脊髓黏液乳头型室管膜瘤的临床特点和治疗(附11例报告)

目的 探讨脊髓粘液乳头型室管膜瘤的临床特点和治疗方法.方法 回顾性分析北京天坛医院神经外科收治并获得随访的11例原发和复发脊髓黏液乳头型室管膜瘤患者的临床特点、治疗和预后.结果 本组11例患者共接受17次手术,首次手术全切3例,近全切除6例,大部切除1例,部分切除1例.术后接受放疗7例,同时接受化疗2例.术后平均随访38.1个月,无死亡病例,原位复发2例,播散转移2例,再次手术切除.结论 脊髓黏液乳头型室管膜瘤容易复发和播散转移,最有效的治疗方法是完整切除肿瘤.无法全切除病变建议放射治疗.     

脊髓黏液乳头型室管膜瘤的诊断与治疗

目的探讨脊髓黏液乳头型室管膜瘤(spinal myxopapillary ependymoma,MPE)的诊断与治疗效果。方法回顾性分析9例脊髓MPE病人的临床资料,均经后正中入路手术切除肿瘤。结果肿瘤全切除8例;次全切除1例,术后行放射治疗。术后病理证实均为脊髓MPE (WHOⅠ级)。随访9例,时间3～60个月;1例出现局部转移,但无临床症状,继续随访;余8例病人无复发。结论脊髓MPE是临床少见的室管膜瘤类型,手术全切肿瘤是病人获得良好预后的主要因素,术中神经电生理监护和术中超声的运用可提高手术的安全性及全切率,无法全切的病人建议术后放疗。     

黏液乳头型室管膜瘤9例临床病理分析

目的探讨黏液乳头型室管膜瘤的临床病理特征、鉴别诊断及治疗与预后。方法回顾性分析9例黏液乳头型室管膜瘤病人的临床资料,行组织学观察及免疫组织化学分析。结果 9例肿瘤均发生于脊髓圆锥、马尾终丝区,典型病理形态为立方形或梭形肿瘤细胞,以乳头放射状结构排列在血管黏液样间质轴心周围,大量黏液聚集在血管和瘤细胞之间或微囊内,伴血管壁增厚、玻璃样变;瘤细胞大小、形态较一致,核分裂像少见或无。免疫组织化学显示肿瘤细胞胶质纤维酸性蛋白(GFAP)、vimentin及S-100阳性,细胞角蛋白(CK)及上皮膜抗原(EMA)阴性。病人均手术全切,术后辅助放疗2例。6例随访17个月～5年,MRI复查仅1例复发。结论黏液乳头型室管膜瘤特征性发病部位、形态学特征及免疫表型是其诊断和鉴别诊断要点,肿瘤全切后预后良好。     

脊髓室管膜瘤的临床特点及显微手术治疗

目的探讨脊髓室管膜瘤的临床特点、显微手术治疗及预后。方法回顾性分析2013年1月至2016年1月显微手术治疗的35例脊髓室管膜瘤的临床资料。结果术前Mc Cormick临床功能分级Ⅰ级15例,Ⅱ级15例,Ⅲ级3例,Ⅳ级2例。肿瘤主体位于脊髓颈段6例,颈胸交界区3例,胸段10例,腰段16例。35例中,肿瘤全切除33例,次全切除2例。30例出院后随访1年,肿瘤复发2例,再次行手术治疗;术后Mc Cormick临床功能分级Ⅰ级19例,Ⅱ级8例,Ⅲ级2例,Ⅳ级1例。结论在神经电生理监测下应用显微神经外科技术治疗脊髓室管膜瘤,术中熟练运用手术技巧以及肿瘤切除后行椎板复位,可以提高手术治疗效果。     

显微手术治疗脊髓室管膜瘤预后的影响因素分析

目的 探讨显微手术治疗脊髓室管膜瘤的疗效及影响预后的因素。方法 对手术治疗的80例脊髓室管膜瘤的临床资料进行回顾性分析,参照欧洲脊髓病评分（EMS）和McCormick分级评估手术前后脊髓功能;采用多因素Logistic回归分析检验预后影响因素。结果 肿瘤全切除51例,次全切除25例,大部分切除4例。术后平均随访（37.6±19.0）个月,复发5例,均为WHOⅡ级,复发时间为（58.4±43.1）个月。末次随访有49例脊髓功能较术前改善,19例无变化,12例加重。术后McCormick分级:Ⅰ级35例,Ⅱ级22例,Ⅲ级12例,Ⅳ级11例。多因素Logistic回归分析发现,术前存在大小便功能障碍是不良预后独立危险因素,而伴脊髓空洞是预后良好因素。末次随访EMS[（15.01±0.3）分]明显高于术前[（13.55±0.35）分;P<0.001]和术后3 d[（13.20±0.31）分;P<0.001]。结论 脊髓室管膜瘤显微手术治疗后脊髓神经功能在短期内以趋于稳定为主,但从长远来看,显微手术能有效治疗脊髓室管膜瘤。存在大小便功能障碍的室管膜瘤建议及早手术治疗;伴有脊髓空洞的脊髓室管膜瘤手术预后良好。     

电生理监测辅助显微手术治疗圆锥、终丝起源室管膜瘤

目的探讨圆锥、终丝起源室管膜瘤的显微手术技巧及疗效。方法回顾性分析15例圆锥、终丝起源室管膜瘤病人的临床资料,均在神经电生理监测下行显微手术切除肿瘤。结果肿瘤全切除13例,次全切除2例。术后病理证实为室管膜瘤Ⅰ-Ⅲ级。术后发生脑脊液漏1例,皮下积液1例,均经对症治疗后痊愈;无手术相关神经功能障碍;无死亡病例。所有病人术后随访3个月-6年,平均2年;病人神经功能改善11例,改善不明显3例,加重1例。肿瘤复发3例,均再次行显微手术治疗,并行放射治疗。结论圆锥、终丝起源的室管膜瘤与脊髓、马尾神经黏连紧密,肿瘤切除程度及术中是否有脊髓神经损伤是决定病人预后的重要因素。神经电生理监测配合显微手术可有效提高肿瘤全切率,并防止术中神经损伤。     

脊髓室管膜下瘤诊断和显微外科治疗

目的总结脊髓室管膜下瘤的临床特点和治疗方法。方法回顾性分析9例脊髓室管膜下瘤的临床资料,均采用显微外科手术治疗,结合术中所见对诊断和治疗进行分析。结果肿瘤全切除3例,近全切除4例,大部分切除2例。病理检查显示:室管膜下瘤8例,室管膜下瘤含室管膜瘤成分1例。术后肢体肌力不同程度好转7例,减退2例;括约肌障碍恢复正常1例;短期浅、深感觉障碍均较术前略加重;无手术死亡病例。随访12~36个月,非全切病人中肿瘤复发3例。结论脊髓室管膜下瘤少见,手术可缓解病人临床症状,显微镜下全切除是最佳治疗选择。     

包膜外整体切除技术在脊髓室管膜瘤手术中的应用

目的 探讨包膜外整体切除技术在脊髓室管膜瘤手术中的应用效果。方法 回顾性分析2018年9月~2020年2月应用包膜外整体切除技术手术治疗的14例脊髓室管膜瘤的临床资料。采用McCormick分级评估脊髓功能。结果 肿瘤全切除13例,次全切1例。术后随访12个月,未见肿瘤复发。术后7 d,脊髓功能改善4例,稳定7例,恶化3例;术后3个月,脊髓功能改善9例,稳定4例,恶化2例;术后12个月,脊髓功能改善11例,稳定2例,恶化1例。结论 在脊髓室管膜瘤手术中,包膜外整体切除技术,有助于提高肿瘤全切除率、减少肿瘤复发风险。     

四脑室室管膜瘤临床特点及显微外科治疗

目的 总结四脑室室管膜瘤的临床特点及显微外科治疗经验。方法 回顾性分析2012年1月至2015年6月我院显微手术治疗的19例四脑室室管膜瘤患者的临床资料。结果 肿瘤全切除15例(78.9%),次全切除4例(21.1%)。术后发生并发症5例,其中眼球活动受限2例,小脑缄默症1例,颅内感染1例,呼吸困难1例。随访1~38个月,术前症状均改善,未见肿瘤复发。结论 显微外科手术是治疗四脑室室管膜瘤的有效手段,最大限度切除肿瘤、最大程度保留脑干功能以及解除脑脊液梗阻,可获得良好预后。     

显微手术治疗脊髓室管膜瘤20例

目的 总结脊髓室管膜瘤的临床特征及治疗方法,以提高诊治水平。方法 回顾性分析2015年1月至2020年6月显微手术治疗的20例脊髓室管膜瘤的临床资料。结果 20例中,肿瘤全切除13例,次全切除4例;术后复发4例,其中全切除1例,次全切除3例。术后McCormick分级Ⅰ级12例,Ⅱ级5例,Ⅲ级1例,Ⅳ级2例;JOA评分改善率在67%~75%。结论 脊髓室管膜瘤早期手术治疗能够提高肿瘤全切除率、减少术后复发率,应用半椎板切除术有助于保护脊柱稳定性。     

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.     

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.