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。     

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.     

Mesial temporal sclerosis and volumetric investigations

Volumetric MRI data acquisition permits reliable and accurate measurement of mesial temporal lobe structures. In normal subjects, these structures are very symmetric. A high degree of pathological specificity is associated with the finding of even minor volume asymmetries. Definition in this manner allows precise estimation of both absolute and relative volume differences, and precise anatomical localisation of volume loss within the hippocampus. There are good EEG and clinical correlates with the distribution of volume loss defined on MRI studies. Volumetric assessment is fast, reliable, non-invasive, and a relatively inexpensive component of the pre-operative work-up. It is the method of choice when imaging patients with clinical temporal lobe epilepsy undergoing pre-surgical evaluation. The finding of significant hippocampal volume asymmetry in a patient with clinical temporal lobe seizures being evaluated for epilepsy surgery may obviate the need for alternative sophisticated, invasive, or expensive investigative procedures. At our centre, such MRI allows "fast track" cases to proceed to surgery without further invasive investigations, and is likely to have a dramatic effect on pre-operative evaluation in most centres practising epilepsy surgery.     

Ictal brain imaging in presurgical evaluation of patients with medically intractable complex partial seizures

At the Indiana University Medical Center, 99 patients with medically intractable complex partial seizures (MI-CPS) had presurgical evaluation with subsequent anterior temporal lobectomy. The majority of the patients had single photon emission tomography (SPECT) performed interictally as well as during an actual epileptic seizure (ictal scan). Decreased regional cerebral perfusion (rCP) was seen in 54/94 (57%) of the interictal scans corresponding to the eventual site of the surgery. However, ictal scans provided a higher yield; increased rCP in the temporal lobe during an actual seizure was observed in 60/82 (73%) concordant to the side of surgery. SPECT is a useful, noninvasive method of localizing the epilepti-form focus in patients with MI-CPS considered for resective surgery. Both interictal and ictal SPECT need to be performed; combined interictal hypo-perfusion and ictal hyperperfusion in the same focal area are unique to epileptogenic lesions. Ictal SPECT studies can be performed in the majority of patients during the period of continuous video/EEG monitoring with only a little additional effort. Combining the results of functional brain imaging (interictal and ictal SPECT, PET) with clinical semiology of seizures, surface and sphenoidal EEG, magnetic resonance imaging and other non-invasive tests, anterior temporal lobectomy can be recommended in approximately two-thirds of the patients without resorting to potentially dangerous intracranial EEG monitoring.     

CT多平面重组显示镫骨的方法及应用价值

目的探讨利用螺旋CT多平面重组(mu ltip lanar reform ation,MPR)技术显示镫骨整体结构的方法和临床价值。方法对50例无耳症状、体征的志愿者行平行枕眶线扫描,应用轴位图像行多方位的MPR。比较轴位MPR、冠状位MPR、矢状位MPR和镫骨斜位MPR显示镫骨整体结构的差异。测量镫骨斜位图像显示层面在轴位定位图像上与矢状面的夹角,在冠状位定位图像上与矢状面的夹角。先天性镫骨异常31例,全部病例经电测听检查均表现有不同程度的传音性耳聋,均行听骨链重建术。结果正常组轴位、冠状位、矢状位均未能显示镫骨弓的整体形态,镫骨斜位MPR图像均直观显示了镫骨弓的整体形态和结构。在轴位定位图像上,镫骨斜位MPR图像显示层面与矢状面的夹角为36°±8°,在冠状位定位图像上与矢状面的夹角为57°±6°。异常组31例,其中一侧小腿缺如3例;镫骨弓不发育3例;镫骨底板增厚与周围骨融合16例;镫骨底板上结构(小头、颈、小腿)完全缺如9例,斜位MPR图像均直观显示其病理改变,轴位、冠状位以及矢状位图像均不能准确显示镫骨发育不良的病理改变。结论镫骨斜位MPR图像,能显示正常镫骨整体结构和镫骨异常改变,对听骨链重建术的术前评价具有重要意义。     

Scalp and Limbic P3 Event-Related Potentials in the Assessment of Patients with Temporal Lobe Epilepsy

Auditory oddball scalp and limbic P3s were recorded from 18 patients with unilateral temporal lobe epilepsy (TLE) prior to seizure surgery. Limbic P3s were unilaterally absent ipsilateral to the seizure focus and were present in the nonepileptogenic temporal lobe in all 18 cases studied. Scalp P3s, recorded from C3 and C4, on the other hand, were elicited bilaterally and there was no significant difference in amplitude or latency between the epileptogenic and nonepileptogenic sides. These data concur with studies of scalp P3 performed following surgery and suggest that the assessment of the contribution of limbic P3 to scalp P3 may be masked by volume conduction effects and other generators of P3. We conclude that the P3 recorded from central scalp sites, unlike its limbic counterpart, offers little clinical information in the presurgical assessment of patients with TLE.     

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.