颅内神经节细胞胶质瘤(附8例报告)

目的探讨颅内神经节细胞胶质瘤的临床特征及治疗效果。方法回顾性分析8例颅内神经节细胞胶质瘤的临床资料。结果平均发病年龄27岁,肿瘤位于大脑半球6例,脑干2例。临床表现主要为癫,影像学检查无特异性表现。显微手术全切5例,大部切除3例,放、化疗4例。随访3个月～5年,7例症状明显缓解,1例复发。结论神经节细胞胶质瘤呈相对良性过程,癫为其主要临床表现,治疗上应手术全切;对有术后残留或间变者可给予放、化疗,预后相对良好。     

颞叶癫痫颅内EEG记录定位与海马病理变化的关系

目的探讨颞叶癫痫颅内EEG记录与颞叶海马病变程度的关系。方法视频EEG证实为颞叶癫痫并经MRI检查的病人序贯进入本研究,采用硬膜下电极及深部电极联合纪录,确定领先发作释放(initialictaldischarge,IID)部位。37例病人中,无或轻度海马萎缩(hippocampalatrophy,HA阴性组)27例,中至中度HA(HA阳性组)者10例。海马病理变化依据MRI检查的海马容积测量及术后组织病理学的海马硬化分级。结果本组HA阴性组27例病人中,9例病人IID广泛出现在海马区、内侧旧皮层及外侧新皮层;3例在海马区及内侧古皮层;14例完全出现在海马区以外的颞叶皮层;仅1例局限于海马区。在HA阳性组中,90%的病人IID局限于海马区(P<0.05),在25例低级别海马硬化(HS)中,IID局限于海马区显著低于12例HS高级别病人(P<0.05)。结论颞叶癫痫的抽搐发作放电定位与海马的病理变化存在着密切关系,无HA和低级别HS病人的IID多出现在海马、颞叶内侧皮层、颞叶新皮层的部位,而HA明显者和高级别的HS病人出现的IID往往仅局限于海马(HF)。     

Computed tomographic changes of the brain and clinical outcome of patients with seizures and epilepsy after an ischaemic hemispheric stroke.

It is not well established whether seizures and epilepsy after an ischaemic stroke increase the disability of patients. Seventy-two patients with delayed seizures after a hemispheric infarct (37 with a single seizure and 35 with epilepsy) were included in the study. The modified Rankin scale was used to compare disability of the patients at 1 month after stroke and at 2 weeks after single or the last seizure, in case of epilepsy. The size of the X-ray hypoattenuation zone was compared on computed tomographic (CT) scans, performed in the weeks after the stroke and 1 week after single or repeated seizures. Lesion size was determined by superimposing the CT slices on digital cerebral vascular maps, on which the contours of the infarct area were delineated. The extent of the infarcts was expressed as the percentage fraction of the total surface area of the cerebral hemisphere. Groups with a single seizure and with epilepsy were mutually compared. Infarcts predominated in the parieto-temporal cortical regions. In the overall group the median Rankin score worsened significantly after seizures. The average size of the X-ray hypoattenuation zone was also significantly increased on the CT scans after the seizures, compared with those after stroke, without clear evidence of recent infarction. Mutual comparison of patients with a single seizure episode and of those with epilepsy showed only a trend of more severe disability and of increase in lesion size in the post-stroke epilepsy group. Delayed seizures and epilepsy after ischaemic stroke are accompanied by an increase in lesion size on CT and by worsening of the disability of the patients. This study does not allow to determine whether this is due to stroke recurrence or due to additional damage as a result of the seizures themselves.     

外伤后癫痫大鼠模型脑内铁蛋白与转铁蛋白含量的动态变化研究

目的:从脑内铁代谢的角度研究铁在外伤后癫(PTE)发病机制中的可能作用。方法:用ELISA方法检测PTE大鼠模型脑组织内铁蛋白(Ft)和转铁蛋白(Tf)的动态含量。结果:早期Ft在整个脑组织内呈现为即刻反应性升高,15d后其含量在实验对侧脑组织内恢复至正常范围,而在实验侧脑组织内呈现继续升高的趋势;Tf含量只在实验侧脑组织内升高,且呈现随时间继续升高趋势。结论:PTE大鼠模型中的铁代谢过程十分复杂,相关蛋白尤其是Tf含量的增加提示铁可能是外伤后癫灶形成机制的一部分。     

Treatment of Status Epilepticus in Adults: Guidelines of the Italian League Against Epilepsy

Summary:  Status epilepticus (SE) is a medical emergency which can lead to significant morbidity and mortality and requires prompt diagnosis and treatment. SE is differentiated into generalized or partial SE on the basis of its electro-clinical manifestations. The guidelines for the management of SE produced by the Italian League against Epilepsy also distinguish three different stages of SE (initial, established and refractory), based on time elapsed since the onset of the condition and responsiveness to previously administered drugs. Treatment should be started as soon as possible, particularly in generalized convulsive SE, and should include general support measures, drugs to suppress epileptic activity and, whenever possible, treatments aimed at relieving the underlying (causative) condition. Benzodiazepines are the first line antiepileptic agents, and i.v. lorazepam is generally preferred because it is associated with a lower risk of early relapses. If benzodiazepines fail to control seizures, i.v. phenytoin is usually indicated, though i.v. phenobarbital or i.v. valproate may also be considered. Refractory SE requires admission to an intensive care unit (ICU) to allow adequate monitoring and support of respiratory, metabolic and hemodynamic functions and cerebral electrical activity. In refractory SE, general anesthesia may be required. Propofol and thiopental represent first line agents in this setting, after careful assessment of potential risks and benefits.     

Epileptic seizures,arthrogryposis, and migrational brain disorders: a syndrome?

Introduction – Arthrogryposis multiplex congenita (AMC) may be associated with multiple developmental defects. In some severely affected newborns with AMC, autopsy studies have suggested a common mechanism of malmigration at the spinal and cerebral levels. To our knowledge, a constellation of arthrogryposis, epileptic seizures, and brain migrational anomalies in adult patients has not previously been described in a clinical material. Material and methods – Six consecutive adult patients with arthrogryposis multiplex congenita and epileptic seizures form the basis of the present study. Five patients had joint contractures and reduced muscle volume restricted to the lower extremities, whereas one patient had predominantly upper extremity affection. They were studied with magnetic resonance imaging (MRI), EEG, EMG, a neuropsychological test battery, and chromosome analysis. Results – Four of them had clear evidence of migrational brain disorders, demonstrated by MRI, in three of them roughly corresponding to the focal epileptiform EEG activity. Five of the patients had partial seizures, whereas one only had generalized tonic-clonic seizures. The MRI findings included polymicrogyria, pachygyria, and fused schizencephaly. Four had neurogenic EMG changes, one had myopathic EMG features, and one had an unremarkable EMG pattern in affected muscles. All patients witL demonstrable migrational disorders showed abnormal neuropsychological features. Three patients were mentally retarded. A chromosome abnormality in the form of a ring chromosome 18 was present in one patient. Conclusion – We suggest that AMC, epileptic seizures, and migrational brain disorders may form the integral parts of a hitherto undescribed syndrome in adults. A wide-spread defect in neuronal migration along the entire neural axis may be the underlying mechanism of the cerebral and the peripheral symptoms.     

Molecular Pharmacology of Topiramate: Managing Seizures and Preventing Migraine

Topiramate is a neuromodulatory compound with stabilizing properties that was initially introduced for the management of partial seizures. Topiramate has been demonstrated to modify several receptor-gated and voltage-sensitive ion channels, including voltage-activated Na⁺ and Ca²⁺ channels and non-NMDA receptors. These receptors have been implicated in the pathophysiology of both epilepsy and migraine. The pharmacological mechanisms of action for topiramate that may explain its antiepileptic and migraine preventive activities will be discussed in this review. In addition, the potential relationship between the molecular activities of topiramate and its efficacy in epilepsy and migraine prevention will be emphasized.     

Effects of dextropropoxyphene on the steady-state kinetics of oxcarbazepine and its metabolites

The effects of dextropropoxyphene on the steady-state kinetics of oxcarbazepine and its metabolites were investigated in eight patients with epilepsy or trigeminal neuralgia. One patient dropped out of the study, presumably due to side-effects of dextropropoxyphene. Dextropropoxyphene did not affect the plasma levels of the principal active metabolite, 10,11-dihydro-10-hydroxy-carbamazepine. Since dextropropoxyphene is known to increase the plasma levels of carbamazepine, leading to toxicity, the findings of this study suggest that oxcarbazepine is a useful alternative to carbamazepine when concomitant dextropropoxyphene therapy is required.     

苯妥英钠、苯巴比妥钠诱导建立难治性癫(癎)动物模型

目的探索性应用苯妥英钠（PHT）、苯巴比妥钠（PB）诱导建立难治性癫痫动物模型，并研究其多药耐药机制。方法将60只大鼠随机分为实验组50只和对照组10只；实验组给予亚抽搐剂量戊四氮腹腔注射，其中45只大鼠确定点燃．将其随机分为给药组35只和未给药组10只。给药组应用较大剂量PHT、PB腹腔注射，其间注射小剂量戊四氮．根据Racine行为分级以及脑电图改变，从中筛选出耐PHT和PB的难治性癫痫动物模型。应用免疫组化法观察比较P糖蛋白（Pgp）在各组脑组织中的表达。结果12只大鼠制成难治性癫痫动物模型（耐药组），11只为药物有效组，12只死亡。耐药组大鼠脑组织Pgp表达较对照组、未给药组和药物有效组增强，差异有高度统计学意义（P〈0．01）。结论应用较大剂量PHT和PB诱导点燃大鼠制作难治性癫痫的动物模型．方法可行。该模型可以用于研究难治性癫痫脑内PgP的表达。PgP的高表达与难治性癫痫发生密切相关。