目的 分析以脑干受损症状为首发表现的视神经脊髓炎谱系疾病(NMOSD)患者的临床特征及预后情况,以提高临床工作者对此病的认识,减少早期误诊率.方法 收集2009年1月～2020年8月本院收治的符合国际NMOSD诊断标准的185例患者的临床资料,分析其中的28例以脑干受损症状为首发表现的NMOSD患者的临床特点、体液学检查、影像学特征及预后.比较以脑干受损症状为首发表现的NMOSD与以其他症状为首发表现的NMOSD的临床特点及预后.结果 以脑干受损症状为首发表现患者共28例(15.14％,28/185),其中17例患者以顽固性恶心、呕吐和/或呃逆为主要症状,4例以复视为主要症状,其余以头晕、面部麻木、瘙痒等为主要症状.血清水通道蛋白4(AQP4)-IgG抗体阳性率为(76％,19/25),MRI检查显示病灶通常位于最后区、延髓背侧、脑桥和第四脑室周围区域.28例患者中,15例被误诊,误诊患者较未误诊患者,神经内科作为首诊科室的比例低(P＜0.05).与其他症状起病的NMOSD患者相比,以脑干受损症状为首发表现的NMOSD患者的误诊率和年复发率均更高(均P＜0.01).结论 以脑干受损症状为首发表现的NMOSD患者在临床上并不少见,病损通常位于最后区、延髓背侧、脑桥和第四脑室周围区域.血清AQP4抗体、头颅MRI检查有助于诊断.此类患者早期误诊率高,首诊于非神经科科室时更易被误诊,且较其他症状起病的NMOSD更易复发.

目的:探讨非感染性颅内静脉血栓形成(CVST)合并脑出血(ICH)的临床特点.方法:2017年1月-2020年1月收治非感染性CVST患者68例,包括出血组22例和未出血组46例.比较两组患者的一般资料、既往病史、潜在危险因素及临床表现.结果:出血组患者年龄高于未出血组,差异有统计学意义(P<0.05);出血组妊娠和产褥期患者的比例高于未出血组,差异有统计学意义(P<0.05);出血组癫痫、失语及意识障碍患者的比例高于未出血组,差异有统计学意义(P<0.05).结论:非感染性CVST合并ICH患者的年龄较大,妊娠和产褥期患者占比高,癫痫、失语、意识障碍患者更为常见.

目的 探讨使用苍白球内侧核低频刺激治疗帕金森病冻结步态的疗效.方法 观察在徐州医科大学附属医院帕金森病中心进行脑深部电刺激手术,并选用苍白球内侧核作为手术靶点的2例患者,每例先后用高频及低频电刺激,观察其对帕金森病冻结步态的疗效.结果 与高频刺激相比,患者使用低频刺激后冻结步态问卷评分改善更多.结论 苍白球内侧核低频刺激可以改善帕金森病冻结步态,且震颤症状并未加重.

Objective To explore the clinical features and affect factors of Parkinson’s disease ( PD) with freezing of gait (FOG).Methods The clinical and related data of 106 patients with PD who were treated in the neurology department of the Affiliated Hospital of Xuzhou Medical University from September 2016 to May 2018 were collected.According to the FOG questionnaire ( FOG-Q), the subjects were divided into PD without FOG group (PD-FOG group, 59 cases) and PD with FOG group ( PD+FOG group, 47 cases).The clinical data of the two groups were compared and analyzed.Results The incidence of PD with FOG in this study was 44.3%.There were no significant differences in gender , age, MMSE, Montreal cognitive assessment scale ( MoCA), Hamilton anxiety scale, Powosi sleepiness scale between PD +FOG group and PD-FOG group (all P＞0.05).The scores of Hamilton depression scale, PD questionnaires-39, rapid eye movement sleep behavior disorder screening scale , Hoehn-Yahr (H-Y) stage, unified PD rating scale-Ⅲ(UPDRSⅢ) in the PD+FOG group were higher than those in the PD-FOG group (all P＜0.05).In the MMSE subproject, the scores of memory and recall items in the PD +FOG group were statistically lower than those in the PD-FOG group (all P＜0.05).In the MoCA subproject, The PD+FOG group had lower visual space and execution ability , naming, and language scores than the PD-FOG group (all P＜0.05). Univariate and Logistic regression analysis showed that the course of disease and H -Y grading were risk factors of the occurrence of FOG in PD patients (all P＜0.05).Conclusions FOG is a common abnormal gait in the middle and late stage of PD patients.PD patients with FOG are more likely to suffer from depression , lower quality of life and sleep than those without FOG.Cognitive impairment is more prominent in memory , memory, naming, language, visual space and executive ability.The duration of disease and high H-Y staging are risk factors for PD patients with FOG, which might aggravate the progression of the disease.

目的 探讨帕金森病(PD)相关性疼痛的相关因素及其对生活质量的影响.方法 根据是否伴有疼痛将120例PD患者分为疼痛组(49例)和非疼痛组(71例).采用PD统一评分量表(UPDRS)和Hoehn-Yahr (H-Y)分级评估患者的严重程度,采用PD生活质量量表-39(PDQ-39)测评其生活质量,用数字评分法(N RS)评估疼痛组患者疼痛程度.采用汉密尔顿抑郁量表(HAMD)、汉密尔顿焦虑量表(HAMA)和MMSE评价患者的焦虑、抑郁及认知情况.结果 与无疼痛组比较,疼痛组H-Y分期及UPDRSI、UPDRSⅢ服药后(med-on)、UPDRSⅢ服药前(med-off)、PDQ-39、HAMA、HAMD评分均显著升高(P＜0.05～0.01).Spearman相关分析显示,NRS评分与H-Y分级及UPDRSI、UPDRSⅢmed-off、UPDRSⅢmed-on、PDQ-39、HAMA、HAMD评分呈正相关(P ＜0.05 ～0.01),与年龄、发病年龄、病程、受教育年限及UPDRSⅡ、MMSE评分无相关性(均P＞0.05).线性回归分析显示,UPDRSⅡ、HAMA、NRS评分对PDQ-39有显著性影响(均P＜0.01).结论 PD相关性疼痛可能与精神活动、运动症状、焦虑抑郁相关.PD相关性疼痛是影响PD患者生活质量的独立预测因子.

目的 探讨丘脑底核脑深部低刺激术对帕金森病患者认知功能的短期影响.方法 17例行丘脑底核脑深部电刺激术帕金森病患者,比较其术前评估及术后随访1年蒙特利尔认知评估量表认知域变化.结果 患者术后1年蒙特利尔认知评估量表总分、视空间与执行、注意力、语言、延迟记忆及定向力评分提高,抽象能力认知域评分下降,无统计学差异(P>0.05),命名认知域评分提高有统计学差异(P<0.05).结论 短期内丘脑底核脑深部电刺激术对部分帕金森病患者大体认知功能可能有改善作用,且以命名认知域为主.

目的 回顾性分析7例抗GAD65抗体脑炎患者的临床特点.方法 回顾7例GAD65抗体脑炎患者,总结其临床表现和脑电图、实验室检查、影像学检查结果.结果 中位年龄44岁,7例患者中仅表现为癫痫4例;表现为头晕,合并有垂直眼震者1例;癫痫合并有僵人综合征1例;表现为精神行为异常症状.脑电图异常者5例,5例表现为局灶性尖波.头MRI显示颞叶异常信号(海马硬化2例)3例.结论 抗GAD65抗体脑炎是可治性疾病,表现为癫痫、精神行为异常、记忆力减退,认知障碍,共济失调、脑干受累症状、僵人综合征,临床表现多样性,早期诊断,免疫治疗提高患者预后.

Objective To investigate the prevalence ,characteristics ,and risk factors of pain in patients with Parkinson’ s disease (PD) . Methods A totaI of 128 PD patients were enrolled in the study and assessed with the following rating scales : Unified Parkinson’ s Disease Rating Scale (U PDRS) , Hoehn‐Yahr Scale (H‐Y) , Hamilton Depression Rating Scale for Depression (HRSD) , Visual Analogue Scale (VAS ) and Montreal Cognitive Assessment (MoCA ).Results There were 63 cases (49.2%) of PD patients w ho repoted pain , and their average mean VAS score was 3.93 ± 2.81. T he pain symptous of 52 cases (82.5%) occarred ohronically , and 11 cases (17.5%) accurred acutely. 76.2% of the PD patients with pain were presented with musculoskeletal pain ,23.8% with radicular‐neuropathic pain ,12.7% with central neuropathic pain ,9.5% with dystonic pain ,and 7.9% with akathisia pain. Further more ,only 42 of the PD patients with pain received pain related treatment. Compared with PD patients without pain , PD patients with pain had higher scores of U PDRS , H‐Y and HRSD scores. Depression (scores of HRSD ) was the only factor associated with pain showed by the logistic regression model (P＝0.006) .Conclusions T he frequency of pain in PD patients is high , the clinical manifestation is diverse , and depression is a predictor of pain in PD patients.

目的探讨磁敏感成像(SWI)在帕金森病DBS手术术前针道及靶点设计中的应用价值.方法10例丘脑底核脑深部电刺激术(STN-DBS)治疗的原发性帕金森病患者,术前行3.0T磁共振3D T1平扫和增强扫描、T2轴位和冠位扫描及SWI扫描.每例患者针道设计:单侧分中央、前方、后方、内侧及外侧共5个针道,双侧共10个针道.本研究10例患者总计100个针道,比较每个针道在T2像、T1增强像及SWI上血管分布情况.测量每个针道在SWI及T2轴位上STN的宽度.结果T2像上12％的针道有血管分布,主要是硬脑膜及脑表面血管.T1增强像上22％针道有血管,主要分布在脑表面及脑组织内,以动脉性血管为主.SWI上62％针道有血管分布,相对于T1增强像与T2像能更多显示额外的脑组织内小血管,特别是小的静脉血管.轴位上SWI显示的STN的宽度明显小于T2像.结论SWI与T1增强像及T2像结合可优化DBS术前针道设计,减少术中穿刺损伤血管导致颅内出血的风险.SWI与T2像结合可科学确定STN的边界.

目的:进一步明确基于中国人群的短暂性脑缺血发作(transient ischemic attack,TIA)病人,3种ABCD评分系统预测TIA发作后7 d内发生新发脑梗死风险的临床应用价值.方法:150例TIA病人于入院48 h内采用ABCD2、ABCD3、ABCD3-Ⅰ3种评分系统,根据评分分为低危、中危及高危组;根据病人TIA发作后7 d内是否继发脑梗死分为新发脑梗死组和非脑梗死组,比较2组TIA病人的3种评分系统评分;记录3种评分的低、中、高危组中TIA后7 d内继发脑梗死病人例数.结果:与非脑梗死组比较,新发脑梗死组的3种不同的ABCD系统的评分结果均升高,差异具有统计学意义(P<0.05～P<0.01);除ABCD2评分外,随着ABCD3、ABCD3-Ⅰ评分分值的升高,7 d内继发脑梗死率不断增加(P<0.01);ROC曲线分析发现,3种ABCD评分系统对7 d内继发脑梗死的预测AUC分别为0.67、0.84和0.91,均大于基准线面积的0.5,差异有统计学意义(P<0.01).ABCD3-Ⅰ预测7 d内继发脑梗死准确度优于ABCD3,ABCD3优于ABCD2,差异具有统计学意义(P<0.01).结论:3种ABCD评分方法均能预测TIA后7 d内继发的新发脑梗死,分值越高,7 d内继发新发脑梗死的危险度越高,ABCD3-Ⅰ预测准确度最高.

目的 探讨丘脑底核深部脑刺激术(STN-DBS)对原发性帕金森病的疗效,分析静止性震颤的改善情况.方法 43例行双侧STN-DBS治疗的原发性帕金森病患者,术后平均随访24.5个月.具体如下:① 统计脑深部电刺激术(DBS)手术对UPDRSⅢ运动评分、H-Y分期及静止性震颤的改善情况.②从多巴胺药物反应型及药物无反应型、左侧肢体起病型与右侧肢体起病型及上肢、下肢、下颌/嘴唇震颤等3个维度重点分析具体的静止性震颤的疗效.③统计震颤的程控参数,研究程控参数与帕金森病静止性震颤改善的相关性.结果 术后随访显示:H-Y分期下降(0.70±0.58)期,UPDRSⅢ运动评分改善47.83％,震颤改善70.19％.具体分析震颤改善率:药物反应型为69.16％、无反应型为76.58％;左侧起病型为60.78％、右侧起病型为76.35％;上肢为85.97％、下肢为70.77％、下颌/嘴唇为53.23％.术后程控需要130 Hz以上的高频刺激震颤才能得到很好改善.结论 STN-DBS治疗原发性帕金森病运动症状疗效显著,UPDRSⅢ运动评分及震颤明显改善,H-Y分期下降.多巴胺药物无反应型静止性震颤改善率好于反应型,右侧肢体起病者好于左侧肢体起病者,上肢好于下肢及头部.震颤的程控仍然以高频刺激为主,其次为高电压.

Objective: To analyze the clinical and imaging features of hypertrophic cranial pachymeningitis (HCP).Methods:One case of HCP was reported,and the relevant literature was reviewed and retrospectively analyzed.Results:The patient experienced headache at the onset of illness along with decreased visual acuity. Hematology test:ANA(+).Lumbar puncture:cerebrospinal fluid pressure≥330 mmH2O.Cerebral MRI,plain and enhanced: cerebral falx and tentorium of cerebellum, bilateral frontal parietal dura thickening and enhancement.Dural biopsy:fibrous hyaline tissue scattered around lymphocytes and plasma cells,most apparent surrounding blood vessels. Immunohistochemistry: CD138 (+), IgG (individual cells +), IgG4 (individual cells+).Hormone therapy significantly improved symptoms.We collected data of 189 cases of HCP reported in the last 5 years. Headache is the most presented initial symptom. Dural hypertrophy and enhancement in different parts of the head was observed in all patients by plain and enhanced cranial MRI. After hormone treatment, combined hormone and immunosuppressive treatment, or surgical treatment, more than 90% of the patients'symptoms were improved. Conclusion: The clinical symptoms of HCP can be characterized by chronic headache, cranial nerve involvement, and ataxia. It is imperative to perform hematological examination, plain and enhanced cranial magnetic resonance imaging,and,when necessary,dura mater biopsy to make a diagnosis.

目的:探讨颅内静脉窦血栓(CVST)形成合并脑出血患者的临床特点及影像学特征.方法:选取确诊为CVST合并脑出血的患者9例,收集其临床资料,并进行回顾性分析.结果:9例患者中病因为产褥期4例,服用避孕药物1例,外伤后1例,面部痤疮挤压后1例,其他既往体健.实验室检查:凝血7项示D-二聚体水平升高者3例;血常规示白细胞增多者5例,后复查降至正常.予低分子肝素治疗8例,仅予对症支持治疗者1例;1例住院期间继发脑疝死亡,另8例好转出院.结论:临床上对存在可疑病因,伴有头痛、视乳头水肿等高颅压症状,应考虑CVST可能,需及时完善实验室及影像相关检查,一旦确诊,及时予抗凝等治疗.

Objective To explore the possible relationship between uric acid and benign paroxysmal positional vertigo . Methods Total of 110 patients with benign paroxysmal positional vertigo from the Department of Neurology ,Affiliated Hospital of Xuzhou Medical College ,were recruited .A cohort of unrelated 60 healthy controls was recruited .Venous blood was collected from peripheral vein of each participant .Then biochemical analyses ,homocysteine ,blood pressure ,were analyzed between two groups .All risk factors of heart and cerebral vascular diseases ,such as smoking ,drinking ,history of high blood pressure ,diabetes were analyzed .Results Difference were statistically significant in the distributions of cholesterol and serum uric acid between BP-PV group and control group (P<0.05) .There was no statistical difference in the distribution of age ,sex ,smoking ,drinking ,fast-ing blood glucose ,blood pressure ,low density lipoprotein cholesterol ,homocysteine ,history of high blood pressure ,diabetes between BPPV group and control group (P>0.05) .Logistic regression analysis found that low level of low density lipoprotein cholesterol ,low level of homocysteine was a risk factor for BPPV ,with the odds rato of 2.329 (1.243~4.364) ,1.084 (1.005~1.169) .However ,the level of uric acid was not the risk factor for the occurrence of BPPV with the odds ratio of 0.995 (0.990~1.000) .Conclusion Based on this study ,the level of serum uric acid with BPPV was significantly higher than that of control group ,but the serum uric acid is not an independent risk factor for BPPV .Further studies are needed to clarify the risk factors of BPPV .

目的:探讨用银杏二萜内酯葡胺注射液联合注射用尤瑞克林治疗大脑中动脉狭窄脑梗死的临床效果.方法:将2014年至2016年徐州医科大学附属医院神经内科收治的90例大脑中动脉狭窄急性脑梗死患者随机分为对照组与观察组,每组各45例患者.对对照组患者进行常规治疗,在此基础上为观察组患者在入院后使用银杏二萜内酯葡胺与尤瑞克林进行治疗.在对两组患者进行治疗前和治疗后第14天分别采用NIHSS评分量表评估其神经功能缺损(NIHSS)评分,分析其临床疗效,测定其脑血流灌注量(CBF),并观察其发生不良反应的情况.结果:与对照组患者相比,观察组患者治疗的总有效率较高,差异有统计学意义(P<0.05).与对照组患者相比,治疗后的第14天观察组患者NIHSS的评分较低,其脑梗死缺血区的CBF较高,差异有统计学意义(P<0.05).两组患者在进行治疗期间均未发生出血性疾病等明显的不良反应,在进行治疗后其凝血功能指标(APTT值)均正常.结论:联用银杏二萜内酯葡胺注射液与注射用尤瑞克林治疗大脑中动脉狭窄急性脑梗死可取得理想的临床效果,能显著改善患者的神经功能及脑血流灌注量,而且用药的安全性较高.

目的 通过研究帕金森病(PD)非运动症状(NMS)的发生率,探讨其与PD的病程、病情的关系及其对生活质量的影响.方法 对116例PD患者(PD组)和60例健康人(正常对照组)进行NMS问卷调查,比较PD组和正常对照组NMS的发生率,并对PD患者进行Hoehn-Yahr(H-Y)分期、统一帕金森病评定量表(UPDRS)Ⅲ评分、MoCA评分,分析其与NMS的相关性.结果 PD组伴NMS者98例(84.48％);PD组NMS总数平均(12.16 ±4.37)个,明显高于正常对照组[(5.25±3.06)个](P＜0.05).PD组NMS发生率最高的为便秘(72.4％),其次为兴趣减退(69.8％)、近事记忆困难(66.4％)、情绪低落(62.1％)、不宁腿(61.2％).PD组NMS总数与UPDRSⅢ评分(r=0.459,P＜0.01)、H-Y分期(r=0.376,P＜0.01)呈正相关,与MoCA评分呈负相关(r=-0.584,P＜0.01).结论 PD患者的NMS发生率高,影响其生活质量,需要重新认识,提高对NMS的判断力,尽早发现和干预.

Objective: To evaluate the effect of original intracerebral hemorrhage score(oICH) on predicting the prognosis of patients with arteriovenous malformation–associated intracerebral hemorrhage(AVM-ICH). Methods: Seventysix patients with AVM-ICH were selected from our college during the period 2005-2012. Clinical data on demographics and radiographics were obtained through retrospective chart review. Clinical outcome at 3 months was assessed by the modified Rankin Scale(mRS). Maximum Youden Index was used to identify cutoffs for age and ICH volume that are associated with optimal predictive accuracy for an unfavorable outcome(mRS ≧ 3). Arteriovenous malformation–associated original intracerebral hemorrhage score(AVM-oICH) based on oICH has the cutoff points of new age and ICH volume. The predictive ability of both oICH and AVM-oICH was evaluated by analysis of receiver operating characteristic(ROC). Results: ① The mean age for patients was(31±18)yeas, and mean ICH volume was(24±10) ml. When follow-up was carried out for 3 months,2 patients(2.5%) were dead, 14 patients(18%) had a poor outcome. ② ICH volume of 35 ml and age of 37 years were identifiedas optimal cutoffs for predicting poor outcome. AVM-oICH and oICH showed good predictive accuracy with area under the curve(AUC) of 0.922 and 0.885(P=0.153), and similarly high sensitivity(P=1.00), but the former had a higher specificity(P<0.05). Conclusion: oICH has a high accuracy in predicting the functional outcome of AVM-ICH. Simple adjustments of the cutoff points of age and ICH volume can improve its performance and reduce the probability of overestimating the risk of poor outcome after AVM-ICH.

目的:研究N-甲基-D-天冬氨酸受体(NMDAR)亚单位2B(NR2B)特异性拮抗剂(Ro 25-6981)对缺氧缺血性脑损伤(HIBD)新生大鼠脑室下区(SVZ)神经干细胞(NSCs)增殖的影响.方法:7d龄新生SD大鼠随机分为Ro 25-6981组(HIBD前2h,腹腔注射Ro 25-6981 10 mg·kg-1)、HIBD组(HIBD前2h,腹腔注射等剂量生理盐水)和假手术组(仅游离右侧颈总动脉,不结扎).采用免疫组化学染色检测SVZ Nestin表达量及BrdU阳性细胞数的变化.结果:HIBD组12h后Nestin表达量开始增多,48 h达峰值,之后缓慢下降；与其相比,Ro 25-6981组在12h和24 h时下降明显(P＜0.05).HIBD组BrdU阳性细胞数在缺氧缺血3h后缓慢上升,72 h达高峰；与其相比,Ro 25-6981组在各时间点BrdU阳性细胞表达均有所下降,以24、48和72 h减少明显,尤以72 h为著(P＜0.05).结论:Ro 25-6981能够降低HIBD新生大鼠SVZ Nestin的表达及Brdu阳性细胞数,对SVZ NSCs增殖起抑制作用,提示NR2B参与并促进HIBD引起的SVZ NSCs的增殖.