9例嗅神经母细胞瘤治疗临床分析

目的探讨嗅神经母细胞瘤手术治疗的疗效及放射治疗的意义。方法回顾性分析2001-01—2013-12 9例嗅神经母细胞瘤经手术治疗患者的临床资料,根据Kadish分期,A期1例,B期7例,C期1例,全部患者随访12~84个月。结果经鼻内窥镜进路手术切除A期、B期5例,鼻侧切开进路手术切除B期3例,鼻侧切开加经颅进路手术切除C期1例,术后1例未作放疗,5个月后复发,8例术后加放疗肿瘤无复发。结论对于嗅神经母细胞瘤A期和B期的患者,经鼻内窥镜手术进路和鼻侧切开手术进路的治疗效果相当,鼻内窥镜手术具有术中视野清晰、局部组织损伤轻微、术后手术创面恢复较快等优势,是治疗鼻腔鼻窦嗅神经母细胞瘤的最有效手术方法,术后配合放疗,疗效更显著。     

经鼻旁——改良翼点入路切除嗅神经母细胞瘤

目的 通过对本组手术方法和入路的探讨,旨在提高嗅神经母细胞瘤的手术疗效。方法 4例嗅神经母细胞瘤患者均先由耳鼻喉科经鼻旁切口入路切除鼻腔及副鼻窦内肿瘤,接着由神经外科医生经改良翼点入路行开颅肿瘤切除及颅底重建。结果 本组4例无手术死亡,其中肿瘤全切除3例,次全切除1例(副鼻窦内残存)。随访3年,3例存活,1例术后死于急性脑梗塞。结论 神经外科、耳鼻喉科联合经鼻旁—改良冀点入路切除嗅神经母细胞瘤是首选治疗手段,术后辅以放疗有预防复发作用。     

脊髓血管母细胞瘤的诊断和显微手术治疗

目的探讨脊髓血管母细胞瘤的诊断和显微手术治疗技巧.方法回顾性分析11例脊髓血管母细胞瘤病人的临床特征、影像学诊断、治疗方法和预后,均行显微外科手术切除肿瘤.结果肿瘤均全切除.病人平均住院15d,术后短时间内症状改善8例,无变化3例;无手术死亡病例.8例随访6～36个月,无肿瘤复发,神经功能均获得不同程度改善.结论MRI对脊髓血管母细胞瘤的诊断是必要的.熟练的显微外科手术技术和丰富的髓内肿瘤切除经验对手术成功非常关键.     

脊髓血管母细胞瘤的显微手术治疗

目的总结脊髓血管母细胞瘤的治疗经验及疗效。方法回顾性分析38例脊髓血管母细胞瘤病人的手术经验。均在显微镜下行肿瘤切除,术前及术后1周采用McCormick分级进行脊髓功能评估。结果肿瘤全切除37例,近全切除1例。术后运动及感觉障碍改善30例,无变化6例,加重2例(1个月内恢复)。无手术死亡病例。随访3～60个月,脊髓功能改善2例,无明显变化36例;未见肿瘤复发。结论血管母细胞瘤为高度血管化的良性髓内肿瘤,可通过显微镜下全切除治愈。     

显微镜开颅联合内镜经鼻入路手术切除颅内侵袭性嗅神经母细胞瘤疗效分析

目的总结颅内侵袭性嗅神经母细胞瘤的临床特点、手术策略和治疗效果。方法纳入2005年1月至2020年12月在首都医科大学附属北京同仁医院诊断与治疗的24例颅内侵袭性嗅神经母细胞瘤患者,均行双侧扩大经基底入路联合内镜经鼻入路手术+颅底重建术。结果 24例患者肿瘤全切除22例(91.67%)、近全切除2例(8.33%),均经术后病理证实为嗅神经母细胞瘤。术后2例(8.33%)发生短暂性脑脊液漏,2例(8.33%)出现颅内感染,均经对症治疗治愈;1例视力下降加重,2例眼动障碍加重。20例完成随访,平均随访54.60个月,均未发生远期手术相关并发症,12例(60%)肿瘤复发,5年生存率为45%(9/20)。结论扩大经基底入路联合内镜经鼻入路手术切除颅内侵袭性嗅神经母细胞瘤安全、有效,术者应同时具备显微神经外科和神经内镜技术,值得临床推广。     

侵袭颅内嗅神经母细胞瘤

目的 探讨侵袭颅内嗅神经母细胞瘤临床特点及治疗方法。方法 对我院神经外科从2001年7月．2005年8月收治的5例侵袭颅内嗅神经母细胞瘤病人的临床表现、影像学特点、病理学特点，手术治疗进行分析，1例行内镜下经鼻活检，4例手术治疗，分别选择经额入路和经额扩展入路，应用不同方法进行颅底重建。全部病人术后行放疗，1例辅助化疗。术后6-45个月进行随访。结果 4例侵袭颅内肿瘤全部切除，术后鼻塞全部改善，3例术前视力下降者术后视力均有一定程度的改善，术后均未发生脑脊液漏，2例术后出现腰骶段椎管内转移，分别为术后6-8个月。其中1例手术，死亡1例。结论 侵袭颅内的嗅神经母细胞瘤治疗仍然需要多手段进行干预，对侵袭到颅内肿瘤要争取全切除，术后应辅以放疗。     

57例髓母细胞瘤的临床回顾性分析

目的 研究髓母细胞瘤的临床特点及显微外科手术治疗的I临床疗效. 方法 回顾性分析经显微手术治疗的57例髓母细胞瘤患者的临床和随访资料.对髓母细胞瘤的显微外科手术技巧进行探讨. 结果 肿瘤全切除42例,近全切除13例.部分切除2例.全部病例均打通中脑导水管.术前有脑积水者43例,术后手术疗效明显,脑积水减少至16例.且较术前均有不同程度改善,其中10例术后需行分流术.术后两年内肿瘤复发19例,中枢神经系统种植转移8例.最早术后20 d肿瘤复发.术后2年生存率68.4%(截至2006年7月病例),术后5年生存率49.1%(截至2003年7月病例). 结论 应用显微外科手术全切除肿瘤和术后全中枢轴放疗可使髓母细胞瘤患者取得较好的临床疗效.     

颅内嗅神经母细胞瘤（附10例报告）

目的 :研究颅内嗅神经母细胞瘤的临床。方法 :回顾性分析经手术病理证实的颅内嗅神经母细胞瘤 10例。结果 :本组患者平均年龄 3 5 5岁 ,平均病程 10个月 ,首发症状以鼻衄、鼻塞为主 ,头颅CT、MRI显示肿瘤大多位于前颅底 ,有骨质破坏及颅内外沟通。术后 8例随访 2个月～ 4年 ,存活 3例 ,死亡 5例。结论 :颅内嗅神经母细胞瘤极为罕见 ,多伴有鼻部症状 ,常发生于以嗅沟为中心的前颅底 ,一般预后差。手术全切除外加放疗、化疗有助于预后。     

经颈前入路切除颈髓腹侧血管母细胞瘤1例

脊髓腹侧血管母细胞瘤少见, 经颈前入路切除脊髓腹侧血管母细胞瘤鲜见报道。本文回顾性报道1例清华大学附属北京清华长庚医院神经外科于2018年10月采用颈前入路手术治疗的颈髓腹侧血管母细胞瘤患者。术中采用O-arm神经导航系统扫描、神经电生理监测以及吲哚菁绿荧光造影技术进行辅助, 行C6椎体次全切除+脊髓髓内肿瘤切除术。术后颈椎MRI和CT显示血管母细胞瘤全切除。术后34个月随访, 患者肿瘤无复发且神经功能明显改善。     

成人髓母细胞瘤的诊断和治疗

目的 探讨成人髓母细胞瘤的临床特点、诊断和治疗方法.方法 回顾性分析我院2000 年8月至2008 年12 月期间收治的16 例成人髓母细胞瘤的临床及影像学表现、治疗措施与效果.结果 肿瘤全切除12 例,次全切除3 例,部分切除1 例,术后均行放疗,3 例同时行化疗.术后复发3 例,术后2 年生存率86.7%,5 年生存率66.7%.结论 成人髓母细胞瘤发生于后颅窝中线区者多见.手术切除及术后全脑脊髓放疗是主要治疗方法.复发者预后差.     

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.