胼胝体变性六例影像学特点和临床表现

目的 探讨胼胝体变性(Marchiafava-Bignami disease,MBD)的影像学特点和临床表现.方法 对我院诊治的6例MBD患者进行回顾性分析,包括影像学检查(如头颅CT、MRI)、实验室检查、临床症状和随访情况.结果 6例患者MRI检查均表现为胼胝体肿胀及T1WI等或低信号,T2WI高信号,液体衰减反转恢复序列高信号,弥散加权成像示弥散受限.其中例1、例4和例6以急性意识障碍起病,CT和MRI检查除累及整个胼胝体外双侧半卵圆区也受累,预后较差.例2和例3以反应迟钝、记忆力下降起病,CT和MRI检查病变主要累及胼胝体膝部和压部,经积极治疗,患者基本恢复正常.例5以双下肢麻木无力、反应迟钝起病,MRI检查病变累及胼胝体压部及体部,经治疗后也恢复正常.结论 MBD临床表现复杂,但具有特征性影像学表现,其起病形式和影像学特征可作为反映其预后的重要因素之一.     

胼胝体变性的临床特点与影像学特征研究

目的　研究分析了一组胼胝体变性 (MBD)患者的临床表现和影像学特征 ,探讨不同临床分型的特点与诊断方法。方法　 4例长期酗酒男性MBD患者 ,急性型 1例 ,慢性型 3例 ,均经CT检查 ,3例经头MR检查 ,1例行尸检病理检查。结果　急性型主要表现为意识障碍、精神症状与癫 ;慢性型以痴呆、精神症状为主要表现 ;CT与MR可见胼胝体膝部和压部病灶。CT病灶为低密度 ,MR表现为T1WI低信号 ;T2 WI高信号 ;2例伴发双侧大脑半球白质对称性病灶 ,1例出现脑干对称性病灶。尸检病理提示 :胼胝体呈多灶性脱髓鞘与坏死改变。结论　急性型MBD意识障碍突出 ,病程进展迅速 ;慢性型以痴呆、精神症状和锥体束损害为主要表现 ;CT与MR为其生前诊断的主要手段 ,以胼胝体膝部、压部对称性病灶为其主要异常表现。     

Marchiafava-Bignami病的临床和影像学特点(附2例报告)

目的探讨Marchiafava-Bignami病(MBD)的临床及影像学特点。方法回顾性分析2例MBD患者的临床资料。结果本组2例患者均为男性,发病年龄分别为54、49岁,均有长期大量饮酒史。例1急性起病,以手抖、意识不清为主要症状。例2为慢性起病,以反应迟钝、记忆力减退以及头痛为主要症状。影像学检查示2例患者胼胝体区均存在异常病灶。给予B族维生素后患者情况均出现明显好转。结论 MBD临床表现各异,影像学检查常于胼胝体处发现异常,及时给予B族维生素治疗效果较好。     

成年男性胼胝体变性病的临床及影像学特点

目的分析胼胝体变性病(MBD)的临床表现及MRI特点。方法回顾性分析8例成年MBD男性患者的临床及影像资料。结果 8例均有大量饮酒史,临床表现主要为意识障碍,精神症状,认知障碍,以及癫痫等。MRI特征表现为病灶位于胼胝体压部两侧,左右对称,病灶表现为T_2加权,Flair及DWI序列高信号,相应ADC序列低信号。结论 MBD的临床表现呈现多样化且不具有特异性。通过在疾病早期对DWI序列出现双侧对称胼胝体病灶有助于疾病的诊断。     

合并皮质受累的胼胝体变性1例报道及国内报道荟萃分析

该文对1例合并皮质受累的胼胝体变性进行了报道,同时将该病例与国内文献检索出的27篇共50例胼胝体变性病例一起作临床特点的分析。在51例胼胝体变性病例中,49例有长期大量饮酒史。胼胝体变性临床表现复杂,难以定位,其中急性发病者19例,多表现为癫痫发作、意识水平下降;亚急性起病者13例,表现为精神异常、反应迟钝、行走不稳、尿便障碍等;慢性发病者19例,表现为言语不清、精神障碍、反应迟钝、肢体无力等。影像学表现为特征性胼胝体脱髓鞘改变,CT表现为胼胝体膝部或压部低密度灶,磁共振表现为相应部位脱髓鞘改变。胼胝体变性如能早期诊断与治疗,大部分患者预后良好。     

Marchiafava-Bignami病的临床及影像学研究

目的 探讨Marchiafava-Bignami病(MBD)的临床及影像学改变.方法 回顾性分析了7例MBD患者的临床和CT、MRI资料,包括病灶形态、分布、信号或密度改变等影像学特征:4例同时行CT和MRI检查,2例仅行CT检查,1例仅行MRJ检查.结果 本组患者急性型5例,均表现为胼胝体肿胀及长T1、长T2信号改变,均有双侧脑室周围白质、额叶皮层下白质对称性累及:慢性型2例,胼胝体明显萎缩变薄,并呈长T1、长T2信号及FLAIR像点片状或线样低信号灶.5例患者DWI显示病灶区信号明显增高并有2例出现弥散受限改变.结论 MBD具有特征性MRJ表现,其影像学改变可能反映其临床及预后.     

胼胝体压部可逆性病变的MRI诊断与临床分析

目的探讨胼胝体压部(splenium of the corpus callosum,SCC)可逆性病变的MRI表现及临床特点。方法回顾性分析8例脑MRI表现为单纯胼胝体压部可逆性病变患者的临床和影像学资料。结果 8例患者的SCC可逆性病变均为继发性,原发病分别为脑内感染5例,肝豆状核变性、低血糖脑病及脑外伤各1例。8例患者均急性起病,临床表现为发热、头痛5例,急性意识障碍2例,肌张力增高2例,颈强2例,均符合原发病的临床表现。8例均行脑MRI检查,均表现为T1低或等信号,T2及FLAIR序列高信号,DWI高信号(提示细胞源性水肿),其中4例行增强扫描未见强化。8例患者均于临床症状好转或消失后复查MRI提示病灶消失。结论 SCC可逆性病变在多种疾病中均可出现,无该病变相关的特异性临床表现,MRI表现提示病灶为细胞源性水肿可能。     

遗传性痉挛性截瘫伴薄型胼胝体的临床特征

目的 探讨遗传性痉挛性截瘫伴薄型胼胝体（HSP-TCC）的临床特征。方法 对4例HSP-TCC患者的临床资料进行回顾性分析。结果 4例患者均于青少年起病，表现为智能低下，痉挛步态，双下肢痉挛，无力，腱反射亢进，病理征阳性，无感觉障碍，2例有共济失调及大小便障碍；1例有双上肢痉挛及肌肉萎缩，头颅MRI显示胼胝体变薄。结论 HSP-TCC的主要临床特征为青少年起病的痉挛性截瘫，智能低下，头颅MRI显示胼胝体变薄。     

MRI在胼胝体梗死中的诊断价值

目的 探讨胼胝体梗死的磁共振影像特点及其临床特征。方法 对32例胼胝体梗死患者的图像进行分析，分析梗死的部位及在不同扫描序列中的表现，结合患者的临床表现进行对比研究。结果 胼胝体膝部梗死9例、胼胝体体部梗死11例胼胝体压部梗死12例；其中超急性梗塞1例，急性梗死2例，亚急性梗死6例。结论 MRI是诊断胼胝体梗死最有意义的检查方法，可以直接清晰显示梗死的有无、部位、范围，并能对病灶的新旧分期指导临床治疗。     

非酒精中毒性亚急性原发性胼胝体变性1例报道并文献复习

原发性胼胝体变性是一种罕见的以胼胝体受累为主的脱髓鞘和继发坏死的病变，其病因及发病机制尚不明确。该病主要见于中年男性，通常与长期大量酗酒或营养不良相关，其缺乏特异的诊断标准，容易漏诊及误诊。该文报道了1例非酒精中毒引起的亚急性原发性胼胝体变性患者。该例患者亚急性起病，有糖尿病病史，无饮酒史，主要临床表现为肢体乏力、进行性认知功能下降、行为异常及轻度意识障碍。颅脑磁共振成像示：胼胝体呈膨胀性改变，其内可见多发片状异常信号灶；液体抑制反转恢复序列呈高信号，弥散加权成像序列呈高信号，表观弥散系数序列呈低信号；以上病灶呈双侧对称性分布，累及胼胝体膝部、体部及压部。在给予补充大剂量维生素B₁、维生素B₁₂，控制血糖后，患者症状明显好转，神志清醒，言语较前流利，认知功能较前明显好转，肢体肌力基本正常。复查颅脑磁共振成像示：胼胝体对称性异常信号灶较前明显减少。该文旨在通过报道1例非酒精中毒性原发性胼胝体变性的病例，并复习该类疾病相关的国内外文献，探讨MBD的病因、发病机制、影像学表现、临床特点、诊断、鉴别诊断及治疗方法的研究进展。     

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.     

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.     

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.     

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.     

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.     

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.     

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.