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。     

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.     

复杂脊髓拴系综合征的手术疗效分析

目的探讨不同类型复杂脊髓拴系综合征的手术时机、方式和疗效。方法回顾性分析兰州大学第二医院自2011年1月~2017年4月收治的154例复杂脊髓拴系综合征患者的资料。按照患者性别、年龄、拴系发生部位及病理表现进行分类。所有患者均接受电生理神经功能监测辅助下的显微外科手术,术中拴系松解程度采用Kirollos和Van-Hille分级;术后依据Hoffman神经功能分级评判疗效。结果手术无死亡病例,术中完全松解率97%。对其中126例患者随访3个月~3.8年。依据Hoffman神经功能分级,术前分级0~3级的患者术后症状改善率显著高于4~5级患者;有1例术后症状加重,其余患者症状或体征均有不同程度改善。结论复杂脊髓拴系综合征的诊断和治疗非常复杂和困难,手术难度较大,传统手术后神经功能障碍的发生率高。对于该病患者,确诊后应尽早行手术治疗。在术中应用手术显微镜及电生理神经功能监测,可以有效降低脊髓及神经损害,提高手术的效果和安全性。     

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.     

髓/囊比值对儿童复杂脊髓脂肪瘤手术后再拴系的影响

目的探讨髓/囊比值对儿童复杂脊髓脂肪瘤手术后远期再拴系的影响。方法回顾性分析2009年1月至2014年6月深圳市儿童医院神经外科采用手术治疗的复杂脊髓脂肪瘤患儿的临床资料,共56例。采用神经电生理监测下显微手术切除脂肪瘤+重塑神经基板+硬膜囊成型的方法。术后应用MRI评估髓/囊比值,分析髓/囊比值对术后远期脊髓再拴系的影响。结果56例患儿均顺利完成手术。术后MRI显示髓/囊比值<30%者10例,30%~50%者31例,>50%者15例。患儿均未发生术后严重并发症。术后中位随访时间为8.9年(5.0~10.5年),失访4例。6例(11.5%,6/52)发生脊髓再拴系需要再次行手术治疗(再拴系组),其中术后髓/囊比值为30%~50%者2例,>50%者4例。无再拴系组的46例患儿髓/囊比值<30%10例,30%~50%27例,>50%9例。两组髓/囊比值差异有统计学意义(P=0.039)。结论髓/囊比值是影响儿童复杂脊髓脂肪瘤术后再拴系的重要因素,髓/囊比值越小,再拴系的概率越小。     

脂肪脊髓脊膜膨出的手术治疗

目的探讨脂肪脊髓脊膜膨出(LMM)的手术疗效。方法回顾性分析20例LMM病人的临床资料,术前Hoffman 0级17例,1级1例,2级2例。疗效评价:术后Hoffman分级较术前降低或无变化为有效,升高为恶化。按Kirollos和Hille术中栓系松解分级评定法评定栓系松解程度。结果 20例随访6～36个月,平均27.6个月。一级松解11例,二级松解9例。Hoffman 0级6例,1级1例,2级12例,4级1例;本组治疗有效11例(55%),恶化9例(45%)。无严重并发症发生。结论 LMM的手术治疗应综合考虑手术时机、脂肪瘤切除程度、神经栓系松解程度、手术疗效的评估手段和术后随访等,从而提高手术疗效。     

良性周围神经鞘瘤的临床特征及显微外科治疗

目的探讨良性周围神经鞘瘤的临床特点及显微外科手术疗效。方法回顾性分析12例良性周围神经鞘瘤,受累神经:臂丛神经6例,尺神经2例,正中神经2例,腓总神经、胫神经各1例。术前均行高频超声检查,对肿瘤较大或部位深在者行MRI检查。均在神经电生理监测下行显微手术切除肿瘤。结果 12例超声或MRI的影像学诊断与病理诊断均一致。肿瘤全切除11例,大部切除1例,术后均无严重并发症。随访4~34个月,肿瘤无复发,预后良好。结论术前通过高频超声或MRI检查常可明确诊断。术中显微外科技术联合神经电生理监测技术有助于提高手术安全性,减少术后并发症。     

尿流动力学检查在脊髓脂肪瘤患儿中的评估价值

目的 探讨尿流动力学检查对脊髓脂肪瘤患儿的评估价值.方法 回顾性分析上海新华医院小儿外科2010 - 2011年间16例脊髓脂肪瘤初次手术患儿的临床资料,对患儿术前及术后半年的临床症状、尿流动力学检查、影像学表现、术中分型及松解程度进行综合评估,比较各型患儿术后恢复情况.结果 根据术中栓系松解程度分为可完全松解型(A组,10例)及不可完全松解型脊髓脂肪瘤(B组,6例).术前无明显排尿功能异常者3例,所有患儿均有不同程度的尿流动力学结果异常.术后半年进行再评估时发现所有患儿经手术治疗后均基本能保持原有神经功能,其中临床症状改善率达43.8％(A组6例,B组1例),加重25.0％(A组1例,B组3例).尿流动力学结果改善率62.5％(A组7例,B组3例),加重率25.0％(A组1例,B组3例).结论 脊髓脂肪瘤患儿常伴有不同程度的膀胱功能障碍,于术前行尿流动力学检查可以早期发现亚临床期膀胱功能障碍的患儿,从而帮助临床医师及时手术干预.术后定期行该检查能够帮助监测患儿术后排尿情况,必要时及时处理.尿流动力学检查对脊髓脂肪瘤患儿具有重要意义.     

神经电生理监测下显微手术治疗脊髓髓内海绵状血管瘤

目的探讨神经电生理监测下显微手术治疗脊髓髓内海绵状血管瘤的效果。方法我院2008年10月至2012年10月收治经病理学证实的髓内海绵状血管瘤25例（男性12例,女性13例）;均在神经电生理监测下完成显微手术治疗,用Frankel分级评价手术疗效。结果术后随访时间为1～4年,平均2.3年。术前Frankel分级A级4例,B级1例,C级5例,D级8例,E级7例;术后1年Frankel分级A级3例,B级2例,C级3,D级7例,E级10例。术后症状改善18例（72%）,症状维持不变4例（16%）,症状加重3例（12%）。结论 MRI是术前诊断脊髓髓内海绵状血管瘤的最佳方式,手术是治疗脊髓髓内海绵状血管瘤的有效方法,术中联合神经电生理监测能够使手术更安全、更有效。     

神经电生理监测下手术切除成熟型脊髓畸胎瘤

目的探讨神经电生理监测下成熟型脊髓畸胎瘤的显微外科手术治疗效果。方法回顾性分析在神经电生理监测下经显微外科治疗的7例脊髓畸胎瘤病人的临床资料,同时复习相关文献。结果 1例脊髓圆锥处肿瘤予以大部分切除,3例原发于终丝的肿瘤予以全切,余3例均予以近全切除。病理诊断均为成熟型脊髓畸胎瘤,术后随访3~38个月,症状明显改善6例,无改善1例。术后McCormick分级:Ⅰ级4例,Ⅱ级2例,Ⅲ级1例。结论在神经电生理监测下最大程度切除成熟型脊髓畸胎瘤,有助于减少手术损伤,改善病人预后。     

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.     

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.     

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.     

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.     

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.     

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.