寡克隆区带及抗水通道蛋白4抗体在多发性硬化诊断中的意义

目的通过观察多发性硬化(multiple sclerosis,MS)患者寡克隆区带(oligoclonal bands,OCB)及抗水通道蛋白4(aquaporin-4,AQP4)抗体的阳性率,为临床MS的诊断及鉴别诊断提供一定的参考。方法入组30例MS、48例神经系统非炎性病变(neurological non-inflammatory disease,NND)及50例外科手术患者。分析3组患者OCB、脑脊液及血清抗AQP4抗体阳性率的差异。结果 NND组与外科手术组OCB、脑脊液及血清抗AQP4抗体阳性率的差异无统计学意义(P>0.05)。MS组与对照组(NND组+外科手术组)脑脊液抗AQP4抗体阳性率差异无统计学意义(P>0.05),MS组与对照组OCB、血清抗AQP4抗体阳性率差异有统计学意义(P<0.01)。结论 OCB阳性对MS具有诊断意义。MS部分患者可表现为血清或脑脊液抗AQP4抗体阳性,诊断时需结合病史、临床体征及影像学特点等综合分析。     

OCB、抗MBP抗体及抗MOG抗体在中枢神经系统炎性脱髓鞘疾病诊断中的意义

目的：通过观察中枢神经系统炎性脱髓鞘疾病患者寡克隆区带（OCB）、抗髓鞘碱性蛋白（MBP）抗体及抗髓鞘少突胶质细胞糖蛋白（M OG ）抗体的阳性率,为临床诊断提供一定的参考。方法入组91例中枢神经系统炎性脱髓鞘疾病患者为观察组（其中MS 30例,NMO 61例）及50例神经系统非炎性病变患者为对照组。首先分析观察组与对照组间OCB、脑脊液及血清中抗MBP抗体、抗MOG抗体阳性率的差异,再分析观察组中MS亚组及NMO亚组间上述指标阳性率的差异。结果2组比较,OCB、血清抗MBP抗体及血清抗MOG抗体阳性率比较差异有统计学意义（P＜0.05）,脑脊液抗MBP抗体及脑脊液抗MOG抗体阳性率比较差异无统计学意义（P＞0.05）。MS亚组与NMO亚组,OCB、血清抗MBP抗体阳性率比较差异有统计学意义（P＜0.05）,脑脊液抗MBP抗体、血清抗MOG抗体及脑脊液抗MOG抗体阳性率比较差异无统计学意义（P＞0.05）。结论 OCB阳性对诊断MS具有重要意义。血清抗MBP抗体及抗MOG抗体可为中枢神经系统炎性脱髓鞘疾病的诊断提供重要实验室依据,血清抗MBP抗体在MS与NMO的鉴别诊断中具有一定价值。     

10例多发性硬化伴癫痫发作与MRI相关性分析

目的:探讨多发性硬化(MS)伴癫癎发作患者的临床特点与MRI所示病灶的相关性。方法:回顾性分析121例确诊为MS住院患者中10例(8.29%)伴癫癎发作的临床特点及MRI表现。结果:癫癎在MS其他症状或体征之前出现2例;癫癎发作为MS复发时唯一症状的1例;癫癎发作时已伴MS其他症状或体征者7例。10例患者头颅MRI均示双侧半球的深部白质、侧脑室旁数个斑块病灶,其中5例伴皮质-皮质下斑块病灶,2例伴局灶性皮质萎缩。5例癫发作与皮质-皮质下斑块病灶有相关性。结论:癫癎可以是MS的首发症状或复发时唯一临床表现,MS患者癫癎发作与皮质-皮质下斑块病灶相关。     

多发性硬化脑内磁共振病灶分布及其特征(附203例2年MRI随访结果)

目的　为了解多发性硬化 ( multiple sclerosis,MS)的病灶特点 ,对确诊的 2 0 3例 MS患者进行系列磁共振 ( magnetic resonance,image,MRI)观察。方法　选择确诊的缓解 -复发型 MS患者 2 0 3例 ,每 6个月检查头颅MRI一次。结果　 ( 1 ) 2 0 3例 MS患者中 ,1 72 ( 84 .7% )例脑 MRI显示脱髓鞘病灶。其中有胼胝体病灶者 90例( 5 2 % ) ,有脑干病灶者 1 0 6例 ( 6 2 % ) ,有小脑病灶者 4 6例 ( 2 8% ) ,有与脑室连接的病灶者 1 5 7例 ( 91 % ) ,有典型的卵圆形病灶者 98例 ( 5 7% ) ,病灶长轴与侧脑室切线垂直的病灶占总病灶一半以上者 1 30例 ( 76 % )。( 2 ) 2 0 3例MS患者中 ,大脑半球内和脑内无病灶者分别为 4 1例 ( 2 0 % )和 31例 ( 1 5 % )。仅脑干有病灶者 6例 ( 3% ) ,仅小脑有病灶者 2例 ( 1 % ) ,脑干和小脑都有病灶者 2例 ( 1 % )。 ( 3) 2 0 3例共检查 82 7例次 MRI,有活动病灶者 77例( 4 1 % ) ,共有活动病灶 2 6 1个 ,分布在大脑、小脑和脑干者依次为 2 2 3、1 0和 2 8个。结论　 ( 1 )在确诊 MS患者中 ,多数 ( 85 % )脑 MRI有脱髓鞘病灶 ,与欧美资料相近。( 2 )脑内有病灶者中 ,小脑有病灶者占 2 8% ,较欧美报道为低。 ( 3) T2像发现活动病灶的机率不高 ,多次复查能提高病灶的检出率 ( 4 1     

脑脊液寡克隆区带在神经系统疾病诊断中的意义

目的 :探讨脑脊液寡克隆区带 (CSF OCB)在神经系统疾患中的临床意义 ,并对多发性硬化 (MS)患者CSF OCB阳性和阴性结果进行比较研究。方法 :收集 3 98例神经系统疾患的脑脊液和血清标本 ,其中 5 1例MS患者 ,用等电聚焦加免疫标记的方法进行OCB的检测。结果 :CSF OCB阳性率在MS、神经系统炎性疾患及神经系统非炎性疾患中分别为 3 5 3 %、3 9 6%及 4 5 %。MS患者CSF OCB阳性率与临床类型、MS活动性、病程和应用激素等免疫抑制剂治疗有关。结论 :CSF OCB的检查对于MS和神经系统炎性疾患有一定的诊断价值。MS患者CSF OCB阳性率较低与国内视神经脊髓炎所占比率较高有关 ;CSF OCB的出现受到MS活动性的影响 ;也和MS病程演变阶段性相关 ;激素等免疫抑制剂的作用可能参与其中。     

多发性硬化、临床孤立综合征患者脑脊液β-淀粉样肽水平及其意义研究

目的 探讨多发性硬化(MS)及其早期表现-临床孤立综合征(CIS)患者脑脊液β-淀粉样肽(Aβ42)表达水平及其与病程、神经功能缺失以及MRI T2高信号病灶数量的关系.方法 对33例MS患者、23例CIS患者及13例对照者进行研究,MS、CIS患者发作期进行扩展残疾状态量表(EDSS)评分及MRI检查,采用液相芯片分析技术检测各组患者脑脊液Aβ42浓度.结果 MS、CIS患者发作期脑脊液Aβ42浓度与对照组相比差异无统计学意义(P>0.05),但继发进展型MS(SPMS)患者脑脊液Aβ42浓度[(167.99±36.39)pg/mL]比复发缓解型MS(RRMS)患者[(92.74±13.64)pg/mL]高,差异有统计学意义(P=0.042).MS、CIS患者脑脊液Aβ42浓度与病程及EDSS评分无明显相关性(P>0.05).病程≥1年的MS患者脑脊液Aβ42浓度比病程<1年的患者低,EDSS评分≥4.5分的MS、CIS患者脑脊液Aβ42浓度比EDSS评分<4.5分的患者低,但差异均无统计学意义(P>0.05).MS、CIS患者脑脊液Aβ42浓度与MRI T2高信号病灶数量呈正相关关系(MS患者:r=0.507.P=0.038;CIS患者:r=0.485,P=0.049).MRI T2高信号病灶总数≥4个的MS患者脑脊液Aβ42浓度[(129.34±19.96)pg/mL]比病灶总数<4个的MS患者[(73.51±12.60)pg/mL]高,差异有统计学意义(P=0.049).结论 SPMS患者轴突损伤比RRMS患者严重;脑脊液Aβ42水平升高可能是MS病情进展的标记之一;MRI T2高信号病灶负荷可能与MS轴突损伤有关.     

髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘疾病临床与预后分析

目的探讨髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein,MOG)抗体相关获得性脱髓鞘综合征(acquired demyelinating syndromes,ADS)的临床特点。方法回顾性分析6例MOG-ADS患者的临床表现、影像学、脑脊液检查以及治疗,用EDSS量表评估预后。结果 6例患者中包括4例女性,2例男性,平均发病年龄29岁(4～64岁),3例表现为视神经炎(optic neuritis,ON)、2例表现为急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM),1例以脑干症状起病;用细胞转染法(cell-based assay,CBA)检测血清MOG抗体滴度在1:100-1:10之间,1例累及视神经眼段,3例头颅MRI累及皮质下白质、脑干,1例且累及大脑皮层、基底节/丘脑,颅内病灶体积较大、边界不清;1例累及脊髓,表现为长节段脊髓炎。所有患者给予激素治疗,均达到部分或完全缓解。平均随访7.8个月,2例复发,2例符合多发性硬化(multiple sclerosis,MS)的诊断标准。结论 MOG-ADS主要表现为ON和ADEM,MRI病灶分布广泛,对激素治疗敏感,部分患者可出现疾病的复发。     

寡克隆区带和IgG指数对多发性硬化的诊断价值

目的　探讨寡克隆区带 (OCB)和IgG指数对多发性硬化 (MS)诊断的敏感性及特异性。方法　收集 4 8例MS、6 8例神经系统炎性疾病 (NID)及 110例非炎性疾病 (NNID) 3组患者的脑脊液 (CSF)和血清标本 ,分别进行OCB的检测 (等电聚焦 )和IgG指数的计算。并对其阳性结果似然比 (PRLR)进行分析。结果　MS组与NID组比较 ,CSF中OCB阳性率和IgG指数异常率的差异均没有显著性 (均P >0 0 5 ) ;但MS组、NID组与NNID组比较 ,差异均有极显著性 (均P <0 0 0 0 1)。MS组CSF中OCB和IgG指数的敏感性分别为 39 6 %、6 0 4 % ;特异性分别为 80 3%、72 1% ;PRLR分别为 2 0、2 2。当用于判断有无IgG鞘内合成时 ,特异性分别为 97 2 %、92 7% ;PRLR分别为 13 5、7 3。结论　CSF中OCB阳性和IgG指数升高强烈提示有中枢神经系统局部IgG合成 ,对MS有一定的辅助诊断价值     

成人MOG抗体病首次发作的临床和影像学特征分析

目的 探讨成人髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein, MOG)抗体病首次发作的临床和影像学特征。方法 回顾性分析2018年1月-2022年1月在江西省人民医院和新余市人民医院神经内科收治的14例MOG抗体病患者首次发作的临床症状、影像学特点、实验室检查、预后情况。结果 纳入的14例患者中男6例，女8例，中位年龄29.5岁。首发症状以发热和头痛(5例)、癫痫(3例)、头晕(3例)多见。幕上病灶包括丘脑(7例)、皮质下白质(6例)、皮质(5例)、胼胝体(2例)、基底节(2例)。幕下多累及脑干，包括桥脑(5例)、小脑中脚(3例)、中脑(2例)、延髓(2例)。脊髓受累3例，长节段横贯性1例。脑脊液细胞数增高13例，7例脑脊液蛋白升高。血清MOG抗体滴度范围在1∶3.2～1∶512。14例患者均接受静脉糖皮质激素冲击治疗，仅1例患者出现视神经炎复发。结论 本组成人MOG抗体病女性稍多于男性，以ADEM表型最多见。幕上病灶以丘脑和皮质下白质多见，幕下以脑桥多见。急性期经静脉激素冲击治疗效果显著，临床预后大多良好。     

脑脊液寡克隆区带对多发性硬化的意义

目的探讨脑脊液寡克隆区带(oligoclonal bands,OCB)在多发性硬化(multiple sclerosis,MS)中的意义。方法收集神经系统疾病患者895例,其中MS患者151例,视神经脊髓炎(neuromyelitis optica,NMO)患者92例,其他神经系统脱髓鞘疾病(other demyelinating diseases,ODD)患者122例,其他神经系统炎性疾病(other inflammatory neurological diseases,IND)患者99例,神经系统非炎性疾病(noninflammatory neurologicaldiseases group,NIND)患者431例。检测所有患者的OCB、IgG指数及24hIgG合成率,比较各组OCB阳性、IgG指数及24hIgG合成率升高情况,并比较OCB阳性和阴性MS患者的主要临床特点。结果 OCB阳性率MS组为32.45%,NMO组为20.65%,ODD组为18.03%,IND组为16.16%,NIND组为2.09%;IgG指数升高比例MS组为29.8%,NMO组为17.39%,ODD组为16.39%,IND组为15.15%,NIND组为1.62%;24hIgG合成率升高比例MS组为30.46%,NMO组为18.48%,ODD组为18.85%,IND组为19.19%,NIND组为1.39%。OCB阳性率、IgG指数和24hIgG合成率升高比例MS组均高于其他各组(均P<0.05);NMO组与ODD组、IND组的OCB阳性率、IgG指数和24hIgG合成率升高比例无统计学差异(均P>0.05);MS组、NMO组、ODD组及IND组OCB阳性率均高于NIND组(均P=0.000)。OCB阳性MS患者的女性比例、EDSS评分、IgG指数和24hIgG合成率〔分别为女∶男2.5∶1、(4.10±1.49)、(0.81±0.31)、(4.98±3.35)〕均高于OCB阴性患者〔分别为女∶男1.17∶1、(3.47±1.39)、(0.51±0.17)、(3.37±3.20)〕(均P<0.05)。结论与其他中枢神经系统疾病相比,MS患者的OCB阳性率更高。OCB阳性MS患者的女性比例、EDSS评分、IgG指数和24hIgG合成率均高于OCB阴性患者。     

Diagnostic Difficulties and Treatment Implications

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.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

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.     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

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.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

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.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

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.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

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.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.     

Treatment Considerations in Anticonvulsant Monotherapy

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.     

Anticonvulsant Drugs and Cognitive Function: A Review of the Literature

Summary: Alterations of cognitive function are separate from disturbances of behavior seen in association with epilepsy. The nature of the cognitive disability may to a certain extent depend on the seizure type. Partial seizures, mainly derived from a temporal lobe focus, impair memory tasks, while generalized seizures seem to have more effect on attentional abilities. A number of studies, reviewed in this paper, suggest that anticonvulsant drugs further impair cognitive function. Maximal impairments are seen in patients receiving polytherapy: rationalization of polytherapy improves cognitive abilities. Studies in children and adults have allowed differentiation of the effects of various commonly used antiepileptic agents. Maximal cognitive deficits are seen with. phenytoin, while phenobarbital and sodium valproate induce moderate disturbances, and carbamazepine seems relatively free from such toxicity. Further research is needed on the interrelationship between types of seizure disorders, types of anticonvulsant medications, and cognitive function.     

Dextromethorphan: Cellular Effects Reducing Neuronal Hyperactivity

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.