波幅下降比率(65.9±32.6)%;23例晚发AchR抗体阳性全身型MG患者中,被检肌肉RNS均为阳性

正a:卡方检验; b:单因素方差分析; c:秩和检验     

22例重症肌无力合并胸腺瘤的手术治疗体会

目的 探讨重症肌无力(myasthenia gravis,MG)合并胸腺瘤患者的围手术期处理方法及手术治疗效果.方法 对本院2006-03～2008-03接受手术治疗的22例重症肌无力合并胸腺瘤患者的临床资料进行回顾性分析.采用改良Osserman标准分为:Ⅰ型9例,Ⅱa型5例,Ⅱb型7例,Ⅲ型1例,手术切口采用胸骨正中切口.手术均行胸腺瘤、胸腺脂肪组织切除及纵隔脂肪组织清扫.结果 22例无手术死亡,3例术后早期发生MG危象,经气管切开、辅助呼吸等抢救治疗治愈.结论 完善围手术期处理措施,减少MG危象的发生,手术治疗重症肌无力合并胸腺瘤可获得良好的疗效.     

应用ELISA法检测重症肌无力患者血清中胸腺瘤相关抗体

本文应用ELISA法检测123例重症肌无力(MG)患者血清中骨骼肌柠檬酸提取物抗体(CAE-Ab)。观察到MG合并胸腺瘤的阳性率为82.6％(19/23),略高于间接血凝法(IHA),而MG无胸腺瘤的检出率为4.0％(3/77)。结果表明CAE-Ab与MG合并胸腺瘤有显著相关性。ELISA法免去醛化、鞣化及抗原致敏绵羊红细胞等繁杂步骤,方法简便,更容易推广使用,可协助临床发现胸腺瘤。     

重症肌无力合并胸腺瘤患者手术疗效（附26例）

目的 :研究重症肌无力 (MG)合并胸腺瘤患者胸腺切除术的疗效。方法 :按Masaoka分期将 2 6例MG合并胸腺瘤者分为Ⅰ期 6例 ,Ⅱ期 13例 ,Ⅲ期 6例 ,Ⅳ期 1例。全部行胸腺切除术。结果 :术后 4例发生危象。随访 2年 ,19例 ( 73 1% )的患者术后病情明显改善 ;7例 ( 2 6 9% )没有变化或者疗效差。结论 :MG合并胸腺瘤的患者应早期适时进行胸腺切除手术。     

65例胸腺瘤伴重症肌无力患者手术治疗及其预后因素分析

目的:观察恶性胸腺瘤伴重症肌无力患者的临床疗效及预后影响因素。方法:对65例恶性胸腺瘤伴重症肌无力患者进行手术治疗并辅以放疗,并按不同术式、Masaoka外科病理分期、L/B病理分型比较。结果:接受根治性手术,Masaoka外科病理分期较早的患者5、10年生存率较高(P<0.05),且前者肌无力症状改善情况亦较好(P<0.05)。不同病理(L/B)类型患者间5、10年生存率及肌无力症状改善无明显差别。结论:恶性胸腺瘤伴重症肌无力患者早期手术并辅以放疗可改善肌无力症状提高生存率;Masaoka外科病理分期和预后密切相关;L/B病理分型和重症肌无力症状的改善及预后无明显相关性。     

中颅窝底中间型纤维组织细胞瘤一例

患者男,25岁.因左耳听力下降1年余,发现左颞部肿块7个月入院.1年多前病人无明显诱因出现进行性左耳听力下降,于7个月前发现左颞部质硬无痛肿块,肿块逐渐增大.查体:左颞部皮下可扪及一3 cm×4 cm大小质硬,界清,不活动,与头皮无粘连凸起肿块,左耳听力下降.CT及MRI检查示:左中颅窝占位性病变.病变向颅内、颅外侵犯,左侧颅中窝骨质破坏.     

重症肌无力(Myasthenia gravis,MG)是一种自身抗体介导的神经-肌肉接头疾病,按其肌无力分布范围可以分为全身型和眼肌型,而后者多数在疾病发展过程中,最终进展为全身型,故全身型MG在整

正个MG群体中占据了较多的比率。同时,全身型MG在临床表现和治疗反应上异质性较高,这对全身型MG的临床诊疗带来了较大的困扰。近年来,根据其临床特征及抗体结果,国际上将全身型MG进一步分为乙酰胆碱受体(Acetylcholine receptor,AchR)抗体阳性早发型(起病年龄小于50岁)、AchR抗体阳性晚发型(起病年龄大于50岁)、肌肉特异性受体酪氨酸激酶(Muscle-specific receptor tyrosine kinase,MuSK)抗体阳性型、低密度脂蛋白受体相关蛋白4(Low-density lipoprotein receptor-related protein 4,LRP4)抗体阳性型、胸腺瘤型和抗体阴性     

KCND3突变致婴儿脊髓小脑性共济失调19/22型1例报告

<正>脊髓小脑性共济失调(SCA)19/22型是一种KCND3突变相关的遗传性神经退行性疾病,临床罕见。现报告1例婴儿起病的SCA 19/22型如下。1病例患者,女,1岁4个月。因"活动能力差4个月"于2018年4月18日就诊于北京某医院。家长于4个月前逐渐发现患儿活动能力差,表现为不能独立坐稳、爬行,不能独自站立,贴墙短暂站立后即需坐卧,不能用勺子将饭送进口中。言语少,能清晰喊"爸爸、妈妈"等简单词汇,能大声哭、笑,有时对家长呼喊     

电压门控钾离子通道复合物抗体相关临床综合征合并伴胸腺瘤的重症肌无力患者2例及文献复习

目的探讨电压门控钾离子通道复合物(VGKCc)抗体相关临床综合征合并伴胸腺瘤的重症肌无力(MG)患者的临床特点及转归。方法回顾分析山东大学齐鲁医院(青岛)分别于2020年9月和12月收治的2例确诊为VGKCc抗体相关临床综合征合并伴胸腺瘤的MG患者, 总结其临床及辅助检查、随访预后等资料, 并结合相关文献进行复习总结。结果例1为64岁女性, 临床表现为认知障碍、精神异常和癫痫样发作, 血清富亮氨酸胶质瘤失活蛋白1(LGI1)抗体阳性, 明确诊断为抗LGI1脑炎, 既往存在球部起病的MG, 胸部CT提示胸腺瘤, 入院后给予免疫治疗后症状改善。例2为67岁男性, MG诊断明确, 后期出现认知功能下降、肌颤搐、自主神经症状, 神经电生理可见F波后放电及肌颤搐电位, 血清接触蛋白相关蛋白2抗体阳性, 明确诊断为莫旺综合征合并伴胸腺瘤的MG, 入院后给予免疫治疗及胸腺瘤切除等治疗, 症状改善。结论 VGKCc抗体相关临床综合征合并胸腺瘤的MG患者同时存在两类疾病各自的临床特点, 同时又有交叉。免疫治疗及针对胸腺瘤的治疗通常能取得较好的疗效。     

内侧型蝶骨嵴脑膜瘤的显微手术治疗(附22例临床分析)

目的探讨内侧型蝶骨嵴脑膜瘤的显微手术方法,提高手术疗效。方法回顾分析显微手术治疗22例内侧型蝶骨嵴脑膜瘤病人的临床资料。其中18例采用翼点入路,3例采用扩大翼点入路,1例采用额颞颧弓入路。结果显微镜下肿瘤全切除14例(63.64%),大部切除5例(22.73%),部分切除3例(13.64%).术后死亡1例。结论娴熟的显微操作技术、选择正确的手术入路以及术中神经和血管保护是手术成功的关键。     

大型听神经瘤术中面神经的保护(附133例报告)

目的 探讨大型听神经瘤术中面神经保护技术、方法.方法 133例听神经瘤全部采用枕下乙状窦后入路,在显微外科基础上采用神经电生理监测技术,配合超声吸引和激光刀切除肿瘤,术后采用House-Brackmann(HB)分级方法对面神经功能进行评价.结果 肿瘤全切除126例(95%),次全切除7例(5%).面神经解剖保留122例(92%),无死亡病例.术后3个月回访108例,按House-Brackmann分级,面神经I～Ⅱ级65例(60.2%),Ⅲ～Ⅳ级36例(33.3%),Ⅴ～Ⅵ级7例(6.5%).结论 熟练的显微外科技术、神经电生理监测的应用及面神经保护的术中技巧是面神经保护的关键.     

Sex-Dependent Behavioral Functions of the Purkinje Cell-Specific Gαi/o Binding Protein, Pcp2(L7)

We previously reported motor and non-motor enhancements in a mouse mutant with an inactivated Purkinje cell-specific gene, Pcp2(L7), that encodes a GoLoco domain-containing modulator of Gi/o protein-coupled receptors. Effects included elevated learning asymptote with repeated rotarod training, increased acquisition rate in tone-cued fear conditioning (FC), and subtle male-specific changes in both acoustic startle habituation and pre-pulse inhibition. We have further analyzed this mutant strain this time with a focus on male?Cfemale differences, and here we report a sex-dependent anxiety-like phenotype: male mutants are less anxious, and female mutants are more anxious, than same-sex wild types. Similarly, the fear responses measured during the tone in FC acquisition are decreased in male mutants and increased in female mutants relative to same-sex wild types. Overall, the dynamics of both acquisition and extinction of FC is affected in mutants but memory was not affected. In the social realm, compositional analysis of sociability and preference for social novelty data supports that both L7 genotype and sex contribute to these behaviors. These results provide direct evidence of emotional functions of the cerebellum due to the unambiguous cerebellar specificity of Pcp2(L7) expression and the lack of any confounding motor defects in the mutant. We attempt to synthesize these new data with what is previously known both about Pcp2(L7) and about the effects of sex and sex hormones on anxiety and fear behaviors: specifically, L7 is a bidirectional and sex-dependent damper that regulates the amplitude and/or rate of sensorimotor responses, potentially acting as a mood stabilizer.     

常染色体46,XY,t(7;12)(q33q21)伴精神障碍一例报告

作者在1990年发现一例男性染色体异常:46,XY,t(7;12)(q33q21)伴有精神症状。现报道如下: 李××,男23岁,昆明籍,未婚。中专文化,工人。一年前无明显诱因性格改变,好发脾气,发呆,反应慢,不理人,上班时注意力不集中,夜眠差,工作不能胜任。于1988年5月11日首次来我院门诊。患者同胞二人,居幼。母孕期患支气管哮喘。足月顺产,幼年生长发育正常。7岁半入学,成绩中上,1987     

低能X线术中放疗在颅内室管膜瘤中的应用(附5例报告)

正室管膜瘤起源于脑室和脊髓中央管的室管膜细胞,仅约占颅内胶质瘤的5%~6%~([1])。室管膜瘤对放疗敏感。目前有效的治疗策略为手术切除及术后放疗~([2-3])。术中放疗技术(intra-operative radiotherapy,IORT)可以给予瘤床直接照射而周围正常组织接受剂量小,抑制肿瘤在等待外放疗期间生长,是外放疗的很好补充治疗方案。新型可移动术中放疗设备术中光子治疗仪(INTRABEAM)使用的射线为低能X线,可应用于普通手术室。目前INTRABEAM在神经外科室管膜瘤领域     

L’impulsivité dans le TDAH : prévalence des troubles du contrôle des impulsions et autres comorbidités,chez 81 adultes présentant un trouble déficit de l’attention/hyperactivité (TDA/H)

Attention deficit hyperactivity disorder (ADH/D) is a neuropsychological developmental disorder characterized by pervasive and impairing symptoms of inattention, hyperactivity, and impulsivity. Whereas it is well known in children, there is still little information about ADH/D in adults, including prevalence. Indeed, there are actually no epidemiological studies in France, despite the considerable impact of this disorder in a patient's professional and affective life. Moreover, ADH/D rarely stays isolated, and many comorbidities often complicate the diagnostic investigation. It is well known that the so-called ADH/D is composed of two main categories of symptoms (Attentional Disorder/Hyperactiviy Disorder), but Impulsiveness also remains a major symptom.ObjectiveThe aim of this study was to evaluate not only the prevalence of Impulse Control Disorders (ICD) but also psychological and addictive comorbidities among adult patients with ADH/D. A total of 100 patients from specialized consultations of adult ADH/D were evaluated in this study, but only 81 were included after presenting all the clinical criteria of ADH/D.MethodWe used the DSM IV-T-R for ADH/D, the Minnesota Impulsive Disorders Interview a semi-structured clinical interview assessing impulse control disorders (ICD) (compulsive buying, trichotillomania, compulsive sexual behaviour, kleptomania, pyromania and intermittent explosive disorder), and the Mini International Neuropsychiatric Interview in order to evaluate psychiatric and addictive comorbidities.ResultsMore than 90 % of the patients met the early apparition criteria of ADH/D (before 7 years). More than half of the patients presented a mixed type of ADH/D (both inattentive and hyperactive-impulsive forms): 55.6 % vs 44.4 % for the inattentive type. The vast majority of patients showed a complete form (with a total of 6 or more symptoms out of 9, of inattentive and/or impulsive-hyperactivity category): 93.8 % and only 6.2 % presented a sub-syndromic form of ADH/D (with 3 symptoms at least of one and/or the other category). Regarding the ICDs, we found a proportion of 66 % of patients manifesting at least one, the most frequent ICD being the Intermittent Explosive Disorder (IED): 29.6 %, followed by Compulsive Buying (CB): 23.4 %, Pathological Gambling (PG): 7.4 %, Kleptomania and Compulsive Sexual Behaviour: 2.4 %, and Trichotillomania: 1.2 %. Among the psychiatric comorbidities evaluated, generalized anxiety disorder: 61.7 %, followed by dysthymia: 44.4 %, major depressive episode: 28.3 %, Agoraphobia: 22.2 %, panic disorder: 17.2 %, hypomanic episode: 16 %, social phobia: 11.1 %, bulimia nervosa: 8.6 %, and antisocial personality disorder and obsessive-compulsive disorder: 3.7 %. Regarding the addictive comorbidities, we found a prevalence of 14.8 % of substance abuse (non-alcohol), followed by 7.4 % of alcohol abuse, 6.1 % of substance dependence (non-alcohol), and 3.7 % of alcohol dependence.ConclusionADH/D in adults continues to be unrecognized in France. The aim of this study was to evaluate the prevalence of impulse control disorders, psychiatric and addictive comorbidities in adults with ADH/D. The results enable us to appreciate quantitative and qualitative data for 81 French adults with ADH/D. This disorder rarely remains isolated and is often associated with many others, especially anxiety and mood disorders. We also observed that impulsivity stays at the heart of the ADH/D, either through impulsive behaviours or addictive disorders. Considering the lack of studies with ADH/D adults, it is difficult to compare our data. The diagnosis of ADH/D is complex and stays controversial, moreover the strong prevalence of comorbidities points out the importance of differential diagnosis.     

Solitaire AB 型支架取栓治疗急性大脑中动脉闭塞(20例报告)

目的探讨Solitaire AB型支架(ev3 Inc,Plymouth,MN)用于急性大脑中动脉闭塞动脉取栓术的效果及安全性。方法回顾我院采用Solitaire AB型支架行急性大脑中动脉闭塞动脉取栓术治疗20例急性大脑中动脉闭塞资料,其中单纯大脑中动脉闭塞14例,合并颈内动脉颅内段闭塞4例,合并基底动脉闭塞1例,合并大脑前动脉闭塞1例。分析即时取栓效果,术后出血情况,对比患者术前及出院时NIHSS评分以及随访90d时mRS情况。结果 18例患者成功获得再通,3例患者在取栓后M1段存在明显狭窄,给予支架成形纠正狭窄满意(残余狭窄率<20%)。3例患者出现颅内出血,1例为外囊出血,约12 mL,2例为蛛网膜下腔少量出血(均为合并颈内动脉颅内段闭塞)。死亡2例,均为合并颈内动脉颅内段闭塞。入院NIHSS评分(10.83±5.95)与出院时NIHSS评分(7.38±5.53)比较,具有统计学差异(P﹤0.01)。90d随访临床结局优良患者13例(mRS<2)。结论使用Solitaire AB型支架进行动脉取栓能获得较好的再通率,对于超过静脉或动脉溶栓传统安全时间窗的患者,根据患者DWI及PWI匹配情况确定是否存在半暗带,取栓时机可扩大到8 h以外。     

偏头痛患者右向左分流发生率及分 流量的研究——一项基于217例中 国偏头痛患者的研究

【摘要】
背景 近年来,对比增强多普勒超声（contrast-enhanced transcranial Doppler,cTCD）的研究发现,右
向左分流（right-to-left shunt,RLS）可能是偏头痛的危险因素,但是这些数据均来自西方国家,尚无
中国人群的数据可用。
目的 评估中国偏头痛人群中RLS的发生率,并分析RLS分流量与偏头痛的关系。
方法 本研究共纳入217例确诊为偏头痛的患者及100例健康志愿者,均给予cTCD评估RLS发生情况
及分流量大小。
结果 在偏头痛组,RLS发生率为44.2%（96/217）,其中大量分流率为23.5%（51/217）;在健康组,
RLS发生率为28.0%（28/100）,其中5.0%（5/100）为大量分流。在先兆偏头痛组,RLS发生率为66.1%
（39/59）,大量分流占37.3%（22/59）;在无先兆偏头痛组,RLS发生率为36.1%（57/158）,大量分流
率为18.4%（29/158）。偏头痛组RLS阳性率和大量分流率高于健康组（P <0.05）。先兆偏头痛组RLS
阳性率和大量分流率高于无先兆偏头痛组（P <0.05）和健康组（P <0.05）。尽管无先兆偏头痛组与健
康组RLS阳性率相似,但是大量分流率在无先兆偏头痛组高于健康组（P <0.05）。
结论 RLS与偏头痛的发生有密切关系,尤以先兆偏头痛为著。     

聚合酶链法检测莱姆病患者尿液中伯氏疏螺旋体DNA(附17例报道)

目的:评估聚合酶链反应(PCR)检测莱姆病患者尿液中伯氏疏螺旋体DNA的诊断价值。方法:2004至2006年来自新疆莱姆病自然疫源地临床疑似莱姆病患者17例(均有蜱暴露史和莱姆病临床证据者)和6例以前确诊并治疗后完全恢复的莱姆病患者,共23例为病例组;另选择25例非疑似莱姆病患者为对照组。应用PCR方法检测尿液中伯氏疏螺旋体DNA,采用间接免疫荧光(IFA)检测血清伯氏疏螺旋体抗体。结果:①17例莱姆病患者临床表现呈多系统损害。12/17例(70.59%)为神经型(周围神经病3例,其中1例合并下肢瘀积性皮炎;游走性红斑并发莱姆脑病2例;脑膜脑炎2例;脑炎2例;脑膜炎、脑干炎和脊髓炎各1例);流感样症状2例,其中伴皮疹1例;余心脏型、精神障碍和肝病型各1例。②PCR伯氏疏螺旋体DNA检测阳性17/17例(总阳性率100%)。4例血清IFA试验伯氏疏螺旋体抗体阳性。以前确诊并治疗者6例及对照组25例尿PCR伯氏疏螺旋体DNA和血清IFA试验伯氏疏螺旋体抗体均阴性。③10例完成抗生素治疗后3个月复查,9例PCR伯氏疏螺旋体DNA检测阴性(包括3例晚期莱姆病患者),1例2年复查时仍阳性;4例血清伯氏疏螺旋体抗体阳性者复查均阴性。④早期莱姆病多采用几周的抗生素治疗方案。结论:PCR检测尿液中伯氏疏螺旋体DNA是莱姆病一个有价值的诊断工具。     

后良好组与不良组中患者年龄、血Hcy无明显差异,差异无统计学意义(P>0.05);预后不良组发病时NIHSS评分高与预后良好组,差异有统计学意义(P<0.001)(见表1)。CAD患者预后良好组侧支

正3讨论CAD的发生率低,是青年卒中的主要原因之一,ICAD发生率约为(2. 5～3. 0)/10万人年,VAD发生率(1. 0～1. 5)/10万人年[5],本研究48例CAD患者年龄相对较轻,其中ICAD患者31例,VAD患者17例,前者是后者的1. 82倍,与文献报道相符合。患者首发症状多种多样,以一侧肢体麻木无力起病最为多见(70. 83%),可合并有头晕、言语不清、头颈部疼痛等;有的以TIA起病,逐步进展或突发加重;单纯头颈部疼痛起病或有头颈部外伤史及可疑头颈部外伤史者甚少;约95%以上患者最终确诊为