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1.
目的:探讨癫痫患儿的免疫功能状态及免疫球蛋白辅助治疗的疗效。方法:测定54例癫痫患儿血中IgG、IgA、IgM、S3及T细胞亚群,与正常对照组比较;对14例常规抗癫痫治疗效果不佳者加用免疫球蛋白辅助治疗,观察疗效。结果:癫痫患儿血清IgG降低(P<0.05),IgA降低(P<0.01),IgM及C3正常,CD3、CD4降低(P<0.05),CD8升高(P<0.01),CD4/CD8降低(P<0.05),免疫球蛋白辅助治疗有效率57.2%。结论:癫痫患儿存在免疫功能紊乱,提示免疫机制参与了癫痫发病机制;免疫球蛋白辅助治疗癫痫有效。 相似文献
2.
报告10例MG病人均检测了周围血淋巴细胞计数、血清免疫球蛋白(IgG、IgA、IgM)、补体C3、CH50、E-玫瑰花结试验(ERFT)和淋巴细胞转换试验(LTT),同时还测定血丙酮酸及酶谱(GOT、LDH、CPK),并设30例正常对照。MG组与对照组比较,除ERFT(P<0.001)、GOT(P<0.01)、CPK(P<0.01)、及LDH(P<0.05)有差异外,余诸项均无统计学意义、提示一般血清免疫学或补体测定对MG诊断无特异性意义,结合文献复习对MG患者若干临床问题分别进行讨论。 相似文献
3.
夏科-马里-图思病Cx32、MPZ和PMP22基因点突变的特点 总被引:2,自引:0,他引:2
目的研究我国夏科马里图思病(CMT)Cx32、MPZ和PMP22基因点突变的特点。方法应用聚合酶链反应单链构象多态性(PCRSSCP)结合DNA序列分析检测30个CMT家系的患者及50名非血缘关系的正常对照的Cx32、MPZ和PMP22基因的编码外显子。结果4个家系(3个为X连锁隐性遗传)有4种不同的Cx32基因点突变(13.3%),其中1个家系为188位的苏氨酸被丙氨酸置换是未见报道的新突变。1个家系(3.3%)有MPZ基因点突变,未发现PMP22基因的点突变。结论Cx32、MPZ和PMP22基因的点突变占CMT致病原因的16.7%。我国也存在不少X连锁遗传的家系(>10.0%),对可疑的家系(家系中患者无男传男的现象)应首先进行Cx32基因的点突变检测。我国的CMT发病率低可能与其基因突变特点有关。 相似文献
4.
脑血管疾病与动态血压监测 总被引:15,自引:0,他引:15
采用动态血压监测技术(ABPM)观察240例脑血管病(CVD)患者和40名正常成人。其中脑动脉粥样硬化46例。短暂性脑缺血发作(TIA)28例,脑出血36例,脑血栓形成40例,腔隙梗塞90例。ABPM监测结果显示:正常人血压波动呈长柄“杓”型,少数正常人(2.5%)昼夜节律消失。240例CVD患者ABPM异常者228例(95.0%),其中平均收缩压(MSBP),平均舒张压(MDBP)超过正常值者1 相似文献
5.
人脑胶质瘤细胞多药耐药性的形成及其耐药特性的研究 总被引:3,自引:0,他引:3
目的探讨人脑胶质瘤细胞多药耐药性(MDR)的形成规律及其耐药特性。方法采用长春新碱(VCR)在体外对人脑胶质瘤BT325细胞持续诱导获得了表现为MDR特征的人脑胶质瘤细胞模型.以MTT法测定胶质瘤细胞增殖,透射电镜行超微结构研究,通过流式细胞仪,检测培养的胶质瘤细胞与32例术后胶质瘤新鲜组织标本中P-糖蛋白的表达。结果耐药的胶质瘤细胞BT325/VCR对长春新碱的耐受程度为其亲本细胞的24倍,对阿霉素、威猛交叉耐药,对5-氟脲嘧啶敏感。透射电镜下见BT325/VCR细胞常染色质明显,线粒体丰富,粗面内质网增多。研究表明BT325/VCR表现为P-糖蛋白介导的典型MDR。胶质瘤新鲜组织标本中,P-糖蛋白表达阳性率为375%(12/32),同肿瘤的恶性程度呈正相关(P<0.01)。结论长春新碱能够诱导人脑胶质瘤细胞产生P-糖蛋白介导的典型MDR。胶质瘤中多药耐药基因产物P-糖蛋白表达的阳性率同肿瘤的恶性程度呈正相关。应用流式细胞仅能早期发现耐药细胞,具有临床推广价值。 相似文献
6.
用ELISA方法,检测36例格林-巴利综合征(GBS)患者和40例其他神经病(OND)患者的血清和脑脊液及40例健康对照(NC)血清标本的抗P2蛋白IgG和IgM抗体。结果发现:GBS和OND血清抗P2Ig6和IgM抗体与NC血清比较无明显差异(P>0.05)。GBS脑脊液抗P2IgG抗体明显高于OND(P<0.05),而抗P2IgM抗体则两者无显著差异(P>0.05)。 相似文献
7.
目的 检测多发性肌炎(PM),皮肌炎(DM)和非肌炎患者肌肉组织中组织相容性白细胞,I,Ⅱ类抗原(HLA-I,Ⅱ)分子,探讨PM和DM的发病机制,方法 用链菌素亲生物蛋白-过氧化物酶连结技术对17例已确诊的PM和DM患者,7例非肌炎患者肌肉组织染色观察。结果 HLA-1和HLA-Ⅱ类分子在肌炎组的阳性率分别是88.24%和64.71%,对照组为阴性,它们主要沉积在肌纤维表面,单核细胞,毛细血管和小 相似文献
8.
脑脊液髓鞘碱性蛋白抗体检测对格林—巴利综合 … 总被引:1,自引:0,他引:1
目的 探讨格林-巴利综合征(GBS)中枢神经髓鞘的免疫性损伤,方法GBS患者20例,神经系统其它疾病组(OND)20例,非神经系统疾病手术者33例,以酶联免疫吸附法检测了73份脑脊液(CSF)中髓鞘碱性蛋白IgG(MBP-IgG)。结果 GBS患者,OND患者和非神经系统疾病手术者CSF中MBP-IgG阳性经分别为55%,30%和9.1%,GBS患者CSF中MBP-IgG阳性率与发病天线有关,结论 相似文献
9.
(1)目的:突发性多发性肌炎(IPM)、皮肌炎(DM)和包含体肌炎(IBM)在形态学上定义明确,但仅有间质性肌炎的诊断标准不够明确。为了确定IBM形态学上的特征,作者用光镜试图探索肌纤维束周Leu19抗原表达对IBM的诊断意义。(2)方法:15例无肌无力的正常对照,12例IPM,14例DM,26例IBM,8例坏死性血管炎(NV),15例神经性肌萎缩(NM),作肌肉活检。除作常规光镜检查外,还作免疫组化研究,用小鼠抗Leu19、Leu2a、CD4、CD16、CD22、CD68、C5b-9单克隆抗体作一抗,再用碱性磷酸酶抗碱性磷酸酶法显色。(3)结果:肌纤维束周Leu19抗原表达阳性率:IBD、DM和NV依次为85%、100%和100%,而正常对照、NM均为0%。(4)结论:在其它方面正常而有肌纤维束周Leu19抗原表达是IBM的病理学特征,所以可能有诊断价值。 相似文献
10.
目的 研究一氧化氮(NO) 在帕金森病(PD)小鼠模型神经损害中的作用。方法 用比色分析、高效液相色谱电化学及免疫组化法检测1甲基4苯基四氢吡啶(MPTP)和7硝基吲唑(7NI)对C57BL小鼠纹状体一氧化氮合酶(NOS) 活性,多巴胺(DA)、二羟基苯乙酸(DOPAC)、高香草酸(HVA) 水平和酪氨酸羟化酶(TH) 免疫阳性神经纤维的影响。结果 注射MPTP后C57BL小鼠纹状体NOS活性增加,7NI能明显抑制MPTP引起的NOS活性的升高( 分别为0 .93 ±0.24 和0.54 ±0.16,nmol·min-1·g-1 组织,P<0.01) 。7NI能明显减轻MPTP引起的C57BL小鼠纹状体DA( 分别为0 .8 ±0 .2 和6.8±0.5,μg/g 组织,P<0.01) 、DPOAC( 分别为0.3 ±0.1 和0 .9 ±0 .3 ,μg/g 组织,P< 0 .01)、HVA(分别为0.4±0.2 和0.9 ±0 .2,μg/g 湿组织,P< 0.01) 的降低及TH 阳性神经纤维损害。结论神经元来源的NO 参与了MPTP的毒性机制,神经元型NOS抑制剂可能有益于PD的治疗。 相似文献
11.
Gui-Lian SUN Shuang ZHAO Ping LI Hong-Kun JIANG Department of Pediatrics the First Affiliated Hospital China Medical University Shenyang China 《中国神经科学杂志》2006,(2)
Objective Tissue inhibitor of metalloproteinase-1(TIMP-1) is a multifunctional protein that has the capacity to modify cellular activities and to modulate matrix turnover. This paper revealed the contributive role of TIMP-1 in progressive muscular dystrophy (PMD). Methods We examined the expression and cellular localization of TIMP-1 protein using biopsied frozen muscle from patients with Duchenne muscular dystrophy ( DMD) , Becker muscular dystrophy (BMD) , congenital muscular dystrophy (CMD) by immunohistochemistry, double immunofluorescence and Western blot analysis. Results The results of immunohistochemistry and double immunofluorescence showed that TIMP-1 was positive only in vascular endothelial cells of normal muscles. Immunohistochemistry and Western blot analysis showed that the staining intensity was distinctly increased in some dystrophic muscles of PMD for TIMP-1. Double immunofluorescence revealed that TIMP-1 strongly expressed in the regenerating muscle fibers, macrophages and macrophage infiltrating necrotic fibers. Some activated fibroblasts in endomysium and perimysium of DMD and CMD muscles were also positive for TIMP-1. Conclusion The functional consequence of overexpression of TIMP-1 in the dystrophic muscles is unknown, but the elevated local expression of TIMP-1 in diseased muscles of PMD and their distinct distribution pattern provide evidence that TIMP-1 may participate in the pathogenesis of PMD. 相似文献
12.
Expression of tissue inhibitor of metalloproteinase-1 in progression muscular dystrophy 总被引:2,自引:0,他引:2
Objective Tissue inhibitor of metalloproteinase-1 (TIMP-1) is a muhifunctional protein that has thc capacity to modify cellular activities and to modulate matrix turnover. This paper revealed the contributive role of TIMP-1 in progressive muscular dystrophy (PMD). Methods We examined the expression and cellular localization of TIMP-1 protein using biopsied frozen muscle from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) , congenital muscular dystrophy (CMD) by immunohistochemistry, double immunofluorescence and Western blot analysis. Results The results of immunohistochemistry and double immunofluorescence showed that TIMP-1 was positive only in vascular endothelial cells of normal muscles. Immunohistochemistry and Western blot analysis showed that the staining intensity was distinctly increased in some dystrophic muscles of PMD for TIMP-1. Double immunofluorescence revealed that TIMP-1 strongly expressed in the regenerating muscle fibers, macrophages and macrophage infiltrating necrotic fibers. Some activated fibroblasts in endomysium and perimysium of DMD and CMD muscles were also positive for TIMP- 1. Conclusion The functional consequence of overexpression of TIMP-1 in the dystrophic muscles is unknown, but the elevated local expression of TIMP-1 in diseased muscles of PMD and their distinct distribution pattern provide evidence that TIMP-1 may participate in the pathogenesis of PMD. 相似文献
13.
There have been several reports concerning elevated glucose 6 phosphate dehydrogenase (G6PDH), the rate-limiting enzyme of pentose phosphate pathway (PPP), in experimental muscle disturbances. PPP produces ribose, a substrate of RNA, and NADPH which is a cofactor of fatty acid synthesis. PPP also has a role of by-path pathway of glycolysis. Then, we evaluated G6PDH activity and RNA content in biopsied quadriceps muscle. The subjects were muscles from 23 neurogenic amyotrophy, 54 myopathy including 19 progressive muscular dystrophy (PMD), and 10 controls whose muscle was obtained at orthopedic surgery. Neurogenic amyotrophy consisted of 12 amyotrophic lateral sclerosis (ALS), 4 spinal muscular atrophy and 7 peripheral nerve disorders. Myopathy were 3 Duchenne dystrophy, 2 congenital muscular dystrophy, 8 limb-girdle type dystrophy, 6 facio-scapular +-humeral muscular dystrophy, 6 myotonic dystrophy, 6 mitochondrial myopathy, 5 endocrinological myopathy, 3 hypokalemic myopathy, 8 polymyositis and 4 other inflammatory myopathy. The assays of G6PDH and RNA were performed after Glock's and Fleck's methods, respectively. The control values were 3.6 +/- 0.8 nmol formed NADPH/mg protein/min (M +/- SD) in G6PDH and 0.69 +/- 0.17 micrograms/mg non-collagen protein in RNA. Most cases of PMD, as well as some cases of ALS, hyperthyroidism, mitochondria hypokalemic myopathy, inflammatory myopathy showed increased values (beyond M + 2SD of control) both in G6PDH and RNA. There were significant positive correlations between G6PDH activity and RNA content in PMD and motor neuron disease. Myotonic dystrophy showed normal values in both G6PDH and RNA. Half number of cases of mitochondrial myopathy demonstrated increased G6PDH alone.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
14.
S Vivekanandan A P Rao M M Sampathkumar T S Kanaka 《Journal of the neurological sciences》1983,59(1):13-19
The authors report the muscular and humoral immunological abnormalities found in a family with progressive external ophthalmoplegia (PEO) of the “pure” form. Serum circulating immune complexes as determined by the polyethylen glycol (PEG) test and double radial immunodiffusion (DRID) were positive for IgG in both cases studied and for IgM and Clq for the propositus. In the latter circulating auto-antibodies against smooth muscle were also present. Immunohistochemical studies on striated muscle of the propositus showed positive perivascular IgG and IgM staining and IgG in the sarcolemma-basement membrane complex. It is suggested that in this family a genetically inherited abnormal immune response to the muscular blood vessel wall has induced vascular injury and ultimately chronic ischemic muscular damage. This is consistent with the view that PEO is a clinical syndrome, i.e. the expression of various defects affecting primarily or secondarily the energy metabolism of the muscular tissue. 相似文献
15.
D W Kingma D L Feeback W A Marks G B Bobele R W Leech R A Brumback 《Journal of child neurology》1991,6(4):329-334
The diagnostic muscle biopsy finding in severe infantile spinal muscular atrophy (Werdnig-Hoffmann disease, SMA type 1) is considered to be large-group atrophy with isolated clusters of hypertrophic type I myofibers. We present a unique case of severe infantile spinal muscular atrophy with selective hypertrophy of type II myofibers. A male infant presented at age 2 months with breathing difficulties and by age 4 months was hypotonic and weak. Electromyography revealed denervation in all extremity muscles, and nerve conduction velocities were normal but with small compound muscle action potentials. Quadriceps muscle biopsy revealed many hypertrophied type II myofibers (myofibers with a mean least diameter of 25.4 microns). In contrast, the largest type I myofibers were 20 microns in least diameter (mean diameter, 14.9 microns), and there was a normal-size population of type II fibers (mean diameter, 15.7 microns). In addition, sheets of atrophic type I and type II fibers averaged 2.0 microns in least diameter. Sural nerve biopsy was normal. Breathing difficulties progressed, with death ensuing at age 5 1/2 months. Autopsy revealed atrophy of ventral spinal roots with normal dorsal roots. There was loss of anterior horn cells, while remnant neurons were reduced in size. No other pathologic changes were identified. This case indicates that in severe infantile spinal muscular atrophy, relative sparing of the motor units with type II myofibers may occur. 相似文献
16.
We report two connatal cases of Pelizaeus-Merzbacher disease (PMD) with cellular ultraviolet (UV)-hypersensitivity. We studied the UV-sensitivity of cultured fibroblast cells derived from these PMD cases, as compared with UV-sensitive Cockayne syndrome (CS) and xeroderma pigmentosum (XP) cells as positive controls. The ability of the PMD cells to form colonies after UV irradiation was intermediate between those of CS cells and normal controls. There were no differences in both colony-forming ability after x-ray irradiation and unscheduled DNA synthesis (UDS) activity after UV irradiation between the PMD cells and the control cells. These cytological results suggest the possibility that a DNA defect might be involved in PMD. 相似文献
17.
Kenji Mokuno Shigeo Riku Yukihiko Matsuoka Itsuro Sobue Kanefusa Kato 《Journal of the neurological sciences》1984,63(3):345-352
Serum muscle-specific enolase (MSE, ββ and αβ enolases) levels were determined in 162 patients with progressive muscular dystrophy (PMD) and other neuromuscular diseases by means of an enzyme immunoassay method. The relationships were examinated between serum MSE, creatine kinase (CK) and other markers of muscle disease.Serum MSE was strikingly increased in Duchenne muscular dystrophy, and this elevation was more prominent in younger patients. Serum MSE was also increased in other types of PMD and certain other diseases. Serum MSE showed the highest correlation with CK. In the PMD group, the frequency of cases with elevated MSE was the same as in CK.These results indicated that serum MSE may well be a specific marker of muscle disease on a par with CK. 相似文献
18.
Miles L Miles MV Tang PH Horn PS Wong BL DeGrauw TJ Morehart PJ Bove KE 《Pediatric neurology》2005,32(5):318-324
The aim of this study is to determine whether coenzyme Q (CoQ) muscle concentrations and redox state are associated with pathologic changes in muscle biopsy specimens. Skeletal muscle biopsies were collected (January 2002-February 2004) and underwent pathologic evaluation. Quadriceps specimens (n = 47) were stratified accordingly: Group 1, controls without evidence of pathologic abnormalities; Group 2, type I myofiber predominance; Group 3, type II myofiber atrophy; Group 4, lower motor unit disease; and Group 5, muscular dystrophy. Ubiquinol-10, ubiquinone-10, total coenzyme Q10 (CoQ10), coenzyme Q9 (CoQ9), total CoQ (CoQ9+CoQ10) concentrations were analyzed in biopsy muscle by high-performance liquid chromatography. Ubiquinone-10, total CoQ10, and total CoQ concentrations were significantly decreased in Group 5. Significant positive correlations (r congruent with 0.40) were found between muscle ubiquinone-10, total CoQ10, and total CoQ concentrations vs the percentage of myofibers having subsarcolemmal mitochondrial aggregates. CoQ redox ratio and the fraction CoQ9/total CoQ were negatively correlated with subsarcolemmal mitochondrial aggregates. A significant correlation (r = 0.328) also occurred between ubiquinol-10 concentration and citrate synthase activity. This study suggests that total CoQ concentration provides a new method for estimating mitochondrial activity in biopsy muscle; and that the muscle CoQ test is feasible and potentially useful for diagnosing CoQ deficiency states. 相似文献
19.
C-protein isoform expression in hereditary dystrophic chicken skeletal muscle was compared with that in normal chicken muscle during postnatal development by immunocytochemical and immunoblot methods. In the pectoralis muscle (PM) of both normal and dystrophic chicken, slow- and fast-type C-proteins were coexpressed in the vast majority of myofibers at neonatal age, but the slow C-protein disappeared, leaving continued expression of only the fast-type C-protein as muscle development progressed up to 2 weeks posthatch. In the dystrophic chicken PM, however, myofibers containing slow-type C-protein reappeared about 1 month posthatch and increased in number with the progression of muscular dystrophy. We conclude that C-protein isoform expression in dystrophic myofibers resembles that in neonatal myofibers and that the expression of slow-type C-protein can be seen as a marker for chicken muscular dystrophy. 相似文献
20.
Margarita VainieneM.D. Anna Czonkowska Janina Korlak 《Acta neurologica Scandinavica》1983,67(5):275-284
Cell-mediated and humoral immunity were studied in 30 patients with subacute sclerosing panencephalitis (SSPE). Marked changes in cell-mediated immunity were observed, manifested as a decrease in total lymphocyte count, decrease of proportion of T cells forming late E rosettes, depression in lymphocyte blastogenesis, production of migration inhibition factor and delayed-type skin reactivity. The humoral immune responses was not so markedly changed and only increased levels of serum IgG, IgM and the C3c fragment of complement were noticed. The changes in immunity were more pronounced in patients with a more advanced stage of the disease. 19 patients were investigated 3 times at 7-week intervals during treatment with isoprinosine. In the group of patients (10 cases) whose clinical status was rapidly deteriorating, a marked decline of cell-mediated immunity was observed. In the group of patients showing a stationary course of the disease, cellular immunity was improving. The mechanism of the disturbances of non-specific immunity in SSPE and the influence of isoprinosine on the immune answer are discussed. 相似文献