肝性脑病10例临床及影像学分析

目的:了解急、慢性肝性脑病的临床表现及影像学特征。方法:回顾性分析10例肝性脑病的发病经过、肝病史特点、临床表现、肝功能、血氨、EEG、诱发电位、CT、MRI改变。结果:肝病史8例、严重胆道病2例、行门—腔分流术2例,起病表现:头晕头痛2例、精神异常3例、锥外系症状3例、癫痫发作1例、视力骤降1例。急性肝性脑病1例,发病时肝功能正常。慢性肝性脑病9例、肝功能有不同程度的损害,主要为TBIL升高、ALP升高和GGT升高,A/G倒置5例、血氨升高10例。EEG检查7例多为弥漫性慢活动。诱发电位检查4例VEP、BAEP均异常。头颅CT检查7例未见异常。头颅MRI表现:急性肝性脑病1例为双侧白质区大片状T_1WI等信号、低信号,T_2WI明显高信号,FLAIR明显高信号改变。慢性肝性脑病检查3例,2例正常,1例为双侧基底节区及苍白球、脑干对称性长T_1、长T_2异常信号改变。结论:肝性脑病的临床表现多样化,急性肝性脑病以脑水肿症状为主,而慢性肝性脑病以精神异常及锥外系症状为主,部分病人肝病史隐匿,肝功能损害不明显,但血氨均为不同程度的升高,EEG在昏迷前期多为弥散性慢活动,三相波少见,诱发电位均有异常,MRI在急性肝性脑病主要为白质信号改变,在慢性肝性脑病则为基底节区核团信号改变。临床医生对本病应提高警惕性。     

肝性脊髓病合并肝性脑病13例临床影像特点与预后

目的探讨肝性脊髓病合并肝性脑病的发病机制、临床表现、影像特点、治疗和预后。方法回顾性分析13例肝性脊髓病合并肝性脑病患者的临床表现、影像特点及预后,并结合文献进行分析。结果本病多发生于肝硬化自发性或手术门体分流后,临床主要表现可逆的脑病症状,进行性以双下肢为主的痉挛性瘫痪,仅1例有括约肌功能障碍,无肌萎缩、感觉障碍。9例血氨增高,9例脑电图表现弥漫性θ波,其中2例有三相波,8例MRIT1像显示基底神经节高信号,脊髓MRI阳性率低。所有患者均限制蛋白摄入量,给予护肝、降血氨、神经营养等治疗,3例加用糖皮质激素。9例患者脑病症状缓解,但瘫痪改善不明显;死亡3例,仅1例肝移植患者预后良好。结论脊髓症状与脑病症状并不平行消长,可能存在不同的发病机制。神经影像学研究为慢性肝病神经系统并发症的诊断提供了新思路。常规治疗脑病症状好转,但脊髓病变预后不良,肝移植为慢性肝病神经系统并发症带来曙光。     

桥本氏脑病脑电图的发现

桥本氏脑病是一种伴有癫痫和弥漫性脑电图(EEG)异常的周期性障碍,本文作者报告7例具有脑病、癫痫或两者均有,且伴有抗甲状腺抗体滴度升高病人的脑电图发现。5例病人有普遍性慢波或额叶节律性慢波,2例有三相波,1例有周期性尖波,3例有局灶性左颞慢波。并对其中3例病人详述如下: 例1,一位68岁妇女,患有发作性意识模糊、定向力障碍、震颤,症状在2周内进行性加重。神经系统检查:反应迟钝,自发性活动。脑脊液蛋白增高,余     

Creutzfeldt-Jakob病临床诊断1例分析

Creutzfeldt-Jakob disease(CJD)是一种由朊蛋白感染引起的罕见的神经变性疾病，称人海绵状脑病，老年人好发，多于65岁左右起病，发病率为0．25～2／百万人口／年。临床上表现为快速发展的进行性痴呆，共济失调，肌阵挛，可伴有锥体系及锥体外系症状，脑电图描记为周期发放的尖波，三相波，MRI上可表现为双侧尾状核T2加权高信号，弥散成像高信号，病理表现为弥散性非炎症性的神经元变性或丧失，星形胶质细胞增生，神经元和星形胶质细胞的胞浆内有空泡形成。     

散发性Creutzfeldt-Jakob病30例临床分析

目的探讨散发性Creutzfeldt-Jakob病(sCJD)的临床、脑电图(EEG)及影像学特征。方法回顾性分析30例sCJD患者的临床资料。结果本组急性起病6例,亚急性起病18例,在发病1～3个月出现意识障碍、肌阵挛、去皮质强直状态;慢性起病6例,发病后1～2年出现上述典型症状。本组患者EEG均异常,早期表现广泛持续性慢波,中晚期出现典型三相波18例、不典型三相波8例。本组MRI表现双侧基底节区T2WI对称性高信号10例,右侧豆状核高信号1例;17例行MR弥散加权成像(DWI)扫描,均出现一侧或两侧额顶叶和/或枕叶皮质高信号,其中8例合并双侧基底节区对称性高信号。结论sCJD以亚急性起病多见,早期头颅DWI即可出现特征性额顶叶和/或基底节区高信号,为早期临床诊断提供依据;中晚期均出现意识障碍、肌阵挛、去皮质状态,EEG特征为三相波。     

9例散发型克雅病患者的临床表现和脑电图特点分析

目的 探讨散发型克雅氏病(CJD)的临床特点、脑电图(EEG)及MRI表现。方法 回顾性分析9例高度疑似克雅氏病患者的临床资料。结果 本组9例患者中7例亚急性起病,2例慢性起病,主要的临床症状和体征有进行性痴呆、精神行为异常、头晕、共济失调、肌阵挛、锥体外系症状和锥体束征等; EEG检查均异常,其中7例表现为典型的周期性三相波,2例为弥漫性慢波; 所有病例均行头部磁共振成像(MRI)检查显示大脑皮层、尾状核和弥散加权像(DWI)的高信号。所有病例行脑脊液14-3-3蛋白检测,其中6例为阳性,3例为阴性。结论 临床上对快速进展性痴呆患者应进行脑电图、头颅MRI检查,并检测14-3-3蛋白,以利于CJD的早期诊断。EEG与临床症状密切相关,7例患者在中晚期表现出典型的周期性尖慢复合波(PSWCs)和肌阵挛的临床特征。     

伴有严重惊厥的获得性癫痫性失语

目的探讨伴有严重癫痫发作的获得性癫痫性失语的病因和发病机制。方法对7例先有癫痫发作继而出现明显失语的儿童患进行了病因、病程、癫痫发作情况、失语表现及治疗情况进行了回顾性分析。同时分析了入院前后的EEG、头CT及MRI。结果全部病人失语症状出现前均有较严重的癫痫发作。其中5例在失语前曾出现过癫痫状态。全部病人表现听不懂问话，5例表现完全不能用语言表达。常规EEG5例异常，主要表现弥漫性慢波，其中3例可见阵性高波幅慢波发放。影像学检查3例异常。经AEDs及神经营养荆治疗，2例语言功能恢复，2例改善，3例无改善。结论本组病人主要表现全面性癫痫发作，惊厥性脑损伤可能为本组病人失语发生的主要原因。失语主要表现为“语言的听觉失认”。早期诊断并采取包括AEDs应用的综合治疗措施有利于改善病人的预后。     

人工肝血浆置换联合血液滤过治疗肝衰竭并肝性脑病临床观察

目的探讨人工肝血浆置换联合血液滤过治疗肝衰竭并肝性脑病的疗效。方法回顾性分析69例肝衰竭并肝性脑病患者,在综合内科治疗的基础上予PE联合HF治疗,观察对肝性脑病不同分期的治疗效果及不良反应。结果PE联合HF治疗后,患者消化道症状均有所改善,黄疸减轻,精神神经症状均有所好转,其中Ⅰ期肝性脑病35例、Ⅱ期17例、Ⅲ期4例均神志转清、扑翼样震颤消失、氨臭味消失,Ⅱ期1例、Ⅲ期3例、Ⅳ期1例精神神经症状减轻、昏迷变浅,Ⅲ期3例、Ⅳ期5例无变化;治疗后TBIL、ALT、AST、血氨、UREA、Cr均较治疗前明显下降,差异均有统计学意义(P0.05);PTA、钾、钠、氯、钙与治疗前比较均明显升高,差异有统计学意义(P0.05);总有效率88.41%,不良反应发生率14.02%,经对症处理均缓解并完成PE联合HF治疗。结论 PE联合HF治疗是一种安全有效治疗肝衰竭并肝性脑病的方法。     

单次发作的诊断性评价——是否必要?

作者对62例单次发作的患者于24小时内作EEG和CT等项检查,并评价诊断中作CT的必要性.病人年龄16～86岁,平均45岁.神经系统检查正常34例,平均年龄37岁,异常28例,平均年龄56岁.部份性发作10例,全身性发作52例.入院病因诊断为特发性30例,脑血管病17例,外伤后6例,戒酒6例,2例肿瘤和1例高血压脑病.40例(64%)EEG异常中,出现弥散和局灶性慢波分别为10和18例,而弥散与局灶性阵发性异常分别为9例和3例.29例显示CT异常,包括脑萎缩13例(21%)、急或慢性     

复发性格林—巴利综合征

报道３例复发性格林－巴利综合征病例，发现其具有明显的前驱因素，从发病到症状高峰少于１个月，易累及颅神经引起呼吸衰竭，发作期脑脊液蛋白质升高，神经传导速度减慢，１例病理上以周围神经脱髓鞘为主，伴有轻度炎性细胞浸润及轴索变性，此与慢性炎性脱髓鞘性多神经病相区别，其临床表现为数次急性炎性脱髓鞘所致。     

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.     

Hepatic Considerations in the Use of Antiepileptic Drugs

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.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

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.     

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.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.     

Special Pharmacokinetic Considerations in Children

Summary: Pediatric patients have greater degrees of pharmacokinetic variability and unpredictability than adults. This variability results from the effects of pharmacogenetics, age and growth, prior and current comedication, and disease. Newborns with seizures have the least predictable dosage requirements, and their needs change as drug-eliminating mechanisms mature in the neonatal period. Infants have the highest relative capacities to eliminate antiepileptics of any age group and require the largest relative doses. In addition to age-related trends, children demonstrate the same drug-specific, pharmacokinetic phenomena that adults do, including nonlinear phenytoin elimination, nonlinear valproate binding, and autoinduction of carbamazepine. Intercurrent illness and drug interactions further modify the age-related pharmacokinetic patterns in children and make dosage requirements even more unpredictable. Recent studies have shown that febrile illness can affect drug elimination, sometimes decreasing drug levels by 50% or more. Intermittent treatment with benzodiazepines administered either orally or rectally can be an important adjunct and help minimize this type of problem for children with marginally controlled epilepsy. Intermittent benzodiazepines are also helpful for children who have febrile seizures and who need only occasional antiepileptic protection.