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find Keyword "皮质发育不良" 12 results
  • Malformations of cortical development and epilepsy: a cohort of 150 patients in western China

    ObjectiveMalformations of cortical development (MCDs) are increasingly identified as important etiology for refractory epilepsy. Little is known about the spectrum, distribution and clinical features of MCDs, especially in a resource-limited region. This study investigated the distribution and compared the clinical features and long-term prognosis between simple and multiple forms. MethodsConsecutive 150 epilepsy patients with pathologically or radiologically confirmed MCDs were included from a tertiary epilepsy center in western China. Patients were divided into three subtypes according to the scheme of Barkovich, also Simple and Multiple forms based on whether single type of MCDs or other brain developmental abnormalities co-existed. ResultsThe most common type of MCDs is focal cortical dysplasia, and China is still in the early phase of implementing surgical treatments. We found perinatal insults more common in sub-group III patients. Furthermore, 'Multiple' form was identified in 36/150 patients. Patients with heterotopias were more commonly associated with other abnormalities. ConclusionMCDs are critical causes for epilepsy, also a big challenge for resource-limited countries. Imaging techniques are crucial in diagnosing and classifying cortical deformities. Multiple malformations lead to more severe clinical features and worsen the prognosis, helping physicians to seek the best therapeutic option, also assists in classifying MCDs.

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  • 局灶性皮质发育不良的病理机制

    局灶性皮质发育不良(Focal cortical dysplasias, FCDs)是儿童难治性癫痫的常见病因,也是常需癫痫手术的原因。尽管近年来在细胞和分子生物上的进展,FCDs的病理机制仍不清楚。该研究旨在回顾FCDs的分子机制,系统地检索FCDs组织、分子和电生理方面的文献,以明确可能的治疗靶点。哺乳动物雷帕霉素靶蛋白信号通路(mammalian target of rapamycin,mTOR)是一些FCDs结构和电生理紊乱的重要机制。其他的假说包括病毒感染、早产、头部外伤和脑肿瘤。mTOR抑制剂(如:雷帕霉素)在动物和少量FCDs患者的队列癫痫控制中取得阳性结果。近期研究在发育不良组织细胞的分子和电生理机制方面取得了令人鼓舞的进展。尽管mTOR抑制剂有良好的治疗前景,但仍需大规模的随机对照研究评估其有效性和不良反应,并且需要基础研究发现新的分子水平诊断和治疗方式。

    Release date:2016-11-28 01:27 Export PDF Favorites Scan
  • The study of electroencephalogram and magnetic resonance imaging about Wistar suckling rats Focal cortical dysplasia model

    ObjectiveTo make the model of Wistar suckling rats Focal cortical dysplasia (FCD) by liquid nitrogen freezing brain cortex and verify it. Analysed the electroencephalogram (EEG) and magnetic resonance imaging (MRI) features of the FCD model, in order to provide theoretical and experimental basis for human FCD diagnosis and treatment. MethodsTake the first day of Wistar suckling rats as experimental object, liquid nitrogen freezing Wistar suckling rats brain cortex.Make examination of EEG and MRI for Wistar suckling rats. The Brain tissue slice of Wistar suckling rats model dyed by HE and check with light microscope examination. ResultsIn experiment group, the sample epileptic discharge rate of EEG was about 41.6% on average, and showed visible spike wave, spine slow wave frequency distribution. Experimental Wistar suckling rats MRI showed positive performance for long T1 and long T2 signal, brain tissue slices HE staining showed brain cortex layer structure and columnar structure disorder, exist abnormal neurons and the balloon sample cells. ConclusionThe method of liquid nitrogen freezing Wistar suckling rats cortex can established FCDⅢd animal models successfully, and showed specific EEG and MRI, which has important value for diagnosis and treatment of human FCD.

    Release date:2016-11-28 01:27 Export PDF Favorites Scan
  • Surgical outcomes of focal cortical dysplasia: a follow-up study of 102 patients

    ObjectiveTo explore the prognostic factors for seizure control in focal cortical dysplasia(FCD)by analyzing the clinical features of FCD patients. MethodsWe conducted a follow-up study of patients, who were confirmed FCD by pathology after resective surgery,in Epileptic Center, Guangdong Sanjiu Brain Hospital, From January 1, 2014 to December 31, 2014. All patients were followed at least 6 months,they were divided into seizure control group(Engel class I) and seizure group(Engel classⅡ-class Ⅳ) according to surgical outcomes. Clinical features,auxiliary examinations and pathological classification were compared between two groups. Results102 patients were included, male 65 cases (63.7%), female 37 cases (36.3%), onset age 0.01~45 years old, average (10.3±8.26) years old, surgery age (3~47) years old, average (21.21±8.9) years old, all had seizure onset. 83 (81.4%) patients in seizure control group, 19 (18.6%) patients in seizure group. There are 14.5% of the patients' onset ages are younger than 3 years old, 59.8% preoperative electroencephalogram recording a diffusion epileptiform discharge, 32.5% orientation of magnetic resonance imaging (MRI) and electroencephalography (EEG) is inconsistent, 49.4% postoperative electroencephalogram (EEG) reveal an epileptiform discharge, 45.2% of the patients had intellectual disability, 36.1% had an absence of a lesion on MRI, in seizure control group. However,in seizure group they respectively 36.8%, 72.2%, 89.5%, 68.4%,94.1%, 89.5%. Patients in seizure control group got an average scores of (89.4±18.53) in performance intelligence quotient (PIQ)test, while, seizure group 65.80±15.71.There has a statistical significance between two groups. ConclusionPostoperative seizure outcome was favorable in patients with FCD, onset ages younger 3 years old, intellectual disability,getting a lower scores in PIQ test, preoperative electroencephalogram recording a diffusion epileptic discharge, inconsistent orientation of MRI and EEG, and postoperative EEG reveal an epileptiform discharge may be predictive for the postoperative outcome.

    Release date:2016-11-28 01:27 Export PDF Favorites Scan
  • 局灶性皮质发育不良和神经发育肿瘤的癫痫发作模式与手术预后和神经病理亚型的联系

    颅内脑电图对癫痫发作模式的研究对癫痫灶的精确定位和指导成功切除有重要作用。它也引出了癫痫发生机制相关的重要病理生理问题。目前,植入硬膜下和深部电极等记录方式已经描述了几种癫痫发作模式 (主要是颞叶癫痫和伴有异质新皮层病变的癫痫)。研究分析了53例患者的连续性队列资料,所有患者均行立体定向脑电图 (SEEG) 监测,且病理证实为皮质发育畸形 (Malformation of cortical development, MCD)——局灶性皮质发育不良 (Focal cortical dysplasia, FCD) 和神经发育肿瘤 (Neurodevelopmental tumors, NDTs)。通过对视觉和时间-频率的分析,证实了存在6种癫痫发作模式:低压快波活动 (Low-voltage fast activity, LVFA);发作前棘波继之LVFA;爆发性多棘波继之LVFA;慢波/直流电漂移继之LVFA;θ或α尖波;节律性棘波/棘波。结果表明包含LVFA的模式 (83%) 普遍性较高,但是LVFA并不是癫痫发作的一个固定特征。癫痫发作模式和组织学类型具有相关性 (P=0.01)。更加普遍的模式如下:① FCD Ⅰ型:LVFA占23.1%,慢波/基线漂移继之LVFA占15.4%;② FCD Ⅱ型:爆发性多棘波继之LVFA占31%,LVFA占27.6%;发作前棘波继之LVFA占27.6%;③ NDTs:LVFA占54.5%。发现包含LVFA的癫痫发作模式与较好的手术预后具有相关性,但癫痫灶切除的完整性是一个独立预测因子;FCD和NDTs有6种不同的癫痫发作模式;包含LVFA的癫痫发作模式的患者手术预后更好。

    Release date:2017-04-01 08:51 Export PDF Favorites Scan
  • 神经元移行障碍发病机制研究进展

    胚胎发育时期因各种因素引起的神经元移行障碍,常导致大脑皮质发育畸形(Malformation of cortical development,MCD),也是引起患儿发育迟缓和癫痫的常见原因。快速发展的分子生物学、影像学和基因遗传学丰富了大脑皮质发育的相关知识,畸形的报道不仅在数量和种类上有所增长,同时相关研究已经确定了几个基因,可能破坏神经细胞增殖、移行以及晚期皮层组织形成的每一个重要阶段。不同的MCD在临床表现上也有很大的表型异质性,现就MCD的发生机制、易感基因进行阐述,为MCD后期研究提供参考依据。

    Release date:2017-09-26 05:09 Export PDF Favorites Scan
  • The characteristics of high-resolution magnetic resonance imaging with focal cortical dysplasia in children

    ObjectiveTo discuss the 3D high resolution Magnetic resonance imaging (MRI) features of focal cortical dysplasia (FCD) in children.MethodsMRI data of 42 children with FCD confirmed by pathology, from April 2015 to June 2018, which were admitted to Qilu Children’s Hospital of Shandong University, were retrospectively analyzed. The following MRI signs were observed, blurring of junction of the gray matter-white matter, abnormality of structure with focal cortex (thick or thin), gray matter and white matter signal, white matter signal increased with T2WI/FLAIR, with or without transmantle sign (abnormal signal of white matter extending in the direction of ventricle), gray matter signal increased with T2WI/FLAIR, the abnormal sulci or gyri morphology and segmental and/or hypoplasia/atrophy of the lobes.ResultsAmong the 42 cases, 37 cases (88.1%) showed MRI positive signs, FCD typeⅠ accounted for 13 cases (35.1%), the main MRI features are focal blurring of junction in the gray matter-white matter, abnormality of structure with focal cortex in the corresponding part,and white matter signal increased with T2WI/FLAIR. FCD TypeⅡ accounted for 17 cases (45.9%), the MRI features are focal blurring of junction in the gray matter-white matter, abnormality of structure with focal cortex, white matter signal increased with T2WI/FLAIR, and transmantle sign. FCD TypeⅢ accounted for 7 cases (18.9%), among which hippocampal atrophy 2 cases (28.6%), dysembryoplastic neuroepithelial tumor (DNET) 2 cases (28.6%), section cell tumor 1 case (14.3%), softening lesion with gliosis 2 cases (28.6%).ConclusionThe 3D high-resolution MRI features of FCD in children are specific and could improve the detection rate of FCD lesions.

    Release date:2020-03-20 08:06 Export PDF Favorites Scan
  • 局灶性皮质发育不良与儿童孤独症谱系障碍的相关性研究进展

    结节性硬化症(Tuberous sclerosis complex,TSC)是一种常染色体显性遗传疾病,已被证实与儿童局灶性脑皮质发育(Focal cortical dysplasia,FCD)和孤独症谱系障碍(Autism spectrum disorder,ASD)的发病机理有关。作为研究 FCD 以及 ASD 的模型,许多文献中都提到了 TSC,但目前尚不清楚具体机制。文章对 TSC 与 FCD 和 TSC与 ASD 患儿之间的关系,以及FCD与 ASD 之间的联系,提出新的想法。

    Release date:2020-05-19 01:07 Export PDF Favorites Scan
  • The value of high-resolution MRI in predicting the outcome of surgery for focal cortical dysplasia in children

    ObjectiveTo explore the value of high-resolution Magnetic resonance imaging (MRI) imaging in predicting the surgical effect of Focal cortical dysplasia (FCD) in children.MethodsThe MRI and clinical data of 143 children with FCD confirmed by surgery and pathology in Qilu Children’s Hospital of Shandong University from July 2013 to July 2018 were analyzed retrospectively, and the MRI signs were analyzed, and the patients were grouped according to different signs to analyze the satisfaction of postoperative epilepsy control in each group.ResultsAmong the 9 groups of children, MRI signs in the group with better postoperative epilepsy control were those with obvious focal gray matter blurring and cortical thickening and combined with Transmantle sign. The MRI signs in the group with poor postoperative results were mild focal gray matter blurring and cortical structure thinning, and those with abnormal sulcus and gyrus morphology, the postoperative effect of patients with only abnormal sulci and gyrus as the main MRI manifestations was significantly better than those with other signs, and the above differences were statistically significant (P<0.05).ConclusionThe high-resolution MRI of children with FCD had a certain specificity, and it is feasible to predict the satisfaction of postoperative epilepsy control from the perspective of MRI signs, which is an important indicator of surgical prognosis.

    Release date:2021-02-27 02:57 Export PDF Favorites Scan
  • Analysis of focal cortical dysplasia with children whose epilepsy was unrelieved thoroughly after operation based on MRI

    ObjectiveTo analyze the causes of unrelieved epilepsy thoroughly in children with isolated focal cortical dysplasia (FCD) based on MRI.MethodsRetrospective analysis of MRI and clinical data of 21 children with isolated FCD during July 2014 to January 2018, which confirmed by pathology and unrelieved thoroughly after operation performed, the pathological types and MRI signs were analyzed, as well as the frequency of different MRI signs in FCD of each pathological type. Analyzed the possible factors of surgical failure.ResultsAmong the 21 cases, there were 15 males and 6 females, with an average age of (5.7±0.3) years and an average course of disease of (3.4±0.5) years.MRI signs of this part of the children were mainly manifested by blurred focal gray matter boundaries, abnormal cortical structure changes (thickening and/or thinning), transmantle signs (abnormal cone signals extending from subcortical white matter to the ventricle) and abnormal gray matter signals, which were similar to MRI signs of FCD with satisfactory postoperative epilepsy control. 17 cases (80.9%) appeared epileptic discharge after operation in the EEG monitoring area 2 weeks to 6 months, FCD type I and type Ⅱ accounted for 35.3%, 64.7% respectively. During intraoperative EEG monitoring, no epileptiform discharge was observed in the transmantle sign region in 6 cases, and the region was retained, and only the surrounding abnormal discharge cortex was removed, complete removal of the tansmantle sign and surrounding abnormal discharge area was performed in 2 cases, and different degrees of epileptic epilepsy were observed in both methods.ConclusionMRI signs of isolated FCD with unrelieved epilepsy after operation were nonspecific, there were still epilepsy of varying degrees after all epileptogenic lesions have been removed, the cause may be related to potential epileptic factors.

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