神经梅毒的临床特征与诊断分析

目的：分析神经梅毒的分型和临床特征及提供早期诊断依据。方法：回顾性分析经临床和实验室检查确诊的18例神经梅毒病人的有关临床资料。结果：神经梅毒的临床特征包括：(1)急性、亚急性起病为主；(2)临床以间质型，尤其是以脑卒中起病常见，症状元特异性；(3)血清学检查以梅毒螺旋体血凝试验(TPHA)及快速血浆反应素试验(RPR)特异性较高；(4)脑脊液检查表现为压力增高(42．9％)、蛋白增高(81．2％)及细胞数增高(56．3％)；(5)头颅CT、MRI表现与高血压、动脉硬化所致脑梗死不同，为多发、散在病灶。结论：神经梅毒早期误诊率高，临床表现与分型密切相关，实验室及影像学检查是诊断的重要依据。     

神经梅毒12例临床特征及影像学分析

目的探讨神经梅毒的临床特征及影像学表现。方法回顾性分析2000年1月~2008年4月我院收治的12例神经梅毒患者的临床资料。结果12例神经梅毒患者中脑卒中样起病6例,麻痹性痴呆2例,脊髓损害2例,脑膜炎及神经炎各1例;血清梅毒甲苯胺红不加热血清反应素试验及梅毒螺旋体明胶颗粒凝集试验均呈阳性反应;部分患者脑脊液检查示蛋白质含量升高、白细胞计数升高(以单个核细胞为主)。MR检查分别表现为脑梗死灶、脑萎缩、脑皮质异常信号、脑动脉狭窄或闭塞及脊髓空洞症等。结论梅毒螺旋体对神经系统的损害比较弥散,神经梅毒的诊断应结合临床表现、实验室及神经影像学检查资料综合分析,早期诊断、早期治疗对本病预后有重要意义。     

线粒体脑肌病的临床、病理与神经电生理

目的探讨线粒体脑肌病的临床、肌肉病理及神经电生理特点，以便早期诊断。方法对6例确诊的线粒体脑肌病患者的临床表现、肌肉组织光镜和超微结构改变以及神经电生理改变进行了回顾性分析。结果本组患者的临床特征主要以运动不耐受，阵挛、抽搐发作，精神障碍，共济失调为主。6例患者中4例发现破碎红纤维（RRF），其平均比例为5．3％；超微结构观察有线粒体异常及糖原颗粒沉积，其中有2例发现有典型晶格状包涵体。以癫痫发作为主要临床表现的患者脑电图明显异常；肌电图以神经源性改变4例，占本组病例的4／6；听觉诱发电位（BAEP）、体感诱发电位（SEP）异常3例，占3／6。结论线粒体脑肌病的临床表现复杂多样，诊断主要依赖于临床特征分析和肌肉活检；电镜超微结构改变为线粒体病的主要诊断依据；神经电生理改变对病理损伤累及范围和程度方面有一定的参考价值。     

不同时期神经梅毒患者临床特征及血清学和脑脊液检测分析

目的总结不同时期神经梅毒患者临床特征以及梅毒血清学和脑脊液检测特点。方法回顾分析12例不同时期神经梅毒患者[早期神经梅毒5例,包括4例脑(脊)膜梅毒、1例脑(脊)膜血管梅毒;晚期神经梅毒7例,均为麻痹性痴呆]的临床症状、影像学表现、梅毒血清学和脑脊液检测、治疗及预后。结果 12例神经梅毒患者血清抗梅毒螺旋体特异性抗体和快速血浆反应素试验(RPR)、脑脊液抗梅毒螺旋体特异性抗体均呈阳性,9例脑脊液RPR试验阳性。5例早期神经梅毒患者中1例脑脊液压力升高、3例白细胞计数增加、4例蛋白定量升高;7例晚期神经梅毒患者中1例脑脊液压力升高、7例白细胞计数增加、7例蛋白定量升高,脑脊液细胞学均呈淋巴细胞反应且以小淋巴细胞为主。12例患者分别静脉滴注不同剂量青霉素或肌肉注射头孢曲松钠,8例神经精神症状明显好转、4例未见明显改善。结论神经梅毒发病形式多样,临床症状不典型,极易误诊。明确诊断需依靠临床表现及梅毒血清学和脑脊液检测,早期诊断和规范治疗对改善预后和减少并发症至关重要。     

神经梅毒的临床特点和诊断

目的分析神经梅毒的临床特点和诊断。方法回顾性分析10例神经梅毒患者的临床资料、实验室和神经影像学检查结果。结果男8例,女2例,平均(45±14.52)岁。无症状神经梅毒1例,脑血管梅毒4例,麻痹性痴呆4例,脊髓痨1例。血清和脑脊液甲苯胺红不加热血清反应素试验(TRUST)和梅毒螺旋体明胶凝集试验(TPPA)均阳性。4例脑脊液白细胞升高(20~80×106/L),分类以淋巴细胞为主,7例脑脊液蛋白升高(0.81~1.56g/L)。3例脑血管梅毒MRI示多发性梗死,3例麻痹性痴呆MRI有脑萎缩表现。结论神经梅毒的临床表现多样,诊断应综合考虑临床表现、梅毒血清学、脑脊液检查、神经影像学和流行病学资料。     

神经梅毒的临床特征(附12例报道)

目的通过分析神经梅毒的临床特征,探讨其诊断的相关问题。方法回顾性分析经临床和实验室检查确诊的12例神经梅毒住院患者的相关临床资料。结果12例患者中男7例,女5例,平均年龄37.25岁。均承认自己或配偶有非婚姻性生活史。脑脊液检查示全部患者脑脊液梅毒螺旋体血球凝集试验(CSF-TPHA)阳性,脑脊液快速血浆反应素试验(CSF-RPR)阳性者8例,白细胞和蛋白均升高者6例,白细胞单独升高3例,蛋白单独升高1例。头颅MRI表现为脑梗死、脑萎缩、占位性病灶、炎症样病变。12例患者中无症状神经梅毒1例,梅毒性脑膜炎2例,脑膜血管梅毒6例,麻痹性痴呆1例,脊髓痨2例。结论神经梅毒的诊断应综合考虑病史、临床表现、梅毒血清学、脑脊液、影像学等资料。     

61例糖尿病患者周围神经传导速度临床分析

目的 探讨周围神经传导速度（NCV）在糖尿病性周围神经病（DPN）诊断中的价值。方法 对61例糖尿病（DM）患者行正中神经、尺神经和腓总神经运动传导速度（MCV）及正中神经、尺神经和腓浅神经感觉传导速度（SCV）进行检测。结果 61例糖尿病患者NCV异常率为81．97％（50／61）。共检测732条神经，MCV366条．SCV366条，异常率分别为27．05％（99／366）、49．45％（181／366），差异有非常显著性意义（P〈0．01）。上肢检测488条神经，异常率为30．94％（151／488）；下肢检测244条神经，异常率为48．77％（119／244），差异有非常显著性意义（P〈0．01）。结论 检测周围神经传导速度不但可以早期诊断糖尿病性周围神经病，而且有助于发现亚临床患者，是DPN的重要辅助检查手段。     

不典型脊髓亚急性联合变性的临床特征分析

目的探讨不典型脊髓亚急性联合变性（SCD）临床特点和诊断思维。方法对46例SCD患者的临床资料行回顾性分析,并对13例SCD患者首诊误诊的临床资料行重点分析。结果在46例SCD患者中,脊髓后侧索和周围神经、侧索和周围神经、后索和周围神经及后侧索同时受损者分别占52．17％,10．87％,10．87％,10．87％,仅有后索或侧索受损者分别占8．70％,6．52％。血红蛋白（Hb）降低者占43．48％,平均红细胞体积（MCV）升高者占71．74％;血清VitB12降低者占50％（21／42）;脊髓MRI检查发现异常者占28．57％（6／21）。在13例首诊误诊患者中,4例仅有后索和3例仅有侧索受损,侧索和周围神经、后索和周围神经同时受损者各2例,后侧索、后侧索和周围神经同时受损者各1例;5例血清VitB12降低,脊髓MRI检查显示异常1例;首诊误诊率占28．26％（13／46）。结论不典型SCD主要临床特点可为仅累及后索或侧索的非联合性脊髓受损表现,或后索和侧索受损表现不均衡,VitB12等检查可正常。准确识别脊髓后侧索受损的阳性表现、查找体内VitB12缺乏的佐证、排他疾病及VitB12试验性治疗等是避免不典型SCD误诊的有效措施。     

麻痹性痴呆的临床特征与诊断(附2例报告)

目的探讨麻痹性痴呆的临床特征，以及实验室及影像学检查在诊断中的价值。方法回顾性分析2例麻痹性痴呆患者的临床资料，并结合文献进行讨论。结果主要临床表现为隐匿起病，进行性加重性痴呆、精神障碍、癫痫发作等；2例患者均被误诊。血清及脑脊液快速血浆反应素环状卡片试验（RPR）阳性，梅毒螺旋体明胶凝集试验（TPPA）阳性，头颅MRI主要表现为弥漫性脑萎缩。结论麻痹性痴呆的临床表现复杂多变，早期误诊率高；诊断主要根据临床特点、血清学及脑脊液检查综合考虑。     

三例神经梅毒的临床特征与诊断

目的分析神经梅毒的临床特征及提供早期诊断依据。方法回顾性分析经临床和实验室检查确诊的3例神经梅毒患者的有关临床资料。结果神经梅毒的临床特征包括:(1)急性、亚急性起病多,少数慢性起病;(2)临床以间质型,尤其脑卒中最常见;(3)荧光螺旋体抗体吸收(FTA-ABS)试验及梅毒快速血浆反应素试验(RPR)阳性率极高;(4)脑脊液检查表现为蛋白含量增加、细胞数增多(以淋巴细胞为主);(5)头颅CT或MRI表现与高血压、糖尿病所致常见的脑梗死不同,病灶多发,可分布在脑的所有部位。结论神经梅毒误诊率高,临床表现、实验室及影像学检查是其诊断的重要依据。     

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.     

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.     

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.     

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.     

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.     

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.     

Effects of NMD A- and AMPA-Receptor Antagonists on Different Forms of Epileptiform Activity in Rat Temporal Cortex Slices

Summary: Lowering extracellular magnesium induces different patterns of epileptiform activity in rat hippocampus and entorhinal cortex. Short recurrent epileptiform discharges in the hippocampus are stable over time, whereas seizurelike events (SLEs) in the entorhinal cortex, the subiculum, and the neighboring neocortex develop into late recurrent discharges which are not blocked by clinically employed antiepileptic drugs. We tested the sensitivity of the different epileptiform discharge patterns to. /V-methyl-D-aspartate (NMDA)- and non-NMDA-receptor antagonists. As NMDA-receptor antagonist we used dextrorphan, ket-amine, and 2-aminophosphonovalerate (2APV); as α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA)-receptor antagonist we employed the quinoxaline derivative glutamate 6-cyano-7-nitroquinoxaline-2,3-dione (CNQX). The findings show that the different patterns of epileptiform activity, including the late recurrent discharges, are sensitive to all NMDA-receptor antagonists. However, when dextrorphan was employed to suppress seizure-like events, later recurrent discharges did not develop during the remaining time course of the experiment. CNQX reversibly suppressed recurrent discharges in the hippocampus and SLEs in the entorhinal cortex. However, late recurrent discharges become insensitive to CNQX, even at a high concentration of 60 μM m. This finding suggests a prominent role for NMDA receptors in the generation of late recurrent discharges.     

Pediatric Epilepsy Surgery

Summary: The use of implantable arrays of epidural electrodes has made it possible to carry out extraoperative electrocorticography (ECoG) and functional localization in the awake child. This has permitted cortical excisions that are determined by criteria similar to those obtained during surgical procedures performed under local anesthesia in adults. In addition, the method also permits simultaneous ECoG and video monitoring during the child's symptomatic seizures, providing additional important localizing information that is impractical to obtain in operations under local anesthesia. We report our experience with 75 children, ages 5 months to 15 years, whom we have managed with epidural electrode arrays. The method of extraoperative ECoG is described and illustrative cases are presented to demonstrate its feasibility and utility in children. In addition, we call attention to gliomas as a common cause of chronic focal seizures in children. Of 49 children undergoing resection and followed for from 1 to 14 years (mean of 5.8 years), 32 (65%) are either seizure free or have had a significant reduction in seizure frequency that has unambiguously improved their quality of life. The results are analyzed further by relating the surgical outcome to each of the pathologic entities that caused the seizures. This analysis reveals the variety of neurological conditions that commonly cause intractable focal seizure disorder in children and distinguishes those pathologic entities in which the seizure disorder is apt to respond to surgical intervention from those that will not.