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1.
肌萎缩侧索硬化105例临床分析   总被引:7,自引:0,他引:7  
目的:探讨肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)的临床特点,为进一步的病因、治疗研究提供临床资料。方法:回顾性研究1980-2000年我院收住的105例ALS患者,对其发病特点、症状、体征及实验室检查进行统计分析。结果:ALS平均发病年龄42.7岁,较国外早,60岁以后男女发患者数均明显减少;男女发病比例2:1,上肢远端为最常见的起病部位,球部起病者发病年龄晚,女性明显多于男性;ALS的发展一般从起病部位水平或垂直的累及邻近的运动神经元。结论:ALS中年发病为主,男性多见,其发展遵循一定的规律。  相似文献
2.
目的:探讨脐血间质干细胞移植对肌萎缩侧索硬化(ALS)患者神经系统功能的影响.方法:移植脐血间质干细胞治疗8例ALS患者,选用"神经康复功能评定系统-V2003"软件对治疗前3 d、治疗后2~3月ALS患者的肢体运动(Carr-Shephard 评分)、构音障碍(Frenchay评分)、平衡能力(Berg评分)、功能独立性评定(FIM)各方面的功能进行评估.结果:移植治疗前ALS患者Carr-Shephard、Frenchay、Berg、FIM评分与治疗后比较,差异均有统计学意义(P<0.05).结论:脐血间质干细胞移植治疗可以显著改善ALS患者的神经系统功能.  相似文献
3.
肌萎缩侧索硬化患者膈神经传导的临床应用及特点   总被引:3,自引:1,他引:2  
Zhuang L  Tang X  Xu X  Li B  Du H  Jiang J 《中华医学杂志》2002,82(3):152-154
目的 探讨膈神经传导在肌萎缩侧索硬化 (ALS)患者中的临床应用及特点。方法 以表面电极在肋间隙处记录电刺激 4 4例ALS患者及 31例正常对照者颈部膈神经时产生的膈肌复合肌肉动作电位之潜伏期和波幅。 2 8例ALS患者同时接受了用力肺活量百分比 (%FVC)测定。结果 ALS患者右、左侧膈神经远端运动潜伏期 (PDML)分别为 8 4ms± 2 2ms和 7 6ms± 1 4ms ,均较对照者延长 ,波幅对数为 2 6 8± 0 37,比对照者低 ;以右侧为例 ,PDML与 %FVC相关 ,但与临床呼吸困难无关 ;波幅与 %FVC和临床呼吸困难均无关。PDML阳性率 (47 7% )高于临床呼吸困难出现率(2 5 0 % )。结论 PDML是膈神经传导参数中反映ALS患者呼吸功能障碍的敏感指标 ,但只有将其与膈肌运动诱发电位的中枢运动传导时间结合 ,方能更全面揭示呼吸受累的本质  相似文献
4.
补肾健脾疏肝法治疗肌萎缩侧索硬化症临床疗效观察   总被引:3,自引:0,他引:3  
目的:探讨补肾健脾疏肝法治疗肌萎缩侧索硬化症的临床疗效。方法:80例肌萎缩侧索硬化症患者随机分为治疗组(n=40)和对照组(n=40),治疗组给予补肾健脾疏肝法的自拟方治疗,对照组给予西药力鲁唑治疗,疗程均为6个月。分别于治疗前、治疗后观察并比较两组患者的ALS功能量表评分及痿病中医证候积分,评价两组患者的临床疗效。结果:①在改善ALS功能量表评分方面,治疗组与对照组的总有效率分别为10.0%和17.5%,两组比较差异无统计学意义(P>0.05);在改善痿病中医证候方面,治疗组与对照组的总有效率分别为55.0%和30.0%,治疗组疗效优于对照组(P<0.05)。②两组治疗前后ALS功能量表评分组内比较,其差异均不具有统计学意义(P>0.05);两组治疗前后评分差值比较,其差异亦不具有统计学意义(P>0.05)。③治疗后,治疗组痿病中医证候总积分及次要症状积分较治疗前均显著降低(P<0.05),且两组治疗前后总积分及次要症状积分差值比较,差异亦均具有统计学意义(P<0.05)。结论:补肾健脾疏肝法在延缓ALS功能量表评分下降趋势方面的效果与西药相似,且能有效改善痿病中医证候,尤其是对次要症状的改善效果更加明显。  相似文献
5.
目的:通过对以往诊断为肌萎缩侧索硬化症(amyotrophiclateralsclerosis,ALS)的48例患者做回顾性重新检查,以探讨肌萎缩侧索硬化症(amyotrophiclateralsclerosis,ALS)和脊髓型颈椎病(cervicalspondylotic myelopathty,CSM)的相互关系。方法:对误诊的48例ALS的患者进行详细的神经系统、临床症状、体征、CT、MRI及神经电生理学检查,同时行相关因素分析。结果:误诊的48例ALS的患者,其中22例患者确诊CSM,18例患者CSM和ALS共存,8例维持原诊断。结论:神经电生理检查是CSM和ALS鉴别的关键;CSM和ALS共存不是偶然的,CSM可能是ALS疾病的重要病因。  相似文献
6.
Objective To study the activation changes of the brain in patients with amyotrophic lateral sclerosis (ALS) while executing sequential finger tapping movement using the method of blood oxygenation level dependent (BOLD) functional magnetic resonance imaging (tMRI). Methods Fifteen patients with definite or probable ALS and fifteen age and gender matched normal controls were enrolled. MRI was performed on a 3.0 Tesla scanner with standard headcoiL The functional images were acquired using a gradient echo single shot echo planar imaging (EPI) sequence. All patients and normal subjects executed sequential finger tapping movement at the frequency of 1-2 Hz during a block-design motor task. Structural MRI was acquired using a three-dimensional fast spoiled gradient echo (3D-FSPGR) sequence. The tMRI data were analyzed by statistical parametric mapping (SPM). Results Bilateral primary sensorimotor cortex ( PSM), bilateral premotor area ( PA), bilateral supplementary motor area (SMA), bilateral parietal region ( PAR), contralateral inferior lateral premotor area ( ILPA), and ipsilateral cerebellum showed activation in both ALS patients and normal controls when executing the same motor task. The activation areas in bilateral PSM, bilateral PA, bilateral SMA, and ipsilateral cerebellum were significantly larger in ALS patients than those in normal controls ( P 〈 0.05 ). Extra activation areas including ipsilateral ILPA, bilateral posterior limb of internal capsule, and contralateral cerebellum were only detected in ALS patients. Conclusions Similar activation areas are activated in ALS patients and normal subjects while executing the same motor task. The increased activation areas in ALS patients may represent neural reorganization, while the extra activation areas in ALS patients may indicate functional compensation.  相似文献
7.
运动神经元病合并额颞痴呆1例报告   总被引:2,自引:0,他引:2       下载免费PDF全文
1临床资料 男性患者,57岁,因"消瘦、行为异常1年半"于2008年6月1日入院.1年半以来家人发现患者消瘦,以双上肢变细明显,左侧重于右侧,同时出现行为异常,表现为与家人通电话时未讲完而突然挂断、频繁无故外出,外出后可自行回家,不能正常完成工作,并有理解力、计算力下降;后逐渐出现言语减少、舌肌发硬、发音含糊,并有饮水呛咳、痰量、唾液增多.病程中患者无头痛、头晕,无恶心、呕吐,无肢体麻木感,无随地大小便等异常行为,饮食睡眠可,大便便秘,小便正常,体质量减轻约15 kg.既往体健,无家族遗传病史.  相似文献
8.
Background  Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive death of the upper and lower motor neurons. Transgenic mice over-expressing a mutant form of the human SOD1 gene develop an ALS-like phenotype. Currently, there is no effective treatment or drug for the fatal disease. Previous studies reported potent efficacy of dl-3-n-butylphthalide (DL-NBP) for several neurodegenerative disorders and cerebral ischemia. SOD1-G93A mice are a mouse model of ALS. In this study, we investigated the efficacy of DL-NBP on this ALS mouse model.
Methods  Sixty SOD1-G93A female mice were divided into four groups. The vehicle control group received 0 mg∙kg-1∙d-1 DL-NBP. The experimental groups received DL-NBP with doses of 30, 60 or 120 mg∙kg-1∙d-1, respectively. For measurement of motor activity, the hanging wire test and rotarod test were performed. Survival statistics were analyzed by Kaplan-Meier survival curves. The body weight of each mouse was recorded twice per week. The statistical motor unit number estimation (MUNE) technique was used to estimate the number of functioning motor units in gastrocnemius muscle. Muscle morphology was evaluated by hematoxylin and eosin staining. Motor neuron quantitation was performed by Nissl staining and microglia activation was observed by immunohistochemistry.
Results  Oral administration of 60 mg∙kg-1∙d-1 DL-NBP significantly prolonged survival ((164.78±16.67) days) of SOD1-G93A mice compared with vehicle control ((140.00±16.89) days). Treating mice with DL-NBP (60 mg∙kg-1∙d-1) significantly decreased the progression rate of motor deficits and suppressed body weight reduction. Furthermore, we found that treating SOD1-G93A mice with DL-NBP (60 mg∙kg-1∙d-1) slowed the rate of MUNE reduction (P <0.01). Motor neurons were remarkably preserved in the anterior horns in mice treated with DL-NBP (60 mg∙kg-1∙d-1) at the stage of 19 weeks (P <0.01). Treating mice with DL-NBP (60 mg∙kg-1∙d-1) significantly reduced CD11b immunoreactivity compared with vehicle control mice (P <0.05). No significant effect was observed in mice treated with DL-NBP of 30 or 120 mg∙kg-1∙d-1.
Conclusions  The post-disease-onset administration of DL-NBP significantly prolonged survival and improved motor performance in SOD1-G93A mice. DL-NBP may be a potential therapeutic agent for ALS.
  相似文献
9.
Background  RNA interference (RNAi) is a potential cure for amyotrophic lateral sclerosis (ALS) caused by dominant, gain-of-function superoxide dismutase 1 (SOD1) mutations. The success of such therapy relies on the functional small interfering RNAs (siRNAs) that can effectively deliver RNAi. This study aimed to design the functional siRNAs targeting ALS-associated mutant alleles.
Methods  A modified dual luciferase system containing human SOD1 mRNA target was established to quantify siRNA efficacy. Coupled with validated siRNAs identified in the literature, we analyzed the rationale of siRNA design and subsequently developed an asymmetry rule-based strategy for designing siRNA. We then further tested the effectiveness of this design strategy in converting a naturally symmetric siRNA into functional siRNAs with favorable asymmetry for gene silencing of SOD1 alleles.
Results  The efficacies of siRNAs could vary tremendously by one base-pair position change. Functional siRNAs could target the whole span of SOD1 mRNA coding sequence as well as non-coding region. While there is no distinguishable pattern of the distribution of nucleobases in these validated siRNAs, the high percent of GC count at the last two positions of siRNAs (P18 and P19) indicated a strong effect of asymmetry rule. Introducing a mismatch at position 1 of the 5′ of antisense strand of siRNA successfully converted the inactive siRNA into functional siRNAs that silence SOD1 with desired efficacy. 
Conclusions  Asymmetry rule-based strategy that incorporates a mismatch into siRNA most consistently enhances RNAi efficacy and guarantees producing functional siRNAs that successfully silence ALS-associated SOD1 mutant alleles regardless target positions. This strategy could also be useful to design siRNAs for silencing other disease-associated dominant, gain-of-function mutant genes.
  相似文献
10.
慢性镉中毒致大鼠肌萎缩侧索硬化症样改变的研究   总被引:1,自引:0,他引:1  
目的:探讨小剂量慢性镉中毒法建立大鼠肌萎缩侧索硬化症动物模型的可行性.方法:成年大鼠饮水慢性染镉.每天饮用含镉去离子水(150mg/L),记录每天饮水量,120 d后取材.采用神经电生理、光镜、电镜技术测定大鼠坐骨神经传导速度、股二头肌形态学变化、脊髓前角运动神经元形态学变化;同时采用原子吸收石墨炉法、生物化学技术测定脊髓内镉含量以及脊髓组织细胞内铜锌超氧化物歧化酶1(CuZn-SOD_1)活性、丙二醛含量的变化.结果:染镉后120d,大鼠运动神经传导速度减慢但股二头肌纤维直径变化不明显;脊髓前角运动神经元胞体平均截面积有变小、神经元胞浆内粗面内质网排列紊乱并出现核糖体脱颗粒现象;染镉后脊髓内镉含量增加、脊髓组织匀浆内CuZn-SOD_1酶活性降低,而丙二醛含量升高.结论:镉可以在成年大鼠脊髓内蓄积,导致前角运动神经元形态和功能损伤.提示慢性镉中毒法可用于建立大鼠肌萎缩侧索硬化症的动物模型.  相似文献
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