婴儿脂肪瘤型脊髓拴系综合征的手术治疗方法及疗效分析

目的 通过对125例婴儿脂肪瘤型脊髓拴系综合征临床数据分析,评价其显微外科治疗方法及疗效.方法 清华大学玉泉医院医院自2004年1月至2012年1月收治并有效随访的婴儿脂肪瘤型脊髓拴系125例,患者术前均行神经系统、MRI、残余尿及神经电生理检查,术中在电生理监测下行显微外科手术治疗.疗效评估分为近期疗效和远期疗效,近期疗效根据Hoffman功能性分级、Kirollos术中松解分级和并发症评估,远期疗效根据临床表现、MRI、膀胱残余尿和神经电生理结果进行评估.统计学通过SPSS16.0分析处理数据.结果 近期随访:按Kirollos术中松解分级评定方法:108例(86.4％)达到Ⅰ级,13例(10.4％)达到Ⅱ级,4例(3.2％)为Ⅲ级;发生并发症14例,占11.2％.远期随访:根据Hoffman功能性分级:术后无症状组、轻度症状组症状组在术前和术后差异有统计学意义(P＜0.05),中度症状组、重度症状组在术前术后差异无统计学意义(P＞0.05);膀胱残余尿检查在术前和术后差异有统计学意义(P ＜0.05);MRI检查未发现脂肪瘤复发病例;神经电生理检查在术前和术后差异有统计学意义(P＜0.05).结论 婴儿脂肪瘤型脊髓拴系综合征的显微治疗可获得较好的疗效,术中神经电生理监测可提高手术安全性.     

儿童脊髓脂肪瘤的分型与疗效探讨(附211例分析)

目的探讨儿童脊髓脂肪瘤手术年龄、病理解剖类型与预后的关系。方法回顾性分析211例儿童脊髓脂肪瘤的临床资料,以手术年龄、手术前后直肠膀胱功能评分和运动功能评价、术中解剖分型、脊髓松解程度进行综合评估。结果各病理解剖类型组中(除髓内型外),术前不同膀胱直肠功能评分的平均年龄之间具有显著差异(P<0.05)。手术能保持原有正常功能,术后直肠膀胱功能总体改善率48.3%、加重率0.97%,运动改善率43.2%、加重率0.96%。依脊髓松解程度分为可分离型和不可分离型,复合型42例(100%)和膨出型13例(32.5%)为不可分离型。结论病儿症状随年龄增长加重,说明早期手术必要性,手术具有稳定和改善症状作用,对不可分离型不可盲目再次手术。     

显微手术治疗脂肪瘤型脊髓栓系综合征的疗效分析(附611例报告)

目的 通过对611例脂肪瘤型脊髓栓系综合征临床数据分析评价其显微外科治疗的疗效.方法 患者均行神经系统和MRI检查,并经显微外科手术治疗.手术前后相应地应用尿动力学、神经电生理等检查手段.术后疗效根据Hoffman功能性分级、Kirollos术中松解分级、尿动力学和神经电生理等结果进行评定.结果 根据Hoffman功能性分级:术后无症状87例(14.2％),轻度289例(47.3％),中度214例(35.1％),重度21例(3.4％).按Kirollos术中松解分级评定方法:587例(96.0％)达到Ⅰ级,16例(2.6％)达到Ⅱ级,8例(1.3％)为Ⅲ级.尿动力学检查在术前和术后差异有统计学意义.神经电生理检查在术前和术后差异有统计学意义.结论 显微外科手术治疗脂肪瘤型脊髓栓系综合征可获得较好的疗效,尿动力学以及神经电生理的临床应用可以提高疗效.     

儿童脊髓脊膜膨出合并脊髓栓系综合征术后功能恢复的随访

目的 评估脊髓脊膜膨出合并脊髓栓系综合征(TCS)患儿手术后的治疗效果,来确定影响预后的决定性因素.方法 收集2003 - 2007年郑州市儿童医院收治153例脊髓脊膜膨出合并TCS患儿的临床数据,男62例,女91例.通过手术前后症状、体征的改善情况以及辅助检查(如:CT、MRI、泌尿系彩超、尿动力学检查等),对患儿术后恢复情况进行综合评估.结果 (1)大小便改善:术前便秘37例,术后便秘18例;术前大便失禁27例,术后大便失禁13例;术前尿潴留23例,术后尿潴留5例;术前尿失禁34例,术后尿失禁7例;(2)足部畸形及下肢轻瘫:术前101例,术后3年随访77例患儿术后足部畸形得到明显改善,24例患儿无改善(P=0.01);(3)膀胱功能恢复情况:28例术前神经源性膀胱患儿,术后20例膀胱顺应性和最大膀胱压测定容量明显改善(P＜0.05).5例单侧肾脏集合系统分离患儿术后半年及1-3年随访,肾脏集合系统分离明显改善.结论 脊髓病变的严重程度、手术是否完全得到松解以及早期手术、术后长期功能锻炼都是影响预后的重要因素,而是否早期手术对预后的影响尤为重要.     

显微松解术治疗儿童脊髓栓系综合征疗效分析

目的 总结分析儿童脊髓栓系的显微松解手术效果.方法 西安西京医院2007年1月至2011年12月收治脊髓圆锥和马尾部神经症状患儿80例.MRI显示全部病例伴有腰骶椎板缺如、脊髓低位、终丝增粗、脊髓末端占位.接受脊髓栓系松解手术(包括脊膜膨出囊和腰骶椎管内病变切除),28例患儿术中获得脊髓圆锥和马尾神经电生理监测,术后对67例患儿随访.结果 患儿术后住院期内自述症状缓解或减轻者69％ (55/80).大、小便异常的患儿中,术后改善者占62％(40/65);下肢感觉、运动以及发育和营养症状40例中,18例术后早期下肢感觉功能好转(45％),11例运动障碍出现改善(28％).随访时大小便功能较术前明显改善者占79％ (45/57);下肢感觉和运动障碍较术前明显改善者占63％ (22/35);术中接受脊髓圆锥和马尾神经电生理监测的患儿,比未接受监测者,自评症状改善率明显增加.结论 显微分离技术和神经电生理监测手段,是保障松解手术效果的关键因素.     

脊髓栓系综合征手术前后尿动力学研究

目的 研究脊髓栓系综合征患者手术前后尿动力学改变.方法 选择21例脊髓栓系综合征患者,手术前后的尿动力学检查指标包括膀胱初感觉容量、膀胱顺应性、最大逼尿肌压力、最大尿流率、残余尿量、逼尿肌反射.结果 21例患者中,术前尿动力学检查正常的2例;异常的19例,其中顺应性正常膀胱13例,低顺应性膀胱4例,高顺应性膀胱2例;逼尿肌反射正常3例,无逼尿肌反射8例,逼尿肌亢进5例,逼尿肌减退3例;术后2周内尿动力学复查结果显示膀胱顺应性、逼尿肌反射、膀胱初感觉容量与术前相比,差异无统计学意义;术后残余尿量较术前明显减少,差异有统计学意义(P＜0.05).结论 脊髓栓系综合征患者常有明显的膀胱功能障碍;手术可以改善患者的部分排尿功能;尿动力学检查对评估脊髓栓系综合征患者手术前后的膀胱功能有重要意义.     

75例儿童脊髓纵裂Ⅰ型的显微外科治疗

目的 通过对75例儿童脊髓纵裂I型临床资料分析,评价其显微外科治疗疗效.方法 患者术前均行神经系统检查、MRI检查以及CT检查,并经显微外科手术治疗,手术中综合运用电生理检测、气动磨钻及显微镜等设备.手术前后相应地应用尿动力学,神经电生理等检查方法.术后疗效根据临床症状、尿动力学和神经电生理等结果进行对比评定.结果 根据程斌等提出的临床疗效:本组75例患儿中,术后疗效优(痊愈)1例,良(显效)30例,可(有效)40例,差(无效)4例,总有效率94.6％.膀胱顺应性降低在术前和术后差异有统计学意义(P＜0.05).神经电生理检查在术前和术后差异有统计学意义(P＜0.05).结论 显微外科手术治疗儿童脊髓纵裂Ⅰ型可获得较好的疗效,并结合应用神经电生理监测和气动磨钻.     

脊髓栓系伴神经原性膀胱的治疗策略

目的探讨脊髓栓系综合征伴有神经原性膀胱的跨学科协作治疗。方法本组脊髓栓系综合征患者24例,年龄4~47岁,平均35.1岁,男10例,女14例。手术前后行尿流动力学测定,神经外科手术分离粘连、松解脊髓,修补缺损及硬脊膜重建。排尿障碍改善不佳者行尿道支架置入及骶神经调控器植入术。结果术前24例患者均诊断为神经原性膀胱,包括逼尿肌无力9例,扩约肌-逼尿肌共济失衡15例。病理诊断包括脂肪瘤8例,表皮样囊肿5例,先天性脊髓脊膜膨出11例。随访8~36月,19例患者术后尿流动力学检查有显著改善;3例放置尿道支架,2例行骶神经调控后改善。所有患者均无神经系统症状加重。结论伴有神经原性膀胱的脊髓栓系综合征应早期行栓系松解术,排尿障碍改善不明显者放置尿道支架或行骶神经调控术。早期诊断及成立医疗协作小组是积极有效的,治疗的最终目的是阻止神经病变加重及预防可能发生的上尿路系统损害。     

显性脊柱裂手术前后尿动力学对比分析

目的 探讨显性脊柱裂患儿手术前后尿动力学指标及临床表现的变化。方法 回顾性分析手术治疗的26例显性脊柱裂患儿的临床资料,包括12例脂肪瘤型脊髓脊膜膨出、10例脊髓脊膜膨出、4例单纯脊膜膨出。术后随访3~22个月,对比手术前后泌尿系超声、尿动力学检查结果。结果 术前出现下尿路症状8例,未发现肾积水;术后出现下尿路症状9例,新发肾积水2例。术后最大膀胱测定容量较术前明显增加（P<0.05）;而膀胱顺应性、残余尿量、最大膀胱容量时膀胱内压、最大尿流率时逼尿肌压力均无明显变化（P>0.05）。结论 显性脊柱裂术前最常见的尿动力学改变为膀胱排空异常及膀胱顺应性降低;手术前后下尿路功能障碍及短期临床表现改善不明显,需警惕远期并发症。     

儿童与成人脂肪瘤型脊髓拴系综合征的疗效对比性分析

目的通过对611例脂肪瘤型脊髓拴系综合征临床数据分析,比较儿童与成人脂肪瘤型脊髓拴系综合征的显微外科治疗疗效。方法清华大学玉泉医院自2004年1月-2010年1月收治并有效随访的脂肪瘤型脊髓拴系611例,分为儿童组和成人组,患者术前均行神经系统、MRI、残余尿及神经电生理检查,术中在电生理监测下行显微外科手术治疗。疗效评估分为近期疗效和远期疗效,近期疗效根据Hoffman功能性分级、Kirollos术中松解分级和并发症评估;远期疗效根据临床表现、MRI、膀胱残余尿和神经电生理结果进行评估。根据这些指标比较儿童组与成人组间的疗效差异。统计学通过SPSS16.0分析处理数据。结果近期随访：根据Hoffman功能性分级：术后改善的患者在儿童组和成人组间有明显差异（P〈0.05）,术后稳定的患者在在儿童组和成人组间无明显差异（P〉0.05）,术后加重的患者在儿童组和成人组间有明显差异（P〈0.05）;按Kirollos术中松解分级评定方法：Ⅰ级的患者在儿童组和成人组间有明显差异（P〈0.05）,Ⅱ级的患者在儿童组和成人组间有明显差异（P〈0.05）,Ⅲ级的患者在儿童组和成人组间有明显差异（P〈0.05）;儿童组发生并发症比成人组少。远期随访：膀胱残余尿减少的患者在儿童组和成人组间有显著性差异（P〈0.05）;MRI检查在儿童组和成人组间均未发现脂肪瘤明显复发病例;神经电生理改善的患者在儿童组和成人组间有显著性差异（P〈0.05）。结论儿童脂肪瘤型脊髓拴系综合征的显微治疗比成人可获得更好的疗效。     

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.