听神经瘤BCL-2蛋白及bcl-2/JH融合基因的研究☆

目的评价石蜡包埋听神经瘤组织中BGL-2蛋白表达及相关的bcl-2(mbr)/JH融合基因改变,以探讨bcl-2癌基因在听神经瘤发病中的可能意义.方法免疫组化检测石蜡包埋组织中BCL-2蛋白的表达;提取石蜡包埋组织的DNA,PCR检测bcl-2(mbr)/JH融合基因.结果本组40例听神经瘤,BCL-2蛋白表达阳性27例(67.5%),bcl-2(mbr)/JH融合基因检出阳性19例(47.5%).结论听神经瘤中存在BCL-2蛋白的高表达及t(14;18)染色体易位,提示雪旺氏细胞凋亡抑制可能是听神经瘤发病的分子病理基础之一.     

Bcl—2及Bax基因蛋白在听神经瘤组织中的表达及意义

目的评价Bcl-2及Bax基因蛋白在听神经瘤组织中的表达及意义。方法取库存石蜡包埋组织标本,40例听神经瘤分作复发组、囊变组及对照组,采用SABC法免疫组化半定量检测Bel-2及Bax蛋白在听神经瘤组织中的表达。结果Bcl-2阳性者27例,多见于AntoniA区;Bax阳性者26例,多见于AntoniB区。不同组间Bcl-2与Bax表达的相对构成有差别(x2=6.64,P<0.05)。结论Bcl-2/Bax蛋白表达水平的相对构成可能与听神经瘤临床生物学行为有关。     

小型听神经瘤的显微手术

目的探讨小型听神经瘤保存面、听神经功能的显微手术方法。方法采用标准的病侧枕下经乙状窦入路开颅。病人头偏一侧卧位、显微手术,高速电钻磨除内听道后壁。术中照相/磁带记录,术后分析所见。结果术后13例保全了面神经及听神经,2例无改善,其中1例且伴有耳鸣。肿瘤与Ⅶ及Ⅷ神经的关系分为5型,其中Ⅰ、Ⅱ、Ⅳ、Ⅴ型效果良好,手术困难的是Ⅲ型。结论肿瘤大小及术前听力水平应作企图保存听神经和面神经功能的主要决策。     

听神经瘤的MRI诊断

本文分析手术和病理证实76例78个听神经瘤的MRI表现,其中2例为内听道内微小听神经瘤,11例作静脉注射Gd-DTPA后MRI成象。下列特征有助于听神经瘤诊断:(1)肿瘤以第Ⅶ、Ⅷ神经束为中心生长,病侧Ⅶ、Ⅷ神经束明显增粗,与肿瘤无明确分界;(2)T_1加权图象肿瘤呈低信号或低等混合信号,T_2加权图象呈高信号或高等混合信号。静脉注射Gd-DTPA对诊断微小听神经瘤常甚有效。     

大型听神经瘤的手术治疗

报告１９９１年１月至１９９４年１２月手术切除的大型听神经瘤１４０例，无术后死亡。肿瘤全切除率８４．３％，面神经保留率８７．３％，术前有听力者听神经保留率６４．１％。绝大多数（９６％）的病例采用一侧枕下乙状窦后入路，作者总结了肿瘤全切除和保留面神经的经验，同时强调手术前后处理也至关重要。（１）危重患者可先行侧脑室－腹腔分流缓解颅压后再开颅手术；（２）术后密切观察病情变化，及时发现和清除术后血肿；（３）术前有后组颅神经麻痹者术后宜早期做气管切开；（４）伤口局部皮下积液在炎症已控制的情况下可行囊腔－腹腔分流术。     

面神经与听神经瘤局部病理解剖关系的研究

一、临床资料一般资料 :本组男 47例 ,女 65例。年龄 1 4～ 69岁 ,平均 46岁。病史 1个月至 2 0年。左侧 52例 ,右侧60例。首发症状 :耳鸣 51例 ,听力下降 30例 ,发作性头痛 1 3例 ,面部麻木 1 0例 ,面肌抽搐 2例 ,面部疼痛 3例 ,发作性眩晕 2例。体征 :听力丧失 1 5例 ,面瘫 32例 ,后组颅神经功能障碍 2 5例 ,小脑体征 73例 ,视神经乳头水肿 33例。ＭＲＩ显示的肿瘤最大径 :1～ 2ｃｍ2例 ,2～ 3ｃｍ 1 3例 ,3～ 4ｃｍ 36例 ,4～ 5ｃｍ 2 9例 ,5～ 6ｃｍ 2 3例 ,大于 6ｃｍ9例。二、手术方法及结果患者均行乙状窦后入路手术治疗 ,肿瘤全切除…     

听神经瘤的生长方式及分型

本文总结了２４年来３２７例经手术及病理证实的听神经瘤，发现听神经瘤大致可分为五种生长类型，即三叉神经型、脑干型、小脑型、乳突型及原位型五型。不同类型的肿瘤各有其特殊的生长方式，这些生长方式决定了其特有的临床表现及其肿瘤与周围结构相对恒定的解剖关系。熟练掌握这些分型及生长特点，对提高听神经瘤的早期诊断率、增加手术的成功率大有裨益。本文还对有关的手术方式作了一些探讨。     

听神经瘤显微手术保留听神经功能及影响因素分析

目的　探讨听神经瘤显微手术保留听神经功能及其影响因素。方法　收集我院近 2年来 2 3例经枕下乙状窦后入路显微手术的初发听神经瘤资料 ,其中包括肿瘤大小、术前术后听力、肿瘤内听道底侵蚀及术后小脑损伤情况。结果　耳蜗神经解剖保留 1 9例 ,保留有效听力 2例 (占术前存在有效听力患者的 33 3 % ) ,有效听力丧失保留可测听力 1 0例。听力的保留与肿瘤大小、术前听力水平、肿瘤内听道底侵蚀、小脑损伤相关。结论　肿瘤的大小、术前听力水平、肿瘤内听道底侵蚀和小脑损伤是听神经瘤术后听神经功能保留的影响因素。     

听神经瘤的显微手术及面神经保留

目的探讨听神经瘤的显微手术及保留面神经的技术。方法回顾性分析了我院近6年经枕下乙状窦后入路显微手术切除的23例听神经瘤病人。结果肿瘤全切18例,次全切3例,部分切除2例;面神经解剖保留15例。随访6月￣4年,全切18例中复发1例,面神经功能恢复12例。结论利用显微外科技术,经枕下乙状窦后入路,能全切听神经瘤,解剖保留面神经,有利于面神经功能的恢复。     

听神经瘤的显微外科手术治疗

目的:分析总结听神经瘤的临床特点、提高面神经保留率、手术入路的选择、降低术后并发症及死亡率。方法:回顾性分析研究我院近12年来经显微手术治疗的97例听神经瘤病例。结果:本组听神经瘤镜下全切除率为97.9％,面神经保留率为89.7％(90例),术后患侧仍有听力者9例,无死亡患者。结论:熟练的显微外科操作技术及丰富的解剖知识是提高听神经瘤治愈率和降低致残率、死亡率的关键。     

两步—多重PCR诊断134例假肥大型进行性肌营养不良症基因缺失

研究临床检测假肥大型进行性肌营养不良症（ＤＭＤ／ＢＭＤ）基因缺失的有效手段。方法运用两步－多重聚合酶链反应（ＰＣＲ）技术诊断ＤＭＤ／ＢＭＤ基因缺失。结果１３４例病人中，检测阳性率为４９．３％（６６／１３４）。结论多重ＰＣＲ诊断ＤＭＤ／ＢＭＤ基因缺失敏感、快速、准确，可在临床推广应用。     

Altered cell cycle-related gene expression in brain and lymphocytes from a transgenic mouse model of Alzheimer's disease [amyloid precursor protein/presenilin 1 (PS1)

Cumulative evidence indicates that aberrant re‐expression of many cell cycle‐related proteins and inappropriate neuronal cell cycle control are critical events in Alzheimer’s disease (AD) pathogenesis. Evidence of cell cycle activation in post‐mitotic neurons has also been observed in murine models of AD, despite the fact that most of these mice do not show massive loss of neuronal bodies. Dysfunction of the cell cycle appears to affect cells other than neurons, as peripheral cells, such as lymphocytes and fibroblasts from patients with AD, show an altered response to mitogenic stimulation. We sought to determine whether cell cycle disturbances are present simultaneously in both brain and peripheral cells from the amyloid precursor protein (APP)/presenilin 1 (PS1) mouse model of AD, in order to validate the use of peripheral cells from patients not only to study cell cycle abnormalities as a pathogenic feature of AD, but also as a means to test novel therapeutic approaches. By using cell cycle pathway‐specific RT²Profiler? PCR Arrays, we detected changes in a number of cell cycle‐related genes in brain as well as in lymphocytes from APP/PS1 mice. Moreover, we found enhanced 5′‐bromo‐2′‐deoxyuridine incorporation into DNA in lymphocytes from APP/PS1 mice, and increased expression of the cell proliferation marker proliferating cell nuclear antigen (PCNA), and the cyclin‐dependent kinase (CDK) inhibitor Cdkn2a, as detected by immunohistochemistry in cortical neurons of the APP/PS1 mice. Taken together, the cell cycle‐related changes in brain and blood cells reported here support the mitosis failure hypothesis in AD and validate the use of peripheral cells as surrogate tissue to study the molecular basis of AD pathogenesis.     

In situ polymerase chain reaction detection of HTLV-I provirus and expression of the p53 tumor suppressor gene in infiltrating cells in skeletal muscle from a patient with adult T cell leukemia

We report the pathological changes in skeletal muscle from a patient with acute adult T cell leukemia (ATL). HTLV-I provirus was detected in infiltrating cells using in situ polymerase chain reaction in frozen sections. Furthermore, aberrant expression of the p53 protein was observed in the infiltrating cells. As p53 protein was not observed in mononuclear inflammatory cells in patients with polymyositis, expression of the p53 protein was considered to be one of the characteristic findings in ATL cells. This is the first direct detection of ATL cells in skeletal muscle.     

DNA microarray analysis of striatal gene expression in symptomatic transgenic Huntington's mice (R6/2) reveals neuroinflammation and insulin associations

Huntington's disease (HD) is an inherited, progressive neurodegenerative disorder caused by CAG repeat expansion in the gene that codes for the protein huntingtin. The underlying neuropathological events leading to the selectivity of striatal neuronal loss are unknown. However, the huntingtin mutation interferes at several levels of normal cell function. The complexity of this disease makes microarray analysis an appealing technique to begin the identification of common pathways that may contribute to the pathology. In this study, striatal tissue was extracted for gene expression profiling from wild-type and symptomatic transgenic Huntington mice (R6/2) expressing part of the human Huntington's disease gene. We interrogated a 15 K high-density mouse EST array not previously used for HD and identified 170 significantly differentially expressed ESTs in symptomatic R6/2 mice. Of the 80 genes with known function, 9 genes had previously been identified as altered in HD. 71 known genes were associated with HD for the first time. The data obtained from this study confirm and extend previous observations using DNA microarray techniques on genetic models for HD, revealing novel changes in expression in a number of genes not previously associated with HD. Further bioinformatic analysis, using software to construct biological association maps, focused attention on proteins such as insulin and TH1-mediated cytokines, suggesting that they may be important regulators of affected genes. These results may provide insight into the regulation and interaction of genes that contribute to adaptive and pathological processes involved in HD.     

Single prolonged stress increases contextual freezing and the expression of glycine transporter 1 and vesicle-associated membrane protein 2 mRNA in the hippocampus of rats

Rats subjected to single prolonged stress (SPS) show enhanced HPA negative feedback, exaggerated acoustic startle response, and enhanced contextual freezing 7 days after SPS, and accordingly, SPS is an animal model of PTSD. To elucidate the influence of contextual fear on gene expression in the hippocampus of SPS rats, we used cDNA microarray followed by real-time quantitative PCR analyses to compare the hippocampal gene expression profiles between rats that were or were not subjected to SPS during exposure to contextual fear. In the behavioral experiments, spontaneous locomotor activity was measured 7 days after SPS. Twenty-four hours after footshock conditioning (7 days after SPS), freezing behavior was measured during re-exposure to the chamber in which footshock was delivered. Based on the behavioral analysis, rats subjected to SPS exhibited a significant enhancement of contextual freezing compared to rats not subjected to SPS, without any changes in locomotor activity. Analyses using cDNA microarray and RT-PCR showed that the hippocampal levels of glycine transporter 1 (Gly-T1) and vesicle-associated membrane protein 2 (VAMP2) mRNA in rats subjected to SPS were significantly increased relative to sham-treated rats. Administration of SPS alone did not affect the expression of these 2 genes. These findings suggest that the upregulation of Gly-T1 and VAMP2 in the hippocampus may be, at least in part, involved in the enhanced susceptibility to contextual fear in rats subjected to SPS.