ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Muscle biopsy remains a critical component in the evaluation of myopathies. Recent literature demonstrates that percutaneous biopsy instruments can effectively procure muscle tissue for analysis in clinical and research settings. We review issues of muscle biopsy technique and specimen processing in the context of available clinical methods for tissue analysis. A survey of recent literature illustrates contributions made by the histologic, histochemical, and ultrastructural aspects of muscle analysis to our understanding of the pathophysiologic mechanisms and clinical manifestations of selected inflammatory, mitochondrial, and infectious myopathies.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Two distinct areas of development contribute to the importance of enzymology in skeletal muscle disease: the measurement of enzymes in serum as a diagnostic aid and the investigation of enzyme deficiencies as the cause of metabolic myopathy. Currently, serum enzyme measurement, particularly creatine kinase, is used to aid in the detection of suspected myopathy, to differentiate myopathy from neurogenic disease, to identify dystrophies at a preclinical stage, to detect female carriers of dystrophies, and to assess response to therapy. Metabolic myopathies are disorders of muscle energy production that result in skeletal muscle dysfunction. The biochemical basis of such disorders is often a specific single enzyme defect. The tremendous advances in the molecular genetics of such disorders has added remarkably to our understanding of the primary defects involved and possible heterogeneity displayed at the molecular level.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Muscular dystrophies primarily affect skeletal muscle and are characterized by progressive muscle wasting and weakness. Although these diseases have been clinically recognized for some time, genetic defects in a number of muscular dystrophies only recently have been identified. One of the most important advances in understanding the molecular genetics of neuromuscular diseases has been the cloning of the gene encoding dystrophin, the protein that is absent in the muscle of patients with Duchenne and Becker muscular dystrophy. Several dystrophin-associated proteins have been identified. Components of the dystrophin-glycoprotein complex are being characterized, and evidence indicates that proteins of this complex may be responsible for other forms of muscular dystrophy.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

This review emphasizes new advances in seeking the pathogenic mechanisms of sporadic inclusion-body myositis and hereditary inclusion-body myopathy syndromes. Clinical and pathologic similarities and differences between sporadic and hereditary forms are described. Hypotheses are presented regarding the possible causes and consequences of abnormally accumulated intramyofiber β-amyloid precursor protein (pAPP) (including β-amyloid protein and C- and N-terminal epitopes), hyperphosphorylated tau, α1-antichymotrypsin, apolipoprotein E, prion protein, ubiquitin, nicotinic acetylcholine receptor and its 43-kD associated protein, fibroblast growth factor, and transforming growth factor-β. Also increased are PAPP mRNA and prion protein mRNA. Striking similarities between the pathology of muscle specimens from sporadic inclusion-body myositis and samples from the brains of patients with Alzheimer's disease in regard to Congo red positivity and accumulations of several proteins are discussed. Because most of the proteins that pathologically accumulate throughout the abnormal muscle fibers also accumulate focally at normal human neuromuscular junctions, the possible “junctionalization” of nonjunctional nuclei as a pathogenic mechanism in the muscle fiber is discussed.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Skeletal muscle involvement may occur at all stages of HIV infection. The most simple classification of muscular disorders in HIV-infected patients is 1) HIV-associated myopathies, 2) zidovudine myopathy, 3) HIV wasting syndrome, and 4) opportunistic infections and tumoral infiltrations of muscle. Immunohistology for major histocompatibility complex class I antigen and histochemical reaction for cytochrome c oxidase are helpful in correctly classifying a myopathy as HIV polymyositis or zidovudine myopathy. Studies of cytokine expression in HIV-infected patients and of supplementation with compounds such as carnitine or micronutrients such as selenium might yield new insights into the pathogenesis and treatment of the various AIDS-associated muscular disorders.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Advances in molecular biologic techniques and the availability of novel immunologic reagents have allowed new approaches to understanding the pathogenesis of human autoimmune diseases, including the idiopathic inflammatory myopathies. Indirect evidence that autoreactive T cells mediate muscle inflammation in the human myositis syndromes has been strengthened by recent studies describing restricted T cell receptor gene expression in certain clinical and/or serologic groups of myositis patients. These findings are supported by other investigations documenting abnormal patterns of cytokine, adhesion molecule, and major histocompatibility complex antigen expression within inflammatory lesions. The major challenge of future studies is to identify the specific antigen(s) responsible for initiating and perpetuating these harmful immune responses.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Intravenous immunoglobulin (IVIg) is a new modality used to help treat conditions associated with immune dysregulation. The inflammatory myopathies are a group of complex diseases including dermatomyositis, polymyositis, and inclusion-body myositis. Overall evaluation of IVIg in myopathy has been hampered by difficulty in accurately diagnosing and assessing disease activity. The lack of large, well controlled, double-blind trials has precluded clear evaluation of the effectiveness of IVIg in these diseases. However, from the data presented in published reports, it appears that IVIg may be useful in the treatment of inflammatory myopathies, particularly dermatomyositis.

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID

ISSN:1040-8711    

出版商:OVID    

年代:1995

数据来源: OVID