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1.
多发性肌炎和皮肌炎201例临床病理研究   总被引:1,自引:1,他引:5  
201例多发性肌炎和皮肌炎,根据Bohan分型标准分成五组类型,其临床上以肢体肌无力、皮肤损害、疼痛、血肌酶升高、肌电图改变、肌活检肌纤维坏死及炎性细胞浸润为特征,但不同类型有所侧重,揭示其发病机制不同。治疗体会仍以激素为首选,必要时加用免疫抑制剂,开始治疗时间不应晚于1.5年,防治肺部感染是改善预后的重要措施之一。  相似文献
2.
Celiac disease is usually associated with autoimmune disorders and has occasionally been reported in patients with inflammatory myopathies. Our aim was to determine the presence of celiac disease and antibodies associated with celiac disease in patients with inflammatory myopathies and to investigate their relationship. Serum antigliadin, anti-tissue transglutaminase, and antiendomysial antibodies were determined in 51 patients with inflammatory myopathies. HLA-DQ2 and -DQ8 alleles were studied to assess their complementary diagnostic value. Jejunal biopsy was performed in patients with moderate to high levels of antigliadin antibodies. Patients with jejunal histology consistent with celiac disease initiated a gluten-free diet. Seventeen patients (31%) were positive for antigliadin antibodies, which were significantly more frequent in patients with inclusion-body myositis than dermatomyositis (P < 0.001). Positive status to HLA-DQ2 and/or -DQ8 did not differ between antigliadin-positive (75% and 12.5%) or -negative (60% and 15%) patients. Three of five jejunal biopsies were diagnostic for celiac disease with histological normalization after a gluten-free diet. Thus, celiac disease is more prevalent in patients with inflammatory myopathies than in the general population. Positive status to HLA-DQ2 allele, which is known to be more frequent in patients with inflammatory myopathies, could explain the high prevalence of antigliadin antibodies in this population. The diagnostic value of HLA-DQ2 or -DQ8 haplotypes to detect celiac disease in patients with inflammatory myopathy is limited.  相似文献
3.
Summary Bizarre configurations of muscle mitochondria containing paracrystalline inclusions were demonstrated in a clinically and pathologically typical case of chronic polymyositis with Raynaud's syndrome. The probable mechanism of formation of these abnormal mitochondria was discussed. From the accompanied alterations in the same fibers, i.e. focal assembly of sarcoplasmic reticulum, membranous profiles, miniature mitochondria, and vesicular nuclei with prominent nucleoli, the overall phenomena were interpreted as a process of atypical regeneration.The detail of the subunits of the paracrystalline inclusions was described and considered to be filamentous structures arranged in double helix. Non-specificity of these mitochondrial abnormalities was evident in 19 other reported cases with a wide range of symptoms, signs, clinical diagnosis, age, and sex distribution. The mitochondrial alterationsper se do not, therefore, reflect any specific muscle disease. Observations of myxovirus-like structures in all 3 biopsy specimens in the present case is discussed in conjunction with the concurrent abnormal mitochondria.Presented at the scientific session of the annual meeting of the American Academy of Neurology, San Francisco, April, 1967.Supported in part by U. S. Public Health Service grants GM09387, G-256-6, and 5 SOI-FR-05433-07.  相似文献
4.
Summary 80% of cultures of foetal human and rat muscle underwent destruction when incubated with lymphocytes from patients with polymyositis compared with 30% of those incubated with serum from patients with polymyositis and 25% of those incubated with lymphocytes from patients with other neurological disorders. Cultures of dural fibroblasts were also destroyed in the presence of lymphocytes from patients suffering from polymyositis associated with collagen disorders. These effects may be the sequel to a specific sensitization of lymphocytesin vivo and the findings are therefore consistent with a mechanism of cellmediated hypersensitivity underlying polymyositis.The work was aided by grants from the Medical Research Council, the Muscular Dystrophy Association of America, Inc., and the Muscular Dystrophy Group of Great Britain. The author would like to thank Miss Aileen Brown for her advice and technical assistance, his co-workers. Dr. Michael Saunders and Mr. Malcolm Knowles of the M.R.C. Demyelinating Diseases Research Unit, the physicians of the region for allowing him to study their patients and Prof. John N. Walton for his advice and encouragement.  相似文献
5.
Summary Muscle biopsies from two cases of polymyositis were examined with the electron microscope, particular attention being given to the inflammatory cells in the lesions. Lymphocytes which were enlarged and possibly activated were found in contact with the endothelium of small venules; they were also seen apparently traversing the vessel wall. Venular endothelial cells were often swollen with reduplication of the basement membrane. Perivascular and interstitial cells comprised small lymphocytes, larger transforming lymphocytes with distinctive morphological features, pleomorphic macrophages and a few plasma cells and granulocytes. In one case cells resembling primitive lymphoid cells were found internal to the basement membrane of several muscle fibres. Peripheral blood lymphocytes from both patients were stimulated on incubation with a crude muscle homogenate. The lymphocytes also appeared to be cytotoxic to cultures of foetal human striated muscle. The significance of these findings is considered in relationship to the pathogenesis of the muscle fibre damage in polymyositis.
Zusammenfassung Muskelbiopsien von 2 Polymyositisfällen wurden elektronenmikroskopisch untersucht, wobei den Entzündungszellen in den Läsionen besondere Aufmerksamkeit geschenkt wurde. Vergrößerte, möglicherweise aktivierte Lymphocyten fanden sich in Kontakt mit dem Endothel kleiner Venolen; man traf sie auch beim Durchtritt durch die Gefäßwand an. Die Endothelzellen der Venolen waren oft vergrößert, die Basalmembran verdoppelt. Die perivasculären und interstitiellen Zellen rekrutierten sich aus kleinen Lymphocyten, größeren Lymphocyten mit den deutlichen morphologischen Zeichen der übergangsformen, pleomorphen Makrophagen sowie einigen Plasmazellen und Granulocyten. Bei einem Fall wurden innerhalb der Basalmembran mehrerer Muskelfasern Zellen gefunden, die primitiven lymphoiden Zellen ähnelten. In der Inkubation mit einem Muskelhomogenat wurden Lymphocyten vom peripheren Blut beider Patienten stimuliert. Die Lymphocyten erwiesen sich auch gegen Kulturen von fetalem quergestreiften menschlichen Muskel als cytotoxisch. Die Bedeutung dieser Befunde wird in Hinblick auf die Pathogenese der Muskelfaserschädigung bei Polymyositis erwogen.
  相似文献
6.
Summary Electron microscopic examination of a biopsy of skeletal muscle from a young girl with chronic polymyositis revealed the presence of sarcoplasmic and perinuclear inclusions.A large number of virus-like particles was demonstrated at the surface of muscle cells and in extracellular space. On the basis of the microscopic appearance it is suggested that the virus particles belong to one of the members of the paramyxovirus group.These studies are part of a research project supported by NIH Bethesda Md (U.S.A.) under agreement No. 05-002-1.  相似文献
7.
19 biopsies of polymyositis patients were compared with 19 matched controls. The presence of smaller fibres in the periphery of the fascicles has been analyzed quantitatively using a perifascicular atrophy factor. The thinner fibres are multiplied by a factor from 1-4, considering their significance for the diagnosis of fibre atrophy. The value obtained with this method from centrally located fibres as related to the value from peripherally located ones is called the perifascicular atrophy factor. If this is less than -300 a myopathy of the group of the polymyositis/dermatomyositis can be assumed. 47 per cent of dermatomyositis biopsies and none of the controls were below this range  相似文献
8.
Summary Lymphocytes of twenty-seven patients with polymyositis were incubated in vitro with cholinergic receptor rich membranes obtained from the electric organs of Torpedo Marmorata.Lymphocytes of polymyositic patients were slightly stimulated; positive responses were present mainly in patients affected from more than a year. Sensitization against the nicotinic cholinergic receptor may explain the occurrence of the myasthenic syndrome with polymyositis.Fellow of the A. Villa Rusconi foundation  相似文献
9.
Zusammenfassung Die Serum-Myoglobinkonzentration wurde mit einem empfindlichen Radioimmunoassay bei gesunden Kontrollpersonen und Patienten mit Polymyositis und progressiver Muskeldystrophie vom Typ Duchenne bestimmt. Die gemessenen Konzentrationen korrelieren eng mit der Serum-Kreatin-Kinase, so daß die Myoglobinkonzentration im Serum als nützlicher Parameter bei der Beurteilung von Muskelzellschäden herangezogen werden kann.Herrn Prof. Dr. H. G. Mertens zum 60. Geburtstag gewidmet  相似文献
10.
Twelve patients with polymyositis or dermatomyositis were examined repeatedly during the course of the illness by muscle strength tests, serum enzyme determinations, frequency analysis of the electromyographic signal, and estimations of the spontaneous activity. All methods can be used to follow the course of the disease. Increasing muscle strength was correlated to decreasing serum enzyme values, decreasing spontaneous activity, and decreasing proportion of high-frequency components in the EMG signal, except during the initial period of treatment, when a temporary increase in high-frequency components was recorded.  相似文献
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