显微松解术治疗26例儿童脊髓栓系综合征疗效分析

目的观察显微松解术对小儿脊髓栓系综合征的治疗效果。方法选择26例脊髓栓系综合征行显微松解术患儿为研究对象,回顾分析其治疗效果。结果 26例患儿中,18例自感症状明显减轻;术前存在大、小便异常症状的21例患儿,术后13例症状显著改善。术前存在下肢运动及感觉功能障碍症状的13例患儿,术后6例下肢感觉功能增强,4例运动障碍明显改善。结论采用显微松解手术治疗脊髓栓系综合征,可显著改善患者的大小便异常、下肢功能障碍等症状,有较高的临床使用价值。     

神经电生理监测技术在圆锥马尾病变手术中的应用

目的探讨神经电生理监测技术在圆锥马尾病变手术中应用价值。方法回顾性分析110例圆锥马尾病变患者临床资料,其显微外科手术均在神经电生理监测下进行,感觉诱发电位(somatosensory evoked potential,SEP)和运动诱发电位(motor evoked potential,MEP)监测脊髓功能,肌电图(electromyography,EMG)确定肿瘤切除范围。结果显微镜下病变全切除92例(83.6%),次全或大部分切除18例(16.4%)。术后随访1~58个月,神经系统查体及JOA评分发现脊髓神经功能改善102例(92.8%),无变化4例(3.6%),下降4例(3.6%)。对病变切除前与切除后SEP潜伏期和波幅以及MEP潜伏期进行自身比较,脊髓神经功能改善和下降患者电生理监测指标改变差异有统计学意义(P0.05),脊髓神经功能无变化患者相关监测指标改变差异无统计学意义(P0.05),神经电生理监测指标的变化与术后脊髓神经功能改善情况基本相吻合。结论术中神经电生理监测可以实时了解脊髓神经功能的完整性,结合显微神经外科技术可以明显提高圆锥马尾病变的全切率,减少术后并发症,提高手术疗效及安全性。     

骶尾部脊髓脂肪瘤的手术技巧

目的 介绍骶尾部脊髓脂肪瘤的病理解剖及相应的手术技巧.方法 2005年9月至2012年1月,上海儿童医学中心手术治疗骶尾部脊髓脂肪瘤119例,年龄1个月至18岁,神经系统症状有大小便失禁和(或)双下肢功能障碍.手术方法包括:切除皮下和椎管内脊髓外的脂肪瘤,脊髓内的脂肪瘤大部分切除,将脊髓从两侧硬脊膜上剪开,圆锥从硬脊膜囊末端剪开并分离下来,松解脊髓栓系.结果 骶尾部脊髓脂肪瘤主要病理解剖与腰骶部脊髓脂肪瘤相似,不同之处在于圆锥生长在末端硬膜囊上,圆锥发出的脊神经从圆锥腹侧斜向下方发出,脂肪瘤仅生长到脊髓内,并未生长到脊髓下方的脊神经内.108例得到随访,随访时间0.5～7.0年.术前有症状的患儿,术后多数有不同程度改善,1例症状加重,6例术后症状一过性加重;10例无症状者术后9例仍然无症状,1例症状一过性加重.结论 只有充分认识骶尾部脊髓脂肪瘤病理解剖,才能最大限度地切除脂肪瘤,彻底解除脊髓栓系,同时又能避免神经损伤,重建脊髓的正常解剖结构,最大限度恢复神经功能.     

脊髓末端积水合并脊髓栓系综合征10例临床研究

目的总结脊髓末端积水合并脊髓栓系综合征的临床表现、影像学特点、诊断与治疗经验。方法与结果 10例脊髓末端积水合并脊髓栓系综合征患者(1例为全脊髓积水、8例为骶管内脊膜囊肿、1例合并骶部皮毛窦),临床表现为不同程度双下肢无力,尤以肢体远端显著,5例伴尿道和肛门括约肌功能障碍;5例存在与脊髓空洞平面相关的感觉障碍平面,尤以浅感觉减退为主。术前MRI显示脊髓低位、脊髓末端无圆锥结构、脊髓和终丝内异常信号。于手术显微镜下切断终丝、引流髓内积水、切除骶管内脊膜囊肿、松解脊髓栓系。手术成功率达100%,平均手术时间2.15 h、术中出血量220 ml。无一例发生手术相关并发症。术后视觉模拟评分降低,下肢肌力、尿道和肛门括约肌功能改善。术后平均随访6.10年,Mc Cormick神经功能分级均达到Ⅰ级。末次随访时复查MRI显示,脊髓圆锥结构恢复,脊髓末端上升,髓内积水消失,脊柱生理曲度未发生变化。结论脊髓末端积水合并脊髓栓系综合征临床罕见,主要表现为慢性脊髓功能障碍;MRI特征性表现为脊髓低位,圆锥结构消失,终丝和髓内呈长T1、长T2信号,囊壁无强化。于手术显微镜下切断终丝、引流髓内积水、松解脊髓栓系,效果满意。     

儿童脊髓拴系综合征的显微外科治疗

目的 研究显微手术治疗儿童脊髓拴系综合征的疗效。方法 回顾性分析35例儿童脊髓拴系综合征，平均年龄2.2岁。均伴有脊柱裂，合并单纯脊髓脊膜膨出20例，脊髓脊膜膨出合并椎管内脂肪瘤13例，畸胎瘤1例，皮样囊肿1例。行显微手术松解黏连的脊髓和神经，切断牵拉的终丝。结果 会阴部感觉异常总有效率为80．0％，排尿功能障碍总有效率为77．1％．结合矫形手术可明显改善运动障碍。结论 显微外科手术松解脊髓圆锥和马尾神经及切断牵张的终丝．是治疗儿童脊髓拴系综合征的关键．结合矫形手术可提高病儿的生存质量。     

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成人脊髓栓系综合征

目的 分析手术治疗成人脊髓栓系综合征患者的疗效,评价脊髓栓系综合征显微手术中运用神经电生理监测的作用和意义。方法 2004年1月至2011年12月收治成人脊髓拴系综合征患者112例,98例获得完整随访资料纳入研究,其中63例患者术中采用神经电生理监测（监测组）,35例未采用神经电生理监测（对照组）。采用超声测定膀胱残尿量评价膀胱功能,术后6个月残尿量/术前残尿量≤50%为改善;采用Fugl-meyer下肢评分评价下肢运动功能,术后6个月评分增加3分以上改善。结果 对照组术后6 个月膀胱功能改善率为28.5%,监测组术后6 个月改善率达56.5%;监测组膀胱功能恢复明显优于对照组（P<0.05）。对照组术后6个月下肢运动功能改善率为62.5%,监测组术后6个月改善率为78.9%;两组下肢功能改善率无明显差异（P>0.05）。结论 脊髓拴系综合征显微手术中,神经电生理监测技术有助于防止术中误伤神经,从而使神经外科医生比较精准的剪断终丝获得最大程度的松解,改善患者生活质量。     

单纯性脊膜膨出只需要行脊膜膨出修补术吗?

目的 探讨单纯性脊膜膨出是否会合并脊髓栓系,是否应在行脊膜膨出修补术时探查椎管腔,解除脊髓栓系.方法 观察上海儿童医学中心收治的单纯性脊膜膨出患儿38例,综合MRI及术中椎管腔探查所见,分析单纯性脊膜膨出是否合并其他导致脊髓栓系的病变.结果 MRI及手术发现37例(97％)患儿除脊膜膨出外还合并有其他相关脊髓病变,如终丝牵拉、蛛网膜囊肿、表皮样囊肿、纤维束带、脊髓神经粘连等.脊膜膨出修补术中同时松解脊髓栓系,术后未见神经功能损害,长期随访患儿症状均改善或稳定.结论 单纯性脊膜膨出常常伴有脊髓栓系,MRI是其必要检查.一旦确诊,建议积极手术治疗,术中除修补膨出脊膜外,还应解除脊髓栓系.     

脂肪瘤型脊髓栓系综合征的手术技巧

目的 探讨脂肪瘤型脊髓栓系综合征的手术治疗技巧. 方法 自2003年至2006年,我科共收治33例脂肪瘤型脊髓栓系患者,均在电生理监测下切除脂肪瘤,显微手术分离粘连、松解脊髓,以无损伤不可吸收缝线(Prolene线,5-0)连续缝合硬脊膜,一期行硬脊膜重建;翻转腰背部筋膜行先天性缺损修补与加固. 结果 29例患者切口一期愈合:4例患者出现脂肪坏死导致皮下积液,其中2例患者出现脑脊液漏,经缝合及引流后治愈.随访1～3年,28例患者临床症状有显著改善,5例患者虽无改善亦无神经系统症状加重;复查均无再栓系形成. 结论 脂肪瘤型脊髓栓系综合征早期电生理监测下行栓系松解术,同时行硬脊膜的修补与重建,对于预防术后再栓系至关重要.     

儿童脊髓栓系综合征的临床分型、手术治疗及疗效分析

目的 探讨儿童脊髓栓系综合征(TCS)的临床分型及手术方法,并对疗效进行分析.方法 218例儿童TCS患儿,根据临床表现和MRI检查结果分为五种类型:终丝紧张型、脊膜脊髓膨出型、脂肪瘤型、术后瘢痕粘连型、脊髓纵裂型.全部病例采取显微手术治疗,对不同类型TCS患儿的临床表现,手术方式及手术疗效进行分析.结果 术后186例获随访3-36个月,采用脊柱裂神经功能量表进行评分,评价不同类型TCS患儿手术前后下肢感觉、运动、反射和大小便功能,并分析影响手术效果和预后的因素.结果表明,本组TCS患儿的术后总有效率为75.2％.不同类型TCS有效率分别为:终丝牵拉型90％,脊膜脊髓膨出型84％,脂肪瘤型65％,瘫痕粘连型75％,脊髓纵裂型77％.结论 TCS一旦确诊,应及早采取显微手术治疗.影响TCS的手术疗效及预后的因素主要是脊髓栓系的类型,手术时机、症状的严重程度、神经松解是否彻底,再栓系的发生.对TCS进行适当的临床分型,有助于指导临床治疗及判断预后.     

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.