口以及明显蜿蜒迂曲延长的引流静脉从而确诊本病.全部6例患者的临床体征水平与影像学上病变水平均不甚一致,影像学表现重于临床.4例患者接受了1～3次的介入拴塞治疗,其中3例取得较好疗效,但这3例患者均有复发.结论 SDAVF有其临床和影像学特点.男性中老年患者多见,主要为胸段脊髓或腰骶段脊髓、神经根的症状体征,下肢感觉异常、疼痛、步态异常或运动障碍、尿便和性功能障碍均是常见的临床表现,可先后受累.病程可呈慢性进展,可在波动中进展,也可以进展中有波动.脊髓MRI检查对于本病的诊断可有提示.及早规范的血管栓塞或手术治疗可能取得较好疗效.

<正>多系统萎缩(multiple system atrophy,MSA)是一组病因不明的,累及锥体外系、锥体系、小脑和自主神经等多系统的神经系统变性病,分为MSA-P(帕金森症状突出)和MSA-C(小脑症状为主)两型。MSA临床症状复杂,不同亚型之间症状重叠,加之病理取材困难,早期诊断颇具挑战。临床结

目的:分析脑脂肪栓塞患者的临床表现、实验室检查及神经影像学特点,以提高脑脂肪栓塞的诊治水平.方法:回顾性分析18例临床确诊的脑脂肪栓塞患者的临床表现、实验室检查及神经影像学资料.结果:经治疗后,16例患者完全恢复未遗留任何神经系统体征,2例患者呈持续昏迷状态未行骨科手术,未出现死亡病例.结论:脑脂肪栓塞好发于骨折及骨科术后2～3 d,其临床表现、实验室检查及神经影像学检查具有一定特征性,早期诊断和及时治疗,可有效地防治呼吸衰竭等潜在的致命性病程.

Objective To study Schwartz-Jampel Syndrome (SJS) in clinical manifestation,laboratory findings,etiology,diagnosis and treatment.Method To analyse clinical findings,imaging,electromyography and muscular biopsy of the first reported siblings of SJS in China.Result The onset of SJS is in childhood with weakness,gait abnormality,facial dysmorphism,myotonia,skeletal abnormalities and joint contracture.Our patients have mild increased creatase,severe deformity of elbows and hips,platyspondyly and scoliolosis with generous myotonia potential in electromyography.Muscle biopsy exhibits both myopathic and neuropathic changes.Conclusion SJS is a rare autosomal recessive inheritance disease and relatived to perlecan.Clinical manifestation of SJS is diversity and characterized by typical facies,generalized myotonia and skeletal abnormalities.Carbamazepine is effective for treatment.

低钾型周期性瘫痪已被公认为是一种骨骼肌离子通道病,但本病确切发病机制仍不清楚,疾病发作时血清钾低于正常是本病的特点之一,但逐渐有学者发现该病患者伴有血清磷酸肌酸激酶(CPK)升高.现将我院1996年1月～2006年12月期间住院的并同期检查血清钾和CPK的周期性瘫痪患者36例临床资料和实验室结果进行回顾性分析.

Objective To investigate the effects of rt-PA on the prognosis of ultra-acute ischemic stroke, we analyzed the outcome of thrombolytic therapy by IV rt-PA at different doses. Methods 308 cases of ultra-acute ischemic stroke patients were divided by intention into two groups: thrombolytic therapy group and control group. For 221 patients in the thrombolytic therapy group: IV rt-PA were given at different doses: 0.9mg/kg for 92 patients(time window within 3hr in 68 cases, 3 to 4hr in 9 cases and 4 to 6hr in 15 cases) and 0.6-0.8mg/kg for 129 patients(time window within 3hr in 72 cases, 3 to 4hr in 24 cases and 4 to 6hr in 33 cases). The control group consist of 87 patients who were not administered by rt-PA. NIHSS score and Barthel Index were evaluated at baseline, 24h after treatment and 90d prospectively. Neurological outcome was categorized as favorable(BI ≥95)or unfavorable(BI≥95) at 90d after treatment. Intracranial hemorrhage included symptomatic intracranial hemorrhage(SIH) and asymptomatic intracranial hemorrhage(AIH). Vascular fatal events and re-stroke events were also recorded at the same time during the follow-up periods. Multivariable logistic regression was used to explore the independent prognostic factors. Results The independent factors for good outcome are baseline NIHSS(OR=2.067, 95%CI 1.201-3.556, P=0.009), Cornory Heart disease(OR=1.942, 95%CI 1.040-3.625, P=0.037)and thrombolytic therphy(rtPA 0.9mg/kg, OR=0.414, 95%CI 0.207-0.826, P=0.012; rtPA 0.6-0.8mg/kg,OR=0.261, 95%CI 0.137-0.497, P<0.001). The rate of symptomatic intracranial hemorrhage(SIH) in the thrombolytic therphy group were 3(3.3%) for rtPA 0.9mg/kg and 6(4.7%) for rtPA 0.6-0.8mg/ kg. The difference is no signifi cant. Conclusion Using IV rt-PA in ultra-acute ischemic stroke may have a good effect at our study. There is no statistic difference on prognosis between B group(rt-PA 0.6-0.8mg/kg ) and A group(rt- PA 0.9mg/kg). Accompanying history of atrial fi brillation and diabetes may affect prognosis.This should be further studied by more strictly designed RCT.