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Myasthenia Gravis and Related Disorders Book

Myasthenia Gravis and Related Disorders
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Myasthenia Gravis and Related Disorders, Basic and clinical investigators have contributed reports to this volume in which they explore new findings in myasthenia gravis and related diseases, exploring topics in basic science, translational research, and clinical science, including advances in t, Myasthenia Gravis and Related Disorders
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  • Myasthenia Gravis and Related Disorders
  • Written by author Kaminski, Henry J
  • Published by Springer-Verlag New York, LLC, 1/19/2011
  • Basic and clinical investigators have contributed reports to this volume in which they explore new findings in myasthenia gravis and related diseases, exploring topics in basic science, translational research, and clinical science, including advances in t
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Pt. I Structure and Function of the Neuromuscular Junction

Structural Factors Influencing the Efficacy of Neuromuscular Transmission 1

Cell-to-Cell Signaling at the Neuromuscular Junction: The Dynamic Role of the Extracellular Matrix 13

The Role of Glial Cells in the Formation and Maintenance of the Neuromuscular Junction 19

Pt. II Structure and Function of the Acetylcholine Receptor

Myasthenia Gravis and the Tops and Bottoms of AChRs: Antigenic Structure of the MIR and Specific Immunosuppression of EAMG Using AChR Cytoplasmic Domains 29

Functional Organization and Conformational Dynamics of the Nicotinic Receptor: A Plausible Structural Interpretation of Myasthenic Mutations 42

Recent Structural and Mechanistic Insights into Endplate Acetylcholine Receptors 53

Peptide-Toxin Tools for Probing the Expression and Function of Fetal and Adult Subtypes of the Nicotinic Acetylcholine Receptor 61

Pt. III Muscle-Specific Kinase and Myasthenia Gravis

Clinical Phenotype of Muscle-specific Tyrosine Kinase-antibody-positive Myasthenia Gravis 71

Response to Therapy in Myasthenia Gravis with Anti-MuSK Antibodies 76

Myasthenia Gravis Seronegative for Acetylcholine Receptor Antibodies 84

Myasthenia Gravis Experimentally Induced with Muscle-specific Kinase 93

Pt. IV Congenital Myasthenic Syndromes

Congenital Myasthenic Syndromes and the Formation of the Neuromuscular Junction 99

Further Observations in Congenital Myasthenic Syndromes 1047

Pt. V Immunology of Autoimmune Disorders Involving Neuronal and Glial Channel Proteins

Anti-ganglioside Antibodies and the Presynaptic Motor Nerve Terminal 114

Neuronal Acetylcholine Receptor Autoimmunity124

Lambert-Eaton Myasthenic Syndrome: Tumor versus Nontumor Forms 129

Pt. VI Autoinunune Pathogenesis of Myasthenia Gravis: Current Concepts

Regulatory and Pathogenic Mechanisms in Human Autoimmune Myasthenia Gravis 135

Common Cellular and Diverse Genetic Basis of Thymoma-associated Myasthenia Gravis: Role of MHC Class II and AIRE Genes and Genetic Polymorphisms 143

How Promiscuity Promotes Tolerance: The Case of Myasthenia Gravis 157

Autoimmunizing Mechanisms in Thymoma and Thymus 163

Treatment of Myasthenia Gravis by Preventing Acetylcholine Receptor Modulation 174

Genetic Factors in Autoimmune Myasthenia Gravis 180

CD4+ T Cells and Cytokines in the Pathogenesis of Acquired Myasthenia Gravis 193

Classical Complement Pathway in Experimental Autoimmune Myasthenia Gravis Pathogenesis 210

Extraocular Muscle Susceptibility to Myasthenia Gravis: Unique Immunological Environment? 220

Pt. VII Clinical Trials of Myasthenia Gravis

Treatment and Clinical Research in Myasthenia Gravis: How Far Have We Come? 225

Rules of Evidence for Clinical Trials: The Science of Finding the Truth? 233

Myasthenia Gravis in the Elderly: Is It Different? 238

Immunosuppression of EAMG by IVIG Is Mediated by a Disease-specific Anti-immunoglobulin Fraction 244

Lessons from Two Trials of Mycophenolate Mofetil in Myasthenia Gravis 249

Tacrolimus for Myasthenia Gravis: A Clinical Study of 212 Patients 254

IVIG Treatment for Myasthenia Gravis: Effectiveness, Limitations, and Novel Therapeutic Strategies 264

Treatment of Myasthenia Gravis Acute Exacerbations with Intravenous Immunoglobulin 271

Pt. VIII Treatments on the Horizon for Myasthenia Gravis

Strategies for Treating Autoimmunity: Novel Insights from Experimental Myasthenia Gravis 276

Antisense Treatment for Myasthenia Gravis: Experience with Monarsen 283

Antigen-specific Apheresis of Pathogenic Autoantibodies from Myasthenia Gravis Sera 291

Etanercept Treatment in Myasthenia Gravis 300

Rebooting the Immune System with High-Dose Cyclophosphamide for Treatment of Refractory Myasthenia Gravis 305

Pt. IX Panel Discussion: Thymectomy for Myasthenia Gravis

Thymectomy for Nonthymomatous Myasthenia Gravis: A Critical Analysis 315

Thoracoscopic Thymectomy with the da Vinci Robotic System for Myasthenia Gravis 329

Extended Transcervical Thymectomy in the Treatment of Myasthenia Gravis 336

Status of the Thymectomy Trial for Nonthymomatous Myasthenia Gravis Patients Receiving Prednisone 344

List of Abstracts 348

Erratum for Ann. N.Y Acad. Sci. 1010: 441-445. Apoptosis: From Signaling Pathways to Therapeutic Tools, Marc Diederich, Ed. 387

Erratum for Ann. N.Y Acad. Sci. 1107: 308-318. Autoimmunity, Part C: The Mosaic of Autoimmunity, Yehuda Shoenfeld and M. Eric Gershwin, Eds. 388

Erratum for Ann. N.Y Acad. Sci. 1112: 140-153. Thymosins in Health and Disease, Allan L. Goldstein and Enrico Garaci, Eds 389

Erratum for Ann. NY Acad. Sci. 1113: 304-310. Stress Responses in Biology and Medicine: Stress of Life in Molecules, Cells, Organisms, and Psychosocial Communities, Peter Csermely, Tamas Korcsmaros, and Katalin Sulyok, Eds 390

Index of Contributors 391


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