婴幼儿癫痫的外科治疗

目的探讨婴幼儿癫痫外科的手术意义。方法从2002-2005年有12例婴幼儿因海绵状血管瘤(4例)、发育不良神经上皮肿瘤(DNTs)(2例)、Sturge.weber综合征(1例)、半侧巨脑病(2例)、外伤后脑膜脑疤痕(1例)、脑脓肿疤痕组织(1例)、脉络丛乳头瘤(1例);由分别位于额(2例)、颞(4例)、顶(2例)、枕(2例)与额顶及颞顶(各1例)病变所致均为灾难性癫痫。当年龄在9—32(平均21.5)个月龄时做了病变及致痫灶切除/功能性大脑半球切除。癫痫开始于出生后5～20 (平均11.8)个月,每天或每周发作多次,抗痫药物治疗无效。患儿做手术评估是6—22(平均12.8)个月。3例患儿有偏瘫,7例显示有缓慢发展的认知功能减低表现。MRI、PET及V-EEG都证实了病灶及致痛灶。结果手术后无死亡及新的神经症状缺失。8例解除了癫痫(EngelⅠ级);2例罕有发作(EngelⅡ级);2例有值得的(worthwhile)改善(EngelⅢ级),7例认知功能也有改善,3例偏瘫者2例有改善。结论灾难性癫痫婴幼儿仔细选择手术病例,外科可以减轻、解除癫痫的疾苦。     

Corpus callosotomy in refractory idiopathic generalized epilepsy

RATIONALE: A small percentage of patients with idiopathic generalized epilepsy (IGE) do not respond to medical therapy. Generalized tonic-clonic (GTC) seizures are especially debilitating and can be associated with severe injuries. The benefit, safety and effect of corpus callosotomy (CC) in patients with IGE have not been studied. METHODS: We reviewed patients with presumed IGE who underwent CC between 1991 and 2000. Criteria for selection included history, examination, brain imagining, interictal and ictal EEG. All patients had refractory and debilitating tonic-clonic seizures (GTCS) and had failed four or more antiepileptic drugs. Seizure frequency was calculated per month over the last year and pre-operative baseline was compared to last follow-up using paired t-tests. IQ, executive function, language and verbal, non-verbal memory and quality of life (QOL) was compared before and after surgery. Serial EEGs after surgery were reviewed. RESULTS: There were nine patients (seven men), mean age 37.9 (range: 22-49), mean IQ 87.3 (range: 75-107). All had anterior CC. Mean follow-up time was 5.4 years (range: 0.6-10.3 years). One patient died from sudden death in epilepsy 9 months after surgery. There was a significant reduction of GTC seizures from 6.3 to 1.1 (p<0.005). Four patients had more than 80% and eight more than 50% reduction. Of five patients with absence seizures, two became seizure free and one had more than 80% reduction and two worsened slightly, and of three with myoclonic seizures one had more than 90% reduction. One patient had completion of the CC with improvement of myoclonus and absence seizures, but not of GTC seizures and suffered a disconnection syndrome. Another had right frontal focal resection without improvement after new seizures of focal onset. Cognitive testing showed a good outcome (improved or no change) in all cognitive domains. Post-surgical EEG showed new focal slowing and sharp waves. There was no change in QOL. CONCLUSION: CC can be effective in reducing GTC, absence and myoclonic seizures in patients with refractory IGE. These findings suggest that interhemispheric communication of the cerebral cortices plays an important role in the generation of seizures in IGE. Anterior CC appears safe while complete callosotomy has a risk of disconnection syndrome.     

匹罗卡品致(癎)大鼠海马中表达生长抑素的中间神经元数目变化及其轴突出芽

Objective To investigate the roles of somatostatin(SS)positive intemeurons in the development and compensation of temporal lobe epilepsy.Methods Piloearpine-induced epilepsy rat model was established.Immunohistochemistry method was used to detect number changes and axonal sprouting of SS positive intemeurons in different domains of the hippocampus at difierent time points.Degeneration of SS positive interneurons and their neurophils were detected by the double immunofluorescence staining with SS and Fluoro-Jade B(FJB)at 7 and 60 days after status epilepticus (SE).Results In the exoerimental rat group,the number of SS positive neurons decreased in each hippocampal domain,and it reached the lowest at 7 days post-SE(There were 11.1±3.3 in hilus,2.8±0.9 in CA1region and 1.8±0.7 in CA1region,t=13.519,9.644 and 8.808,all P<0.01).In chronic phase,the number of SS neurons gradually recovered,and exceeded the control group in CA1 area at 60 days post-SE(12.8±1.5 vs 8.8±1.3,t=-4.506,P<0.01),however,the number of SS neurons in the hilus(25.5±4.6)and CA1 area(4.8±0.8)remained significantly less than normal levels(t value were 4.691 and 3.953.both P<0.01).Increased SS positive fibers were found in the lacunosum-molecular (1m)layer and outer molecular layer of dentate gyrus after 30 days post-SE,and numerous SS positive fibers were seen threnghout the layers of area CA1 at 60 days post-SE.Double immunofluuorescence revealed that a few SS positive interneurons and fibers were also labeled by FJB in area CA1 at 7 days post-SE and in CA domain/hilus at 60 days post-SE.Conclusions SS intemeurons loss plays an important role in the development of temporal lobe epilepsy.The loss is partially caIlsed by the degeneration and death of neurons;SS positive neurophils increase within area CA1 in chronic phase may play a significant role in the generation and compensation of temporal lobe epilepsy.     

连接蛋白拟似肽对海人酸致痫大鼠脑电活动的影响

目的观察针对连接蛋白43(CX43)合成的特异性的缝隙连接阻断剂-连接蛋白拟似肽对海人酸致痫大鼠脑电活动的影响。方法建立18只大鼠癫痫动物模型,分连接蛋白拟似肽组、甘珀酸组和对照组(每组6只),在在体上分别局部给予连接蛋白似似肽、甘珀酸和生理盐水,用脑电图仪观测用药前后每组大鼠皮层脑电活动的变化情况。结果连接蛋白拟似肽组及甘珀酸组给药后癫痫的发作次数明显比给药前发作次数减少,癫痫波的平均振幅也明显变小,给药前后比较有显著性差异(P<0.01),生理盐水组给药前后癫痫的发作次数及平均振幅几乎没有变化。连接蛋白拟似肽组给药前后癫痫的发作次数和波幅的变化值与甘珀酸组给药前后癫痫的发作次数和波幅的变化值相比无显著性差异。结论针对CX43合成的连接蛋白拟似肽可以特异性地抑制癫痫的发作。     

癫痫患者执行功能损害特点及影响因素研究

目的分析癫痫患者执行功能损害的特点及相关影响因素。方法1999年1月～2006年4月在我院癫痫专病门诊就诊或长期随访的109例成年癫痫患者和75例正常对照者进行神经认知心理测验，包括语义流畅性试验、数字广度测验、数字符号测验、色词干扰测验和连线试验检查。结果癫痫患者组语义流畅性显著差于正常对照组，数字广度、数字符号亦显著低于正常对照组．癫痫组连线时间、Stroop试验反应时显著延长，Stroop试验错误数显著增加（P〈0．01）。颞叶癫痫患者数字广度低于额叶癫痫患者。多元线性回归显示：文化程度高、非颞叶癫痫对语义流畅性有保护作用：文化程度高、单一抗癫痫药物治疗、病程短、年龄大对数字广度有保护作用；病程短、文化程度高和颅内无病灶对数字符号有保护作用。结论癫痫患者在多项执行功能方面均存在损害．文化程度高的患者执行功能相对较好；颞叶癫痫患者语义流畅性差；病程、药物、年龄对数字广度有影响：病程、有无病灶对数字符号有影响。     

The role of formative courses in the professional integration of patients with epilepsy. An Italian experience

Abstract The aim of the present paper was to describe some formative projects on work integration of patients with epilepsy and mild cognitive deficits, so that similar initiatives may be promoted. The different phases of the formative projects were described: the inclusion criteria, the selection of participants, the assessment instruments, the courses themselves and the final results in terms of working experience. Thirteen participants were selected for each course. Patients were treated in different Lombardy Epilepsy departments, which addressed them to the Epilepsy Center, S. Paolo Hospital, Milan, where the selection took place. Seventy percent of the participants in the formative courses found a job in 1 year, even though most of the positions were temporary. The work integration seemed to be mainly related to the level of intelligence and to verbal memory. We stress the importance of organising similar courses in the future, in the context of wider networks. This will help more patients in acquiring both economic and psychological autonomy from their families.     

立体定向开放显微手术治疗脑内致痫小病灶

目的脑内致癫痫小病灶术前、术中的精确定位和病灶切除,是手术治疗效果的关键。探讨立体定向开放微创手术,皮层电极监测下切除脑内致痫小病灶的手术方法。方法53例症状性癫痫病例,CT、MR I检查有脑内小病灶(直径在0.5~3.0 cm),24 h视频脑电图确认致痫灶为脑内单发病灶。ASA 601S型立体定向仪CT引导辅助全麻环钻开颅,导针穿刺放置导管引导,显微镜下手术分离、切除病灶,皮层脑电图确认将致痫灶切除。结果病灶全切率达96.2%,术后50例得到随访,随访时间5~12个月,平均6.3个月,癫痫消失45例,脑电图检查记录到癫痫波11例,临床癫痫发作5例。因肺癌死亡3例。结论CT立体定向引导,显微手术切除颅内致痫小病灶,术中皮层电极确认将致痫灶切除,是一种定位精确、微创、安全、有效的治疗方法。     

实验性癫痫大鼠海马结构内一氧化氮-环磷酸鸟苷途径的研究

目的研究实验性癫痫发作大鼠海马结构内一氧化氮（ＮＯ）环磷酸鸟苷（ｃＧＭＰ）信使机制及其意义。方法雄性ＳＤ大鼠４１只，随机分为对照组（５只）、红藻氨酸（ＫＡ）１０、３０、６０分钟组（每组６只）和Ｌ硝基精氨酸甲酯（ＬＮＡＭＥ）＋ＫＡ１０、３０、６０分钟组（每组６只）。用放射免疫法测定ＫＡ诱导性癫痫发作中各时点海马结构内ｃＧＭＰ含量及ＬＮＡＭＥ的干预效应。结果ＫＡ注射引起大鼠海马结构内ｃＧＭＰ浓度升高，并加重大鼠癫痫发作（湿狗样摇动提早出现和发生次数增多）；ＫＡ注射前３０分钟给予ＬＮＡＭＥ可明显抑制ＫＡ１０、３０分钟组ｃＧＭＰ浓度的升高，但ＬＮＡＭＥ对ＫＡ６０分钟组ｃＧＭＰ的抑制作用不显著。结论在ＫＡ发作早期，ｃＧＭＰ浓度升高与内源性ＮＯ有关；ＮＯ的抗发作效应可能与ｃＧＭＰ信使机制存在某种联系。     

癫痫患者的红细胞免疫粘附功能的初步研究

本文用Ｃ（３ｂ）受体花环试验及ＩＣ花环试验分别测定了５０例正常人及５０例癫痫患者的红细胞Ｃ（３ｂ）受体花环率及ＩＣ花环率。结果提示癫痫患者Ｃ（３ｂ）受体花环率（８．４６±３．９８％）明显低于正常对照组（１３．９２±４．３２％）（Ｐ＜０．００１），ＩＣ花环率（５．２２±２．５９％）稍低于正常对照组（５．９０±１．６７％）（Ｐ＜０．０５）。表明癫痫患者的红细胞免疫粘附功能是低下的。文章还对影响正常人及癫痫患者红细胞免疫粘附功能的因素进行了讨论。