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1.
神经系统副肿瘤综合征临床分析   总被引:11,自引:0,他引:11  
目的 了解神经系统副肿瘤综合征患者的临床特点.方法 收集我院收治的神经系统副肿瘤综合征患者28例,对其临床资料进行回顾性分析.结果 患者多为慢性隐袭或亚急性起病,进行性加重,治疗后无明显缓解,3.8%患者在发现肿瘤后才出现神经症状;96.2%在出现副肿瘤症状后才发现肿瘤,副肿瘤综合征的临床类型有Lambert-Eaton肌无力综合征8例、周围神经病7例、多发性肌炎和皮肌炎4例、脑干脑炎3例、进行性小脑变性2例、边缘系统脑炎2例、运动神经元病1例、进行性多灶性白质脑病1例.结论 神经系统副肿瘤综合征临床表现形式多样,容易误诊,临床早期确诊对于隐匿肿瘤的发现和治疗非常重要.  相似文献   

2.
目的 提高对副癌神经综合征的临床特点的认识 ,减少误诊误治率。方法 对 2 0例副癌神经综合征患者的临床资料进行回顾性分析。结果 本组副癌神经综合征患者均亚急性、慢性起病 ,15例以神经系统症状为首发。周围神经病 8例 ,脊髓病 2例 ,小脑变性 3例 ,肌无力综合征 5例 ,多发性肌炎 2例。原发肿瘤常见于支气管肺癌、淋巴瘤、乳腺癌和卵巢癌 ,常规检查难以早期诊断 ,治疗原发肿瘤和免疫抑制剂可改善神经症状。结论 副癌神经综合征大多先于肿瘤发病 ,影响神经系统各部位 ,临床症状复杂 ,预后较差。  相似文献   

3.
副癌神经综合征15例临床分析   总被引:4,自引:0,他引:4  
目的 提高对副癌神经综合征临床特点的认识。方法 对15例副癌神经综合征患者的临床资料进行回顾性分析。结果 本组副癌神经综合征患者均亚急性,慢性起病,13例以神经系统症状首发。周围神经病8例,小脑变性3例,Ewaton-Lambert综合征2例,运动神经元病1例,多发性肌炎1例。原发肿瘤常见于肺癌,淋巴瘤,乳腺癌和生殖器肿瘤,常规检查难以早期诊断,治疗原发肿瘤和免疫抑制剂治疗可改善神经症状。结论 副癌神经综合征大多先天肿瘤复发,影响神经系统各中位,临床症状复杂,预后较差。  相似文献   

4.
目的 探讨抗Hu抗体检测在神经系统副肿瘤综合征诊断中的临床意义.方法 采用间接免疫荧光方法和蛋白免疫印迹法对送检至北京协和医院神经病理实验室的1500余例患者的血清和脑脊液进行抗Hu抗体检测,回顾性分析抗Hu抗体阳性患者的临床资料及诊断.神经系统副肿瘤综合征的诊断标准参照Graus等的诊断标准.结果 共有27例患者抗体阳性,其中血清抗Hu抗体阳性25例,脑脊液抗Hu抗体阳性8例.临床主要表现为感觉神经元神经病、亚急性小脑变性、Lambert-Eaton综合征和抗利尿激素分泌不当综合征引起的脑病等,其中20例(74.1%)患有肿瘤,包括肺癌17例,胃癌2例,不明性质腹部肿瘤1例.7例患者未发现恶性肿瘤,包括多发性肌炎和系统性红斑狼疮(SLE)合并神经肌肉病各1例.根据Graus等神经系统副肿瘤综合征的诊断标准,27例中22例可确诊神经系统副肿瘤综合征或相关的肿瘤,抗Hu抗体的阳性预测值为81.5%.结论 抗Hu抗体阳性对神经系统副肿瘤综合征的诊断具有一定意义;其相关肿瘤以肺癌,特别是小细胞肺癌最常见.其他自身免疫性疾病偶可见抗Hu抗体阳性,需要全面检查和密切随访以排除恶性肿瘤的可能.  相似文献   

5.
单纤维肌电图在68例运动神经元病患者中的研究   总被引:15,自引:3,他引:12  
目的 研究运动神经元病 (MND)患者的单纤维肌电图 (SFEMG)改变、病理生理机制及临床意义。方法 对 6 8例 (男 4 3例 ,女 2 5例 )经病史、临床表现和神经电生理检查测定证实的MND患者进行了伸指总肌SFEMG测定 ,并与年龄匹配的正常人对照。结果 所有患者感觉神经传导速度测定均正常 ,肌电图为广泛神经源性损害。SFEMG检查发现 :颤抖值 (jitter)为 30~ 178μs,平均 (80 2± 32 6 ) μs ;jitter>5 5 μs占 5 0 %~ 10 0 0 % ,平均 6 0 6 %± 2 9 0 % ;阻滞 (block)所占百分比为 0 0 %~90 0 % ,平均 2 9 3%± 30 0 % ;纤维密度 (FD)为 1 4~ 4 0 ,平均 2 6± 0 6。其中 5 1例确诊和拟诊的肌萎缩侧索硬化患者jitter增宽、block和FD增高最明显。还发现伸指总肌肌无力的程度与jitter增宽和block百分比呈明显的负相关。结论 SFEMG的异常改变在确诊患者组最明显 ;jitter增宽、block百分比升高和FD增高与肌肉无力的严重程度呈明显的负相关 (P <0 0 0 1) ;jitter增宽、block百分比升高和FD增高反映进行性失神经、神经再生和神经肌肉接头处的传递情况  相似文献   

6.
神经导航辅助胶质瘤手术   总被引:11,自引:1,他引:10  
目的 评价神经导航系统在胶质瘤手术中的应用。方法 在 80例胶质瘤手术中 ,应用StealthStation神经导航系统指导手术操作。在其中 2 7例手术中测量硬膜、皮质以及病灶移位程度。在大脑浅表胶质瘤手术中 ,使用微导管栅栏法纠正脑移位的影响 ,判断肿瘤边界。结果  80例平均座标误差为 (2 .0 3± 0 .89)mm ,10cm预期准确性 (2 .4 3± 0 .99)mm。术中硬膜、皮质以及病灶移位分别为 (3.4 4± 2 .39)mm、(7.5 8± 3.75 )mm以及 (6 .5 5± 3.19)mm。 5例虽然导航显示肿瘤残留 ,但未强行切除 ;其余 75例肿瘤全切 6 2例 (82 .7% ) ,次全或大部切除 13例 (17.3% )。术后症状改善或不变 6 8例 (85 .0 % ) ,术后症状加重或出现新症状 12例 (15 .0 % ) ,无死亡病例。结论 在胶质瘤手术中 ,术中脑移位是影响神经导航准确性的重要因素。微导管栅栏法简便、实用 ,有助于提高肿瘤切除率 ,降低手术并发症  相似文献   

7.
目的了解神经系统副肿瘤综合征的临床特点。方法回顾性分析24例神经系统副肿瘤综合征患者的临床资料并进行总结。结果 24例神经系统副肿瘤综合征患者平均年龄68.7岁,男女比例为1∶0.7。神经系统表现为边缘叶脑炎、亚急性小脑变性、脑干脑炎、周围神经病、Lambert-Eaton肌无力综合征和恶病质肌病。肿瘤来源以肺癌为主,占79.1%。75%的病例先于肿瘤表现出PNS的症状。结论 PNS表现多样,早期识别症状对于诊治PNS、发现肿瘤非常重要。  相似文献   

8.
副肿瘤综合征   总被引:7,自引:0,他引:7  
副肿瘤综合征是恶性肿瘤的破坏性远隔效应,它可影响神经系统的任何一个部位。多数神经系统副肿瘤综合征是免疫介导的,神经系统异常通常在肿瘤之前出现,症状和体征多种多样,取决于受损的部位。诊断主要依靠临床表现和相关辅助检查。近年来副肿瘤综合征患者血清或脑脊液中特异性抗神经元抗体的发现可作为肿瘤相关疾病的诊断标志,并为癌肿早期甚至超早期诊断提供了依据。治疗主要包括两个方面:一是针对神经系统副肿瘤综合征的免疫治疗;二是针对肿瘤的手术、放疗、化疗等。副肿瘤综合征的神经病理改变及肿瘤发现是否及时是影响预后的重要因素。  相似文献   

9.
目的探讨神经系统副肿瘤综合征(PNS)临床表现及神经副肿瘤抗体的特征。方法分析2009年1月至2019年9月北京大学第一医院神经内科确诊的110例PNS患者的临床特征及神经副肿瘤抗体类型。结果110例患者出现17种临床综合征,其中43例(39.1%)为经典临床综合征,最常见的为亚急性小脑变性14例(12.7%)和边缘性脑炎11例(10.0%);62例(56.4%)为非经典临床综合征,最常见的为感觉运动周围神经病19例(17.3%)和重症肌无力10例(9.1%)。69例(62.7%)患者发现恶性肿瘤,肺癌20例(18.2%)及血液系统肿瘤12例(10.9%)为最常见的肿瘤。71例(64.5%)患者在血液中检测到特征性副肿瘤抗体,最常见的为抗Amphiphysin抗体26例(23.6%)和抗Yo抗体24例(21.8%)。结论临床表现为非经典副肿瘤综合征的患者,亦应积极进行特征性副肿瘤抗体的检测,并根据抗体结果对特定肿瘤进行重点筛查。  相似文献   

10.
多系统萎缩与帕金森病的早期鉴别诊断   总被引:4,自引:0,他引:4  
目的 为多系统萎缩 (m ultiple system atrophy,MSA)与帕金森病 (Parkinson disease,PD)的鉴别诊断提供依据。方法 对 12名首发锥体外系症状的 MSA患者和 40名 PD患者的临床症状及主要辅助检查行比较分析。MSA诊断依据 Quinn(1994)提出的临床诊断标准。PD诊断参考英国帕金森病协会 1997年提出的诊断标准。结果 与 PD患者比较 ,MSA患者发病年龄较小 (5 4.3 2± 9.5 0 vs 61.5 6± 9.5 5 ,P<0 .0 5 ) ,主要以行动困难或僵硬为首发症状 (66.7% vs 2 5 % ,P<0 .0 5 ) ;少数以震颤为首发症状 (3 3 .3 % vs 75 % ,P<0 .0 5 ) ,也可以单侧症状发病(5 8.3 % vs 80 % ,P>0 .0 5 ) ,多数对多巴胺反应不良 (66.7% vs 2 0 % ,P<0 .0 5 ) ,植物神经症状、语言障碍比 PD患者显著多见 (分别为 83 .3 % vs 2 5 % ;5 0 % vs12 .5 % ;P<0 .0 5 )。辅助检查 :MRI:大部分 MSA患者有阳性表现 ,橄榄体、脑桥和小脑部位有萎缩 ,PD患者未发现特征性病变。 PET:3例 MSA患者均呈阳性表现。神经心理 :MSA患者未出现智能障碍的情况 (0 % ) ,少数 PD(10 % )患者出现智能障碍 ,但两者比较无统计学意义 (P>0 .0 5 )。结论 依据详细的病史和全面的神经系统查体 ,并结合临床辅助检查 ,可提高 MSA和 PD早期鉴别诊断的准确性  相似文献   

11.
Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.  相似文献   

12.
Diagnostic Difficulties and Treatment Implications   总被引:1,自引:0,他引:1  
Robert J. Gumnit 《Epilepsia》1987,28(S3):S9-S13
Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.  相似文献   

13.
Cognitive Dysfunction Associated with Antiepileptic Drug Therapy   总被引:7,自引:5,他引:2  
Eileen P.G. Vining 《Epilepsia》1987,28(S2):S18-S22
Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.  相似文献   

14.
B. J. Wilder 《Epilepsia》1987,28(S2):S1-S7
Summary: The long-standing practice of polypharmacy in treating epilepsy is giving way to use of monotherapy. Monotherapy can improve seizure control as well as reduce the risk of serious idiosyncratic reactions, dose-related side effects, and complex drug interactions. Monotherapy also offers improved compliance and cost-effectiveness. The basis of monotherapy is accurate diagnosis and assessment of the patient's seizure type(s), followed by selection of a single appropriate anticonvulsant drug. Many patients currently treated with multiple anticonvulsants can be successfully converted to monotherapy with a carefully monitored program in which troublesome and redundant drugs are gradually withdrawn from the therapeutic regimen.  相似文献   

15.
Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.  相似文献   

16.
Hepatic Considerations in the Use of Antiepileptic Drugs   总被引:5,自引:4,他引:1  
Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.  相似文献   

17.
Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.  相似文献   

18.
Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.  相似文献   

19.
Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.  相似文献   

20.
Dextromethorphan: Cellular Effects Reducing Neuronal Hyperactivity   总被引:5,自引:1,他引:4  
G. Trube  R. Netzer 《Epilepsia》1994,35(S5):S62-S67
Summary: Dextromethorphan is a dextrorotary morphinan without affinity for opioid receptors, commonly used as an antitussive medication. During the past 5 years, interest in the compound and its demethylated derivative, dextrorphan, has been revived because additional neuroprotective and an-tiepileptic properties were found in in vitro studies, animal experiments, and a few clinical cases. Both morphinans are able to inhibit N -methyl-D-aspartate (NMDA) receptor channels and voltage-operated calcium and sodium channels with different potencies. The inhibition of the NMDA receptor is believed to be the predominant mechanism of action responsible for the anticonvulsant and neuroprotective properties of the compounds.  相似文献   

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