痛性周围神经病的诊断和治疗共识

痛性周围神经病(painful peripheral neuropathy)是以神经病理性疼痛(neuropathic pain)为突出表现的周围神经病,通常是指痛性感觉周围神经病(painful sensory peripheral neuropathies)或痛性感觉运动周围神经病(painful sensory and motor peripheral neuropathies).在本文中是指痛性感觉周围神经病以及泛化于伴有疼痛症状的周围神经病,包括可有运动-感觉共存的混合体.痛性周围神经病可以是单一的疾病主体,也可以是系统性疾病的表现.病变主要累及小或无髓神经纤维(C类纤维),可伴有或不伴有大纤维的病变.     

小纤维感觉神经病概述

小纤维感觉神经病(sm a ll fiber sensory neuropath ies,SFSN)或称小纤维神经病(SFN),为一类主要波及小直径有髓纤维和无髓纤维的感觉性周围神经病。它多为自发性,主要临床表现为感觉障碍、周围神经痛和(或)自主神经功能障碍,而神经病学及常规电生理检查正常,因此给临床工作带来很多困难。由于其发病机制至今尚未完全阐明,目前其治疗常集中于直接缓解疼痛的对症治疗。现就SFN简要概述如下。1解剖学基础神经纤维可根据其直径大小分为3类,直径小于5μm统称为细纤维。E rlanger和G asser又将细纤维分为2类:Aδ和C纤维。Aδ纤维直径为1~5…     

小纤维神经病

小纤维神经病(small fiber neuropathies,SFN)是指主要累及小直径有髓纤维和无髓纤维的感觉性周围神经病。虽然临床上SFN很常见,有时以全身性周围神经病为首发表现,因此很难诊断。有资料统计美国约两千万40岁以上的人有周围神     

小纤维神经病诊断方法进展

小纤维是指薄髓的Aδ及无髓C类纤维,纤维直径小,传导速度慢,纤维性质包含躯体感觉神经成分和自主神经成分,其中躯体感觉神经纤维主要介导痛温觉传导,而自主神经成分完成神经对内脏及皮肤附属器的支配.小纤维神经病(small-fiber neuropathy,SFN)是一组主要累及周围神经的小纤维成分,大纤维成分不受累或很少受累的周围神经病.疼痛、感觉异常和(或)自主神经功能障碍是SFN最典型的临床表现,也是影响患者生活质量的最主要原因.     

小纤维感觉神经病皮肤神经活检五例报告

小纤维感觉神经病 (ｓｍａｌｌｆｉｂｅｒｓｅｎｓｏｒｙｎｅｕｒｏｐａｔｈｉｅｓ,ＳＦＳＮ)或小纤维神经病为一类主要波及小直径有髓纤维 (<5 μｍ)和无髓纤维的感觉性周围神经病。小直径纤维的损害程度大大超过大直径纤维时 ,称为小直径纤维感觉神经病。主要临床表现为刺痛、感觉迟钝、痛觉及温度觉降低 ,肢体远端多见 ,常见于糖尿病、淀粉样变性、营养障碍、肿瘤、药物中毒、人类免疫缺陷病毒 (ＨＩＶ)感染、遗传性感觉神经病、莱姆病、血管炎等疾病。长期以来缺乏检查方法 ,此类疾病一直不被认识 ,近年来由于建立了皮肤神经活检免疫染…     

小纤维神经病的诊断和治疗

小纤维神经病是一种很常见的周围神经病,通常表现为神经痛和（或）自主神经功能障碍。尽管病因不清楚,但主要的病因包括糖尿病前期状态和免疫介导的疾病等,其中以糖尿病性最为常见。由于在一般的临床体格检查和常规电生理中没有异常表现,所以诊断较困难。目前常用的诊断方法包括有定量感觉测定、定量发汗轴索反射测定及皮肤活检躯体表皮内的神经纤维定量检查等,其中皮肤活检被认为是目前诊断的“金标准”。目前尚无特异的治疗方法。     

小纤维神经病诊断研究进展

小纤维神经病系指主要累及小直径有髓鞘Aδ类纤维和无髓鞘C类纤维的一组疾病,典型表现为异常疼痛、痛温觉缺失和自主神经功能障碍。神经传导速度检测可以检出大纤维(Aα类和Aβ类纤维)病变,而无法评价小纤维功能。本文拟对近年来小纤维神经病的神经病理学、神经电生理学和自主神经功能检测等筛查和诊断方法研究进展进行简要概述。     

有机磷中毒后迟发性周围神经病的临床和神经病理四例

目的探讨有机磷中毒后迟发性周围神经病(OPIDP)的临床及神经病理改变特点。方法对4例口服有机磷后出现周围神经损害症状的患者进行电生理和腓肠神经活检检查。结果 4例患者于急性中毒后平均20.5(10～24)天出现以下肢受累为主的逆行性运动感觉周围神经病,其中2例患者存在锥体束征。电生理检查提示以运动神经受累为主的轴索性周围神经损害。腓肠神经活检主要表现为与病程相关的轴索损害及再生现象,可见急性期的炎性反应、小纤维损害和继发的髓鞘改变。结论有机磷中毒后迟发性周围神经病表现为以运动障碍为主的逆行性神经病,中枢神经系统亦可能受累及;周围神经病理表现为轴索损害为主,同时存在小纤维损害及继发的髓鞘改变。     

神经病理性疼痛与头面痛

神经病理性疼痛为躯体感觉系统病变或疾病直接导致的疼痛,其中头面部常见神经病理性疼痛包括三叉神经痛、舌咽神经痛、枕神经痛、带状疱疹后遗神经痛等,其疼痛性质多呈刀割样、烧灼样、过电样,表现为自发性疼痛、痛觉过敏、异常性疼痛等临床特征.偏头痛为常见的原发性头痛,发作时伴恶心、呕吐或畏光、畏声、畏嗅,活动后症状加重;慢性每日头痛包括慢性偏头痛、慢性紧张型头痛、新发持续每日头痛等,每月头痛时间超过15天,持续时间3个月以上.上述两类头痛的发病机制中均存在中枢敏化,而这也是神经病理性疼痛的重要发病机制之一.     

急性运动轴索性神经病伴颅压增高二例报道

吉兰-巴雷综合征(Guillain-Barre syndrome,GBS)是一类免疫介导的急性炎性周围神经病.临床特征为急性起病,临床症状多在2周左右达高峰,表现为多发神经根及周围神经损害,常有脑脊液(CSF)蛋白-细胞分离现象,多呈单时相自限性病程,静脉注射免疫球蛋白和血浆置换治疗有效.该病包括急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating polyradiculoneuropathy,AIDP)、急性运动轴索性神经病(acute motor axonal neuropathy,AMAN)、急性运动感觉轴索性神经病(acute motor-sensory axonal neuropathy,AMSAN)、Miller Fisher综合征(Miller Fisher syndrome)、急性泛自主神经病(acute panautonomic neuropathy)及急性感觉神经病(acute sensory neuropathy,ASN)等亚型.其中AMAN是以广泛的运动脑神经纤维和脊神经前根及运动纤维轴索病变为主.现将作者医院收治的2例AMAN伴颅压增高病例进行报道.     

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.     

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.