椎管内肠源性囊肿

目的探讨椎管内肠源性囊肿的临床特点和外科治疗。方法回顾性分析2004年1月-2009年1月收治的12例椎管内肠源性囊肿患者的临床表现、影像学特征及治疗效果。本组男8例,女4例。囊肿位于颈段6例,胸段3例,腰段3例。结果全部病例均行显微手术治疗,术中全切8例,次全切2例,部分切除2例。术后除1例患者早期肌力下降外其余患者神经功能均明显改善。随访6月～5年（平均18个月）,8例全切者无复发,4例次全或部分切除者2例复发。结论椎管内肠源性囊肿为先天性良性疾病,早期显微手术切除可获得良好效果。     

儿童椎管内肠源性囊肿(附五例报告)

目的 探讨儿童椎管内肠源性囊肿的临床表现，影像学检查及显微手术治疗。方法 收集自1996年至2004年收治的5例椎管内肠源性囊肿的临床资料，结合文献加以回顾性分析。结果 全部病例均为儿童，确诊后采用显微外科手术治疗，其中2例全切，2例次全切除，1例大部切除，无一例死亡。经病理报告证实均为肠源性囊肿，术后神经根痛症状基本消失。随访0.5-2年，无一例复发。脊髓损伤的Frankel分级，E级4例，D级1例。结论 儿童椎管内肠源性囊肿有典型的临床表现和MRI特征，其最佳治疗方法是早期确诊并采取显微外科手术方法切除。     

椎管内肠源性囊肿临床分析(附5例报告)

目的探讨椎管内肠源性囊肿的临床特点、诊断和治疗。方法回顾性分析1998年至2010年收治的5例椎管内肠源性囊肿病人的临床资料并结合相关文献加以分析讨论。5例患者均有不同程度运动障碍,4例出现神经根性疼痛,3例出现浅感觉异常。结果所有患者临床症状术后均有所改善。手术全切囊肿3例,次全切除和部分切除各1例。5例病人术后病理学证实为肠源性囊肿。5例病人术后随访1～5年均未见复发。结论椎管内肠源性囊肿术前诊断有一定困难,但是一旦确诊,早期手术治疗是最佳选择。     

椎管内肠源性囊肿的手术治疗

目的 分析总结椎管内肠源性囊肿的手术治疗方法和操作技巧.方法 通过回顾性分析总结华山医院神经外科2006年1月至2010年11月收治的20例椎管肠源性囊肿的手术入路、操作方法及手术疗效.结果 20例患者中,18例获得囊肿全切除,2例(其中1例为复发病例)获大部切除;19例患者术后症状体征不同程度改善,1例出现一侧下肢肌力减退;17例患者获得临床随访,平均随访31.2个月.随访患者中16例症状体征不同程度改善,15例囊肿全切除患者MRI复查未见囊肿复发,2例囊肿大部切除病例见囊肿复发.结论 适当的手术入路和正确的操作方法,对于全切除、彻底治愈椎管内肠源性囊肿非常重要.     

椎管内先天性肿瘤的诊断与治疗

目的分析椎管内先天性肿瘤的临床特点、诊断及治疗方法。方法回顾分析了2002年1月至2006年4月间在我院手术治疗的68例椎管内先天性肿瘤患者的临床资料,所有患者均行显微手术治疗,随访54例。结果肿瘤全切18例,大部分切除40例,部分切除10例。表皮样囊肿22例,脂肪瘤20例,畸胎瘤14例,皮样囊肿8例,肠源性囊肿4例。随访2～6年,平均4.3年,症状明显改善42例,没有变化10例,恶化2例,无患者死亡。结论先天性肿瘤多为良性病变,好发于青少年。术前MRI及CT分析和显微手术是治疗的关键。     

椎管内肠源性囊肿11例临床分析

目的　对椎管内肠源性囊肿的临床表现 ,影像学特征及治疗方法进行探讨。方法　分析和总结我科 1994年至 2 0 0 1年收治的 11例椎管内肠源性囊肿的临床特点、影像学资料及手术方法。结果　 11例病人中 ,10例髓外硬膜下者 ,均一次性手术全切除 ;1例髓内患者 ,行大部分切除。结论　椎管内肠源性囊肿术前定性诊断困难 ,CT及MRI有助于定位诊断。手术全切除是唯一的根治方法     

椎管内肠源性囊肿的诊断与治疗(附29例报告)

目的 探讨椎管内肠源性囊肿的临床表现、MRI特征及治疗方法.方法 回顾性分析29例经手术及病理证实的椎管内肠源性囊肿患者的临床资料、影像学资料.结果 29例病人中,全切除10例,近全切除15例,大部分切除2例,部分切除2例,术后所有病人均恢复良好.结论 椎管内肠源性囊肿是一种少见的先天性疾病,MRI检查是首选的检查手段,手术切除是惟一有效的治疗方法.     

椎管内肠源性囊肿诊治体会(附12例报告)

目的探讨椎管内肠源性和气管源性囊肿的诊断及显微外科治疗的效果。方法回顾性分析经手术及病理证实的10例椎管内肠源性和2例气管源性囊肿患者的临床资料。结果该12例椎管内囊肿均常规行脊柱X线及MRI检查,术前无1例确诊,术中仅1例高度怀疑,术后病理证实肠源性囊肿Ⅰ型7例、Ⅱ型3例,支气管源性囊肿Ⅱ型2例;12例中6例全切,4例大部分切除,2例行囊壁部分切除+囊腔-蛛网膜下腔沟通术。结论①椎管内肠源性囊肿术前诊断较困难,脊柱MRI是其首选辅助检查,确诊有赖于病理诊断;②对于椎管内囊性病变术前均应考虑肠源性囊肿的可能,术中应留取标本进行组织病理学检查;③椎管内肠源性和气管源性囊肿手术应在保留脊髓功能的前提下尽可能全切囊壁,大多数患者预后良好。     

成人椎管内先天性肿瘤的手术治疗

目的 探讨成人椎管内先天性肿瘤的临床特点及手术治疗方法。方法 回顾性分析2006年1月至2014年12月手术治疗的48例成人椎管内先天性肿瘤的临床资料。结果 肿瘤全切除11例,大部分切除21例,部分切除16例;无手术死亡病例。术后病理结果显示（表）皮样囊肿20例,脂肪瘤13例,畸胎瘤10例,支气管源性囊肿3例,肠源性囊肿2例。43例术后随访1~6年,平均2.8年;2例皮样囊肿复发,二次手术切除;25例症状改善,15例稳定,3例恶化。结论 对成人椎管内先天性肿瘤,手术不能强求肿瘤的全切,需尽量保护神经功能和维护脊柱稳定。     

椎管内肠源性囊肿的诊断与显微外科治疗

目的 总结椎管内肠源性囊肿的诊断及治疗的经验.方法 回顾性分析11例椎管内肠源性囊肿的临床表现、MRI特点和手术结果.结果 11例均有不同程度的肢体活动障碍,部分伴有脊柱发育畸形,主要的临床表现为神经根性痛和脊髓压迫症状.MRI可清晰显示囊肿及其周围结构.囊肿通常表现为稍长T1、长T2均匀信号.11例中4例全切,7例大部切除.所有病例术后神经功能恢复良好.结论 肠源性囊肿是少见的先天性疾患,多发生于颈胸段椎管硬膜下脊髓腹侧,常伴有脊椎畸形.MRI是最方便有效的检查方法.手术切除囊肿,神经功能多能恢复.     

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.     

Diagnostic Difficulties and Treatment Implications

Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.     

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.     

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.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.     

Treatment Considerations in Anticonvulsant Monotherapy

Summary: The long-standing practice of polypharmacy in treating epilepsy is giving way to use of monotherapy. Monotherapy can improve seizure control as well as reduce the risk of serious idiosyncratic reactions, dose-related side effects, and complex drug interactions. Monotherapy also offers improved compliance and cost-effectiveness. The basis of monotherapy is accurate diagnosis and assessment of the patient's seizure type(s), followed by selection of a single appropriate anticonvulsant drug. Many patients currently treated with multiple anticonvulsants can be successfully converted to monotherapy with a carefully monitored program in which troublesome and redundant drugs are gradually withdrawn from the therapeutic regimen.