血管网状细胞瘤的CT及MRI诊断

目的探讨血管母细胞瘤CT及MRI影像学表现特征,提高其影像检查诊断率。方法回顾分析13例经CT及MRI检查及手术病理证实的血管网状细胞瘤病例。结果位于小脑半球11例,小脑蚓部2例。11例表现为囊实性,大囊小结节,瘤壁结节大小在0.5~2 cm,1例为囊性,1例为实性。增强后肿瘤实性部分会明显强化。结论血管网状细胞瘤的CT及MRI影像学表现具有特征性,综合影像分析有助于对该病诊断。     

脑血管母细胞瘤的诊断和治疗

目的：探讨脑血管母细胞瘤的诊断和治疗。方法：对23例经手术及病理证实的脑血管母细胞瘤的临床表现、CT、MRI、手术疗效进行回顾性分析。结果：行肿瘤或瘤结节全切除术21例，大部分切除2例，死亡2例。结论：实体性血管母细胞瘤仍是神经外科较难处理 的肿瘤，完整地切除肿瘤或瘤结节是提高肿瘤治愈率，降低术后复发率的关键。     

小脑血管网织细胞瘤MRI诊断与鉴别诊断

目的探讨小脑血管网织细胞瘤MRI表现特点,总结其诊断和鉴别诊断要点。方法回顾分析经手术病理证实的30例小脑血管网织细胞瘤MRI表现。结果30例中发生于右侧小脑半球15例,左侧13例。双侧1例,小脑蚓部1例。其中囊性肿瘤26例．实质性4例。肿瘤最大径25～60mm。26例囊性肿瘤中25例平扫表现为圆形或类圆形脑脊液样长T1、长T2信号,1例肿瘤合并出血在T1WI及T2WI均呈明显高信号;23例可见壁结节,直径3～18mm;增强扫描所有病变囊壁和囊液均不强化,24例显示强化壁结节;4例实质性肿瘤呈稍长T1、长T2信号,增强扫描均明显强化。23例肿瘤边缘或内部可见1～2条流空血管影。结论囊性小脑血管网织细胞瘤MRI表现颇具诊断特征性,但有时需与小脑星形细胞瘤鉴别,实质性肿瘤需注意与转移瘤、髓母细胞瘤及脑膜瘤鉴别。     

颅内血管母细胞瘤的诊断及显微手术治疗（附34例分析）

且的探讨颅内血管母细胞瘤的诊断和显微手术治疗疗效。方法回顾性分析34例血管母细胞瘤病人的病历资料。肿瘤位于小脑半球27例，脑干区3例，幕上4例。囊性结节样肿瘤31例，实性肿瘤3例。家族性发病3例，诊断为von Hippel-Lindau综合征：复发性2例。均行显微手术治疗，采用神经导航辅助手术10例，锁孔显微手术1例。结果肿瘤全切除30例，次全切除4例。术后重残1例，死亡1例。随访2～10年，2例肿瘤全切除后分别于术后1年、6年复发。结论CT、MRI是诊断血管母细胞瘤的有效检查方法。神经导航对减少术中出血及完整切除瘤结节有较好作用。显微手术是治疗颅内血管母细胞瘤的较佳方案。     

12例小脑血管网织细胞瘤诊治体会

目的探讨小脑血管网织细胞瘤的诊断和治疗。方法回顾分析12例血管网织细胞瘤患者的临床特征、影像学表现和治疗方法。结果11例囊性肿瘤行瘤结节全切，1例实体性肿瘤行全切除。术后无并发症。结论CT及MRI对小脑血管网织细胞瘤诊断具有特异性，手术治疗效果好。瘤结节和瘤体的完整切除是手术成功的关键。     

小脑海绵状血管瘤（18例分析）

目的 :研究小脑海绵状血管瘤的临床。方法 :回顾性分析经手术病理证实的小脑ICCA 18例。结果 :18例共 19个病灶 ,单发17例 ,多发 1例 2个病灶。 11例表现为小脑自发性出血 ,7例表现为颅高压及小脑症状。 15例行头颅CT检查 ,诊断小脑ICCA 2例 ,2个病灶 ;头颅MRI检查 16例 ,诊断小脑ICCA 15例 ,16个病灶。 3个病灶为囊性变。 18例均行手术治疗 ,其中 2例急诊手术清除血肿并全切除病灶。手术无死亡 ,疗效满意。结论 :自发性出血是本病的主要症状。MRI具有较高的诊断价值。首选手术切除病灶。囊性小脑ICCA可能为实质性ICCA内部慢性出血的结果。     

神经导航辅助下显微手术切除血管母细胞瘤

目的 探讨神经导航辅助下显微手术切除血管母细胞瘤的治疗效果。方法 回顾性分析2011年10月至2013年12月神经导航辅助下显微手术治疗的26例血管母细胞瘤的临床资料。结果 21例囊性血管母细胞瘤瘤结节全切除;5例实质性血管母细胞瘤中,3例全切除,2例次全切除,术后辅以γ-刀治疗。术后随访6个月~1年,治愈24例,21例行γ-刀治疗者肿瘤未见增大迹象,无死亡病例。结论 神经导航辅助下显微手术切除血管母细胞瘤,创伤更小,对于实质性血管母细胞瘤及瘤结节位于浅表的囊性血管母细胞瘤,神经导航均可准确定位,疗效确切。     

小脑血管母细胞瘤的MRI分析

目的 探讨血管母细胞瘤的MRI影像学特点,提高对该病的认识.方法 回顾性分析经手术病理证实的9例小脑血管母细胞瘤的MRI资料.结果 9例病灶,4例位于右侧小脑半球,3例位于左侧小脑半球,2例位于小脑蚓部.MRI表现为大囊小结节型的8例,壁结节明显强化;表现为实质肿块型1例,增强明显强化.5例肿瘤实质部分见血管流空信号影...     

右小脑血管母细胞瘤合并右侧侧脑室中枢神经细胞瘤1例

正1临床资料病人,女,42岁,因头痛2个月入院。入院时体格检查未发现明显神经系统阳性体征。头颅MRI示:右侧脑室可见一T_1WI呈中等稍低信号,T_2WI呈中低混杂信号影,增强后呈显著不均匀强化;右小脑见囊性病灶,内有结节,考虑右小脑血管母细胞瘤并右侧脑室肿瘤。完善术前准备,首先取左侧卧位行枕下正中入路右小脑肿瘤切除术,术中发现典型囊性小脑血管母细胞瘤改变,呈大囊小结节,内有黄色囊液,完整切     

后颅窝血管母细胞瘤的显微手术治疗

目的 探讨后颅窝血管母细胞瘤的诊断及手术治疗方法。方法 回顾性分析我科2003—2013年收治的37例经手术及病理证实的后颅窝血管母细胞瘤。结果 发病年龄10~62岁,囊实性28例,实质性9例,所有病例术前均行MRI检查。囊实性肿瘤全切除28例,无死亡病例;实质性肿瘤全切8例,部分切除1例,死亡1例。结论 显微外科手术是后颅窝血管母细胞瘤最重要的治疗手段。     

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.     

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.     

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.     

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.     

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.     

Anticonvulsant Drugs and Cognitive Function: A Review of the Literature

Summary: Alterations of cognitive function are separate from disturbances of behavior seen in association with epilepsy. The nature of the cognitive disability may to a certain extent depend on the seizure type. Partial seizures, mainly derived from a temporal lobe focus, impair memory tasks, while generalized seizures seem to have more effect on attentional abilities. A number of studies, reviewed in this paper, suggest that anticonvulsant drugs further impair cognitive function. Maximal impairments are seen in patients receiving polytherapy: rationalization of polytherapy improves cognitive abilities. Studies in children and adults have allowed differentiation of the effects of various commonly used antiepileptic agents. Maximal cognitive deficits are seen with. phenytoin, while phenobarbital and sodium valproate induce moderate disturbances, and carbamazepine seems relatively free from such toxicity. Further research is needed on the interrelationship between types of seizure disorders, types of anticonvulsant medications, and cognitive function.