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1.
肌萎缩侧索硬化症90例胸锁乳突肌肌电图的特点   总被引:24,自引:1,他引:23  
目的 探讨胸锁乳突肌肌电图 (EMG)检测在肌萎缩侧索硬化症 (ALS)诊断中的价值。方法 对ALS患者均进行常规EMG检测 ,包括上肢、下肢及胸锁乳突肌 ,测定自发电位 ,募集相 ,运动单位电位的时限、波幅及多相波。比较有、无球部症状的患者之间胸锁乳突肌EMG改变的差异 ,以及不同部位即球部、颈部及腰骶部之间EMG检测结果的差异。结果 有球部症状的患者胸锁乳突肌EMG病变程度重于无球部症状者 (时限增宽幅度分别为 60 6 %± 2 1 2 %和 50 0 %± 1 9 2 % ,P <0 0 5)。胸锁乳突肌EMG自发电位、募集相、运动单位电位时限和波幅改变的程度均不同于上肢和下肢 ,存在显著性差异。结论 胸锁乳突肌EMG检测的异常与球部症状相关 ,而与肢体无关 ,其神经源性改变可反映球部下运动神经元病变  相似文献
2.
对106例肌萎缩侧索硬化(ALS)与颈椎病性脊髓病(CSM),及两病鉴别困难者进行胸锁乳突肌、肢体肌及舌肌EMG检查。结果ALS组胸锁乳突肌神经源性损害的异常率高于三肢体肌、舌肌;CSM组胸锁乳突肌无1例异常。表明该肌神经源性损害能明显提高ALS亚临床的阳性率,有助于ALS的早期诊断及ALS与CSM两病的鉴别。  相似文献
3.
Riluzole inhibits the persistent sodium current in mammalian CNS neurons   总被引:18,自引:0,他引:18  
The effects of 0.1-100 microM riluzole, a neuroprotective agent with anticonvulsant properties, were studied on neurons from rat brain cortex. Patch-clamp whole-cell recordings in voltage-clamp mode were performed on thin slices to examine the effects of the drug on a noninactivating (persistent) Na+ current (INa,p). INa,p was selected because it enhances neuronal excitability near firing threshold, which makes it a potential target for anticonvulsant drugs. When added to the external solution, riluzole dose-dependently inhibited INa,p up to a complete blocking of the current (EC50 2 microM), showing a significant effect at therapeutic drug concentrations. A comparative dose-effect study was carried out in the same cells for the other main known action of riluzole, the inhibitory effect on the fast transient sodium current. This effect was confirmed in our experiments, but we found that it was achieved at levels much higher than putative therapeutic concentrations. Only the effect on INa,p, and not that on fast sodium current, can account for the reduction in neuronal excitability observed in cortical neurons following riluzole treatment at therapeutic concentrations, and this might represent a novel mechanism accounting for the anticonvulsant and neuroprotective properties of riluzole.  相似文献
4.
Recently, 43-kDa TAR DNA-binding protein (TDP-43) was identified as a component of ubiquitinated inclusions (UIs) in sporadic amyotrophic lateral sclerosis (SALS). To clarify whether TDP-43 immunoreactivity is present in neuronal inclusions in familial ALS (FALS), we examined immunohistochemically the brains and spinal cords from four cases of FALS, two with Cu/Zn superoxide dismutase (SOD1) gene mutation and two without, together with three cases of SALS and three control subjects, using two antibodies, one polyclonal and one monoclonal, against TDP-43. Neuropathologically, the SOD1-related FALS cases were characterized by Lewy body-like hyaline inclusions (LBHIs) in the lower motor neurons. On the other hand, the SOD1-unrelated FALS cases showed degeneration restricted to the upper and lower motor neuron systems, with Bunina bodies (BBs) and UIs in the lower motor neurons, being indistinguishable from SALS. No cytoplasmic TDP-43 immunoreactivity was observed in the control subjects or SOD1-related FALS cases; LBHIs were ubiquitinated, but negative for TDP-43. UIs observed in the SALS and SOD1-unrelated FALS cases were clearly positive for TDP-43. BBs were negative for this protein. Interestingly, in these SALS and FALS cases, glial cells were also found to have cytoplasmic TDP-43-positive inclusions. These findings indicate that the histological and molecular pathology of SALS can occur as a phenotype of FALS without SOD1 mutation.  相似文献
5.
Prosody is an important feature of language, comprising intonation, loudness, and tempo. Emotional prosodic processing forms an integral part of our social interactions. The main aim of this study was to use bold contrast fMRI to clarify the normal functional neuroanatomy of emotional prosody, in passive and active contexts. Subjects performed six separate scanning studies, within which two different conditions were contrasted: (1) "pure" emotional prosody versus rest; (2) congruent emotional prosody versus 'neutral' sentences; (3) congruent emotional prosody versus rest; (4) incongruent emotional prosody versus rest; (5) congruent versus incongruent emotional prosody; and (6) an active experiment in which subjects were instructed to either attend to the emotion conveyed by semantic content or that conveyed by tone of voice. Data resulting from these contrasts were analysed using SPM99. Passive listening to emotional prosody consistently activated the lateral temporal lobe (superior and/or middle temporal gyri). This temporal lobe response was relatively right-lateralised with or without semantic information. Both the separate and direct comparisons of congruent and incongruent emotional prosody revealed that subjects used fewer brain regions to process incongruent emotional prosody than congruent. The neural response to attention to semantics, was left lateralised, and recruited an extensive network not activated by attention to emotional prosody. Attention to emotional prosody modulated the response to speech, and induced right-lateralised activity, including the middle temporal gyrus. In confirming the results of lesion and neuropsychological studies, the current study emphasises the importance of the right hemisphere in the processing of emotional prosody, specifically the lateral temporal lobes.  相似文献
6.
TDP-43 in differential diagnosis of motor neuron disorders   总被引:15,自引:13,他引:2  
Motor neuron disorders are clinically and pathologically heterogeneous. They can be classified into those that affect primarily upper motor neurons, lower motor neurons or both. The most common disorder to affect both upper and lower motor neurons is amyotrophic lateral sclerosis (ALS). Some forms of motor neuron disease (MND) affect primarily motor neurons in the spinal cord or brainstem, while others affect motor neurons at all levels of the neuraxis. A number of genetic loci have been identified for the various motor neuron disorders. Several of the MND genes encode for proteins important for cytoskeletal stability and axoplasmic transport. Despite these genetic advances, the relationship of the various motor neuron disorders to each other is unclear. Except for rare familial forms of ALS associated with mutations in superoxide dismutase type 1 (SOD1), which are associated with neuronal inclusions that contain SOD1, specific molecular or cellular markers that differentiate ALS from other motor neuron disorders have not been available. Recently, the TAR DNA binding protein 43 (TDP-43) has been shown to be present in neuronal inclusions in ALS, and it has been suggested that TDP-43 may be a specific marker for ALS. This pilot study aimed to determine the value of TDP-43 in the differential diagnosis of MND. Immunohistochemistry for TDP-43 was used to detect neuronal inclusions in the medulla of disorders affecting upper motor neurons, lower motor neurons or both. Medullary motor neuron pathology also was assessed in frontotemporal lobar degeneration (FTLD) that had no evidence of MND. TDP-43 immunoreactivity was detected in the hypoglossal nucleus in all cases of ALS, all cases of FTLD-MND and some of cases of primary lateral sclerosis (PLS). It was not detected in FTLD-PLS. Surprisingly, sparse TDP-43 immunoreactivity was detected in motor neurons in about 10% of FTLD that did not have clinical or pathologic features of MND. The results suggest that TDP-43 immunoreactivity is useful in differentiating FTLD-MND and ALS from other disorders associated with upper or lower motor neuron pathology. It also reveals subclinical MND in a subset of cases of FTLD without clinical or pathologic evidence of MND.  相似文献
7.
胸段脊旁肌肌电图在肌萎缩侧索硬化诊断中的作用   总被引:15,自引:1,他引:14  
目的 研究胸段脊旁肌自发电位在诊断肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)中的价值。方法 50例确诊的ALS患者分别行胸段脊旁肌自发电位、胸锁乳突肌运动单位,部分患者行舌肌自发电位检测;同时,将性别、年龄相匹配的30例根性损害的患者和30名健康人作为对照,行胸段脊旁肌自发电位检测。结果 50例ALS患者中,41例(82%)胸段脊旁肌肌电图可见大量纤颤电位和正锐波;胸锁乳突肌肌电图无一例见自发电位,但有48例(96%)运动单位时限增宽,波幅增高,符合慢性神经源性损害改变;6例患者行舌肌自发电位检测,3例可见自发电位。30例根性病变的患者中,2例(7%)可见胸段脊旁肌有少量自发电位;健康对照组未见胸段脊旁肌自发电位。结论 胸段脊旁肌大量自发电位对诊断ALS具有一定的敏感性。  相似文献
8.
Dopamine inhibition of auditory nerve activity in the adult mammalian cochlea   总被引:14,自引:0,他引:14  
Efferent feedback systems provide a means for modulating the input to the central nervous system. The lateral olivocochlear efferents modulate auditory nerve activity via synapses with afferent dendrites below sensory inner hair cells. We examined the effects of dopamine, one of the lateral olivocochlear neurotransmitters, by recording compound and single unit activity from the auditory nerve in adult guinea pigs. Intracochlear application of dopamine reduced the compound action potential (CAP) of the auditory nerve, increased the thresholds and decreased the spontaneous and driven discharge rates of the single unit fibres without changing their frequency-tuning properties. Surprisingly, dopamine antagonists SCH-23390 and eticlopride decreased CAP amplitude as did dopamine. In some units, both SCH-23390 and eticlopride increased the basal activity of auditory nerve fibres leading to an improvement of threshold sensitivity and a decrease of the maximum driven discharge rates to sound. In other units, the increase in firing rate was immediately followed by a marked reduction to values below predrug rates. Because CAP reflects the summed activity of auditory nerve fibres discharging in synchrony, both the decrease in sound-driven discharge rate and the postexcitatory reduction account for the reduction in CAP. Ultrastructural examination of the cochleas perfused with eticlopride showed that some of the afferent dendrites were swollen, suggesting that the marked reduction in firing rate may reflect early signs of excitotoxicity. Results suggest that dopamine may exert a tonic inhibition of the auditory nerve activity. Removal of this tonic inhibition results in the development of early signs of excitotoxicity.  相似文献
9.
Activation of the satiety center by auricular acupuncture point stimulation   总被引:13,自引:0,他引:13  
Stimulation of the rat inner auricular regions that correspond to the human pylorus, lung, trachea, stomach, esophagus, endocrine, and heart acupuncture points evoked potentials in the hypothalamic ventromedial nucleus (HVM), the satiety center. Needle implantation into any of these points reduced the body weight to its initial 290 g after the rat had gained about 410 g in 20 days, and significantly reduced initial 450-g body weights (p less than 0.01, Student's t test) in 14 days. Stimulation of other acupuncture points did not evoke HVM potentials and did not reduce body weight. After the HVM was lesioned, body weight increased and acupuncture point needling had no effect on body weight. Needling of the auricular acupuncture points evoked no potentials in the lateral hypothalamus (LHA), the feeding center, and had almost no influence on weight reduction induced by LHA lesion.  相似文献
10.
Baldauf K  Reymann KG 《Brain research》2005,1056(2):158-167
The persistence of neurogenesis in the adult mammalian forebrain suggests that endogenous precursors may be a potential source for neuronal replacement after injury or neurodegeneration. On the other hand basic fibroblast growth factor (bFGF) and epidermal growth factor (EGF) can facilitate neural precursor proliferation in the adult rodent subventricular zone (SVZ) and dentate gyrus. As the application of EGF and bFGF was found to boost neurogenesis after global ischemia, in this study we investigated whether a combined intracerebroventricular (i.c.v.) EGF/bFGF treatment over a period of 2 weeks affects the proliferation of newly generated cells in the endothelin-1 model of transient focal ischemia in adult male Sprague-Dawley rats as well. As assessed by toluidine blue staining, EGF/bFGF substantially increased the infarct volume in ischemic animals. Chronic 5'-bromodeoxyuridine (BrdU) i.c.v. application revealed an EGF/bFGF-induced increase in cell proliferation in the lateral ventricle 14 days after surgery. Proliferation in the striatum increased after ischemia, whereas in the dentate gyrus and in the dorsal 3rd ventricle the number of cells decreased. Analysis of the neuronal fate of these cells by co-staining with a doublecortin (DCX) antibody showed that the growth factors concomitantly nearly doubled early neurogenesis in the ipsilateral striatum in ischemic animals but diminished it in the dentate gyrus. Because of the increased infarct volume and unclear long-term outcome further modifications of a chronic treatment schedule are needed before final conclusions concerning the perspectives of such an approach can be made.  相似文献
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