应用C1-2螺钉棒系统行后路复位、固定和融合治疗合并寰-枢椎脱位的颅底凹陷症

目的探讨应用C1-2螺钉棒内固定系统行后路复位、固定和融合治疗寰枢椎脱位的手术疗效。方法 2013年4月至2013年10月,对30例我科收治的合并寰枢椎脱位的颅底凹陷症患者采用寰椎侧块螺钉和枢椎椎弓根峡部螺钉(或下关节突螺钉、颈3椎弓根螺钉)棒内固定系统进行复位、固定并取髂后上嵴松质骨植骨融合。通过术后3D-CT评判复位程度,JOA评分评判临床疗效,并探讨影响手术效果的因素。结果 30例患者中26例达到完全复位,4例为部分复位。其中25例完成了3个月以上随访,CT显示植骨愈合良好,未出现植骨的吸收及内固定的松动。结论 C1-2椎弓根钉棒内固定系统对治疗合并寰枢椎脱位的颅颈交界区畸形可以获得满意的疗效,安全可行。     

Goel技术治疗颅底凹陷及寰枢椎脱位

目的 探讨应用寰椎侧块螺钉、枢椎椎弓峡部螺钉棒内固定术治疗颅底凹陷合并寰枢椎脱位的可行性及临床疗效.方法 回顾分析76 例颅底凹陷合并寰枢椎关节脱位患者临床诊断与治疗经过,其中合并寰椎枕骨化畸形的寰枢椎脱位50 例,未合并寰椎枕骨化畸形的寰枢椎失稳和脱位26 例(齿状突不连性或发育不良性寰枢椎脱位14 例、未合并脱位的颅底凹陷经前路切除齿状突减压所致医源性寰枢椎不稳12 例).全部病例均采用寰椎侧块螺钉和枢椎椎弓峡部螺钉棒或下关节突螺钉棒系统进行复位固定,髂后上嵴松质骨颗粒植骨.结果 74 例获得满意临床治疗效果,出院时日本骨科协会(JOA)评分(17 分法)由术前的9.43 ± 3.16 提高至13.80 ± 2.07(t = 4.063,P = 0.037),Odom 评级优19 例、良49 例、可7 例、差1 例.未合并寰枕融合者经后路固定手术均获得解剖学复位;50 例伴寰枕融合患者中15 例完全复位、35 例部分复位;其中26 例经口腔入路施行减压.共50 例获3 个月以上随访,JOA 评分由术前的8.90 ± 1.22 提高至14.72 ± 1.57(t = 4.914,P = 0.015),Odom 评级优18 例、良30 例、可2 例、差0 例.随访期间未出现断钉、断板现象,内固定稳固、植骨完全融合.1 例术后清醒拔管12 h 突发呼吸、心跳停止,复苏成功后深度昏迷,家属放弃治疗出院;1 例术后第6 天发生全身凝血机制障碍,随后出现四肢完全瘫痪,目前仍然在康复治疗中,肌力恢复至3 级;2 例术后发生呼吸衰竭;2 例出现切口延迟愈合.无一例发生感染和后组脑神经损伤并发症.结论 应用Goel 内固定技术行寰枢椎关节复位、固定及植骨融合治疗畸形寰枢椎脱位安全可行,疗效满意.     

自发性寰枢关节脱位后路复位内固定术中枢椎椎弓根螺钉置入不能时的备选方法

目的探讨自发性寰枢关节脱位后路内固定过程中枢椎椎弓根螺钉置入不能时,其他备选螺钉内固定技术的安全性及有效性。方法对贵州省人民医院神经外科未采用枢椎椎弓根螺钉内固定治疗的11例自发性寰枢关节脱位患者的临床资料进行回顾性分析。在枢椎椎弓根螺钉置入不能时,采用枢椎椎板螺钉、峡部螺钉、枢椎下关节突螺钉及延长固定节段至C3侧块螺钉来增加稳定性的方法。手术前后分别行CT及MRI检查,评价脊髓受压程度、脱位复位情况、螺钉位置、骨融合情况;通过比较术前、术后日本骨科协会(JOA)评分来评价疗效。结果 11例患者均为枢椎椎弓根置钉不能,改用备选方法置钉,全部行枕颈钉棒内固定。共置入枢椎椎板锣钉14枚,枢椎峡部螺钉5枚,枢椎下关节突螺钉1枚,延长固定节段至C3侧块螺钉4枚。术中均未发生椎动脉和脊髓神经根损伤。11例患者的寰枢关节脱位均得到不同程度的复位,随访中无患者出现螺钉松动、滑脱、断钉及复位丢失等情况,JOA评分为显著增加。结论对自发性寰枢关节脱位后路内固定过程中枢椎椎弓根螺钉置入不能时,可根据情况,个性化选用枢椎椎板螺钉、峡部螺钉、枢椎下关节突螺钉及延长固定节段至C3侧块螺钉的方法来固定,是可行且有效的。     

应用改良Goel技术手术治疗合并寰枕融合的寰枢椎脱位

目的探讨应用改良Goel技术治疗合并寰枕融合的寰枢椎脱位的临床疗效。方法回顾分析2013年12月~2014年7月间解放军总医院神经外科用改良Goel技术手术治疗的58例合并寰枕融合的寰枢椎脱位患者的临床资料。结果 57例患者得到3~12个月以上的随访。随访患者功能评价(Odom评级):优23例,良32例,可2例,差1例(术后出现严重并发症放弃治疗)。后组颅神经功能障碍和肢体无力均恢复正常。术后影像学复查:完全复位32例,近完全复位26例;小脑下疝及脊髓空洞患者术后3个月复查时均恢复和缩小。随访期间1例患者于出院4个月后发现内固定松动,做了翻修手术;其余患者均未出现断钉、断棒及脱位现象。结论应用改良Goel技术对合并寰枕融合的寰枢椎侧块关节进行复位、固定及植骨融合手术安全可靠,疗效满意。     

齿状突游离所致寰枢椎脱位的外科治疗

目的 总结齿状突游离小骨导致寰枢椎脱位患者的临床治疗经验。方法 回顾分析2004年10月至2010年3月宣武医院神经外科治疗的10例齿状突游离小骨所致寰枢椎脱位患者的临床经验。采用颈后路寰枢椎椎弓根螺钉或枕颈螺钉内固定植骨融合术进行治疗,术前、术后采用JOA评分评价手术疗效。结果 手术平均时间3h,未发生与手术相关并发症,术后8例患者症状改善,2例患者症状无明显变化,术前与术后3个月JOA评分差异有统计学意义。术后3个月复查寰枢椎均达到复位和骨性融合。结论 采用寰枢椎椎弓根螺钉或枕颈螺钉内固定技术治疗齿状突小骨可以有效地进行寰枢椎之间的融合,改善齿状突小骨导致的神经功能症状。     

寰椎侧块-枢椎椎弓根钉棒技术在先天性颅颈交界区畸形翻修手术中的应用

目的 探讨寰椎侧块-枢椎椎弓根螺钉棒复位内固定技术对于已行不当后颅窝减压术的先天性颅颈交界区畸形患者进行翻修手术的可行性及临床疗效。方法 回顾性分析2013年1月—2016年1月中国人民解放军总医院第一医学中心神经外科收治的21例先天性颅颈交界区畸形患者的临床资料。其中18例患者在外院已行后颅窝减压术,3例患者行后颅窝减压术+枕颈内固定术。患者术前及术后均行颅颈交界区3D-CT及MRI检查,评估寰枢椎脱位和上颈髓受压的程度;采用日本骨科协会(JOA)评分标准对患者的临床状况进行评价。所有患者均采用后路寰椎侧块-枢椎椎弓根螺钉-棒技术行寰枢复位内固定,并取髂后上棘松质骨颗粒植骨融合。结果 本组患者的翻修手术均成功实施,术中未出现脊髓、椎动脉损伤。术后20例患者完成了6～24个月,平均12. 2个月的随访。3D-CT复查示,19例患者(90. 5%)获得垂直方向的完全复位,18例患者(85. 7%)获得水平方向的完全复位;植骨均出现融合,未出现钉棒脱落或复位丢失者。MRI复查显示,上颈髓受压均获得缓解。术后3个月的JOA评分从术前的(9. 8±2. 1)分提高到(14. 1±1. 9)分,差异有统计学意义(P 0. 01)。结论 寰椎侧块-枢椎椎弓根螺钉-棒复位内固定技术治疗已行不当后颅窝减压术的先天性颅颈交界区畸形是安全有效且可行的。     

齿状突游离小骨合并寰枢椎脱位的外科治疗临床经验

目的总结齿状突游离小骨合并寰枢椎脱位患者外科治疗的经验。方法回顾性分析唐都医院神经外科2010年8月~2015年12月治疗的9例齿状突游离小骨合并寰枢椎脱位患者的临床资料。所有患者均采用颈后路寰枢椎椎弓根螺钉或者枕颈螺钉内固定植骨融合术进行治疗,统计并比较术前术后影像学测量指标(改良寰齿前间距MADI,脊髓可用间隙SAC)及JOA评分评价手术疗效。结果 9例患者均成功实施手术,无手术相关并发症发生;术后患者的症状均明显改善,MADI明显缩小,SAC明显增加,颈髓角135°;手术前和手术后3个月时的JOA评分比较,差异有统计学意义(P0.05);术后3个月时复查寰枢椎均达到复位和骨性融合。结论对齿状突游离小骨合并寰枢椎脱位患者,采用术中寰枢椎复位,寰枢椎椎弓根螺钉或枕颈螺钉内固定术可以有效进行寰枢椎的融合,固定效果良好,缓解脊髓受压,改善了齿状突游离小骨及寰枢椎脱位导致的神经功能症状。     

单纯后路复位及固定治疗自发性寰枢椎脱位

目的 利用单纯后路复位,同时行内固定治疗寰枢椎脱位的方法,既不需要颅骨牵引,也不需经口腔齿状突切除.方法 2004年5月至2007年12月,收治自发性寰枢椎脱位病人20例,手术前后利用CT及MRI进行影像学测量,评价脱位及脊髓延髓受压程度.根据是否合并寰枕融合分别采用C1侧块～C2椎弓根螺钉技术3例及C2椎弓根～枕骨螺钉技术17例.手术中向前推压C2棘突或通过C2椎弓根及枕骨螺钉间撑开将齿状突向前、下牵拉以恢复齿状突与C1前弓的解剖关系.结果 20例病人随访6-48个月,1例术后1周因基底动脉内血栓形成死亡,其余19例均明显改善.手术后影像学检查见脊髓延髓均获彻底减压,合并脊髓空洞的5例病人,空洞均明显缩小;各项影像学测量指标均明显好转(P<0.01).1例于术后3个月时CT提示复位部分丢失,但螺钉位置良好,脊髓延髓减压良好,脊髓空洞继续缩小,6个月时骨性融合.结论 首先选择后路复位及固定,而不是前路经口腔齿状突切除减压,是治疗寰枢椎脱位简单有效,相对安全的方法.     

单纯后路复位及固定治疗自发性寰枢椎脱位

目的 利用单纯后路复位,同时行内固定治疗寰枢椎脱位的方法,既不需要颅骨牵引,也不需经口腔齿状突切除.方法 2004年5月至2007年12月,收治自发性寰枢椎脱位病人20例,手术前后利用CT及MRI进行影像学测量,评价脱位及脊髓延髓受压程度.根据是否合并寰枕融合分别采用C1侧块～C2椎弓根螺钉技术3例及C2椎弓根～枕骨螺钉技术17例.手术中向前推压C2棘突或通过C2椎弓根及枕骨螺钉间撑开将齿状突向前、下牵拉以恢复齿状突与C1前弓的解剖关系.结果 20例病人随访6-48个月,1例术后1周因基底动脉内血栓形成死亡,其余19例均明显改善.手术后影像学检查见脊髓延髓均获彻底减压,合并脊髓空洞的5例病人,空洞均明显缩小;各项影像学测量指标均明显好转(P<0.01).1例于术后3个月时CT提示复位部分丢失,但螺钉位置良好,脊髓延髓减压良好,脊髓空洞继续缩小,6个月时骨性融合.结论 首先选择后路复位及固定,而不是前路经口腔齿状突切除减压,是治疗寰枢椎脱位简单有效,相对安全的方法.     

单纯后路复位及固定治疗自发性寰枢椎脱位

目的 利用单纯后路复位,同时行内固定治疗寰枢椎脱位的方法,既不需要颅骨牵引,也不需经口腔齿状突切除.方法 2004年5月至2007年12月,收治自发性寰枢椎脱位病人20例,手术前后利用CT及MRI进行影像学测量,评价脱位及脊髓延髓受压程度.根据是否合并寰枕融合分别采用C1侧块～C2椎弓根螺钉技术3例及C2椎弓根～枕骨螺钉技术17例.手术中向前推压C2棘突或通过C2椎弓根及枕骨螺钉间撑开将齿状突向前、下牵拉以恢复齿状突与C1前弓的解剖关系.结果 20例病人随访6-48个月,1例术后1周因基底动脉内血栓形成死亡,其余19例均明显改善.手术后影像学检查见脊髓延髓均获彻底减压,合并脊髓空洞的5例病人,空洞均明显缩小;各项影像学测量指标均明显好转(P<0.01).1例于术后3个月时CT提示复位部分丢失,但螺钉位置良好,脊髓延髓减压良好,脊髓空洞继续缩小,6个月时骨性融合.结论 首先选择后路复位及固定,而不是前路经口腔齿状突切除减压,是治疗寰枢椎脱位简单有效,相对安全的方法.     

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.     

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.