Myasthenia Gravis and Related Disorders
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Table of Contents

Preface: Henry J. Kaminski and Richard J. Barohn.

Part I: Structure and Function of the Neuromuscular Junction:.

1. Structural Factors Influencing the Efficacy of Neuromuscular Transmission: Clarke Slater.

2. Cell to Cell Signaling at the Neuromuscular Junction: The Dynamic Role of the Extracellular Matrix: Michael Werle.

3. The Role of Glial Cells in the Formation and Maintenance of the Neuromuscular Junction: Zhihua Feng and Chien-Ping Ko.

Part II: Structure and Function of the Acetylcholine Receptor:.

4. Myasthenia Gravis and the Tops and Bottoms of AChRs: Antigenic Structure of the MIR and Specific Immunosuppresssion of EAMG Using AChR Cytoplasmic Domains: Jon Lindstrom.

5. Functional Organization and Conformational Dynamics of the Nicotinic Receptor: A Plausible Structural Interpretation of Myasthenic Mutations: Antoine Taly and Jean Pierre Changeux.

6. Recent Structural and Mechanistic Insights into Endplate Acetylcholine Receptors: Steven M. Sine, Fan Gao, Won Yong Lee, Nuriya Mukhtasimova, Hai-Long Wang and Andrew G. Engel.

7. Peptide-Toxin Tools for Probing the Expression and Function of Fetal and Adult Subtypes of the Nicotinic Acetylcholine Receptor: Russell W. Teichert, Carmen C. Garcia, Joseph G. Potian, James J. Schmidt, Veit Witzemann, Baldomero M. Olivera, and Joseph J. McArdle.

Part III: Muscle-Specific Kinase and Myasthenia Gravis:.

8. Clinical Phenotype of Muscle-Specific Tyrosine Kinase Antibody–Positive Myasthenia Gravis: Gil I. Wolfe and Shin J. Oh.

9. Response to Therapy in Myasthenia Gravis with Anti-MuSK Antibodies: Amelia Evoli, Maria R. BianchiI, Raffaella Riso, Giacomo M. Minicuci, Ana P. Batocchi, Serenella Servidei, Flavia Scuderi, and Emanuela Bartoccioni.

10. Antibodies in Myasthenia Gravis without Acetylcholine Receptor Antibodies: Angela Vincent, Maria Isabel Leite, Maria Elena Farrugia, Saiju Jacob, Stuart Viegas, Hiro Shiraishi, Olivier Benveniste, Paul Morgan, David Hilton-Jones, John Newsom-Davis, David Beeson, and Nick Willcox.

11. Myasthenia Gravis Experimentally Induced with Muscle-specific Kinase: Kazuhiro Shigemoto, Sachiho Kubo, Chen Jie, Naohito Hato, Yasuhito Abe, Norifumi Ueda, Naoto Kpbayashi, Kenji Kameda, Katsumi Mominoki, Atsuo Miyazawa, Akihito Ishigami, Seiji Matsuda, and Naoki Maruyama.

Part IV: Congenital Myasthenic Syndromes:.

12. Congenital Myasthenic Syndromes and the Formation of the Neuromuscular Junction: David Beeson, Richard Webster, Judith Cossins, Daniel Lashley, Hayley Spearman, Susan Maxwell, Clark Slater, John Newsom-Davis, Jacqueline Palace, and Angela Vincent.

13. Further Observations in Congenital Myasthenic Syndromes: Andrew G. Engel, Andrew G. Engel, Xin-Ming Shen, Duygu Selcen, and Steven M. Sine.

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

14. Antiganglioside Antibodies and the Presynaptic Motor Nerve Terminal: Hugh John Willison and Jaap J. Plomp.

15. Neuronal Acetylcholine Receptor Autoimmunity: Steven Vernino.

16. Lambert-Eaton Myasthenic Syndrome: Tumor versus Non-tumor Forms: Maarten Titulaer and Jan JGM Verschuuren.

Part VI: Autoimmune Pathogenesis of Myasthenia Gravis: Current Concepts:.

17. Pathogenic and Regulatory Mechanisms in Myasthenia Gravis: Rozen Le Panse, Géraldine Cizeron-Clairac, Mélinée Cuvelier,Frédérique Truffault, Jacky Bismuth, Patrice Nancy, Nicole Kerlero de Rosbo, and Sonia Berrih-Aknin.

18. Common Cellular and Diverse Genetic Basis of Thymoma-associated Myasthenia Gravis: Role of MHC class II and AIRE Genes, and Genetic Polymorphisms: Philipp Ströbel, Wen-Yu Chuang, Sergei Chuvpilo, Andreas Zettl, Tiemo Katzenberger, Hubert Kalbacher, Peter Rieckmann, Wilfred Nix, Berthold Schalke, Ralf Gold, Hans-Konrad Müller-Hermelink, Pärt Peterson, and Alexander Marx.

19. How Promiscuity Promotes Tolerance: The Case of Myasthenia Gravis: Bruno Kyewski and Richard Taubert.

20. Autoimmunizing Mechanisms in Thymoma and Thymus: Nicholas Willcox, Maria Isabel Leite, Yoshihisa Kadota, Margaret Jones, Anthony Meager, Peddasomayajula Subrahmanyam, Bhaskar Dasgupta, B Paul Morgan, and Angela Vincent.

21. Prevention of Acetylcholine Receptor Modulation in Myasthenia Gravis: Mario Losen, Pilar Martínez-Martínez, Marko Phernambucq, Janine Schuurman, Paul W.H.I Parren, and Marc De Baets.

22. Genetic Factors in Autoimmune Myasthenia Gravis: Matthieu Giraud, Claire Vandiedonck, and Henri-Jean Garchon.

23. CD4+ T Cells and Cytokines in the Pathogenesis of Acquired Myasthenia Gravis: Bianca Conti-Fine, Monica Milani, and Wei Wang.

24. Classical Complement Pathway in Experimental Autoimmune Myasthenia Gravis Pathogenesis: Premkumar Christadoss, Erdem Tüzün, Jing Li, Shamsher S. Saini, and Huan Yang.

25. Extraocular Muscle Susceptibility to Myasthenia Gravis: Unique Immunological Environment?: Jindrich Soltys, Bendi Gong, Henry J. Kaminski, Yuefang Zhou, and Linda L. Kusner.

Part VII: Clinical Trials of Myasthenia Gravis:.

26. Treatment and Clinical Research in Myasthenia Gravis: How Far Have We Come?: Richard J. Barohn.

27. Rules of Evidence for Clinical Trials—The Science of Finding the Truth?: Gary Cutter and Inmaculada Aban.

28. Myasthenia Gravis in the Elderly: Is It Different?: Johan A. Aarli.

29. Immunosuppression of EAMG by Intravenous Immunoglobulin Is Mediated by a Disease-specific Anti-immunoglobulin Fraction: Sara Fuchs, Tali Feferman, Roberto Meidler, Raanan Margalit, Camille Sicsic, Talma Brenner, Orgad Laub, and Miriam C. Souroujon.

30. Lessons from Two Trials of Mycophenolate Mofetil in Myasthenia Gravis: Donald B. Sanders and Zaeem A. Siddiqi.

31. Tacrolimus for Myasthenia Gravis: A Clinical Study of 212 Patients: Jose M. Ponseti, Josep Gamez, Jamal Azem, Manuel López-Cano, Ramón Vilallonga, and Manuel Armengola.

32. Intravenous Immunoglobulin Treatment for Myasthenia Gravis: Effectiveness, Limitations and Novel Therapeutic Strategies: Lorne Zinman.

33. Treatment of Myasthenia Gravis Acute Exacerbations with Intravenous Immunoglobulin: Philippe Gajdos and Sylvie Chevret.

Part VIII: Treatments on the Horizon for Myasthenia Gravis:.

34. Strategies for Immunoregulation: Novel Insights from Experimental Myasthenia Gravis: Matthew N. Meriggioli, JianRong Sheng, LiangCheng Li, and Bellur S. Prabhakar.

35. Antisense Treatment for Myasthenia Gravis: Experience with Monarsen: Jon D. Sussman, Zohar Argov,David McKee, Eli Hazum, Solli Brawer, and Hermona Soreq.

36. Antigen-specific Apheresis of Pathogenic Autoantibodies from Myasthenia Gravis Sera: Socrates J. Tzartos, Kalliopi Bitzopoulou, Ira Gavra, Grigoris Kordas, Leslie Jacobson, Kalliopi Kostelidou, George Lagoumintzis, Orestis Lazos, Kostantinos Poulas, Sotiris Sideris, Alexandros Sotiriadis, Nikos Trakas, and Paraskevi Zisimopoulou.

37. Etanercept Treatment in Myasthenia Gravis: Julie Rowin.

38. Rebooting the Immune System with High Dose Cyclophosphamide for Treatment of Refractory Myasthenia Gravis: Daniel B. Drachman, Robert N. Adams, Rong Hu, Richard J. Jones, and Robert A. Brodsky.

Part IX: Panel Discussion: Thymectomy for Myasthenia Gravis:.

39. Thymectomy for Nonthymomatous Myasthenia Gravis—A Critical Analysis: Joshua R. Sonett and Alfred Jaretzki III.

40. Thoracoscopic Thymectomy with the Da-Vinci Robotic System for Myasthenia Gravis: Jens C. Rückert, Mahmoud Ismail, Marc Swierzy, Holger Sobel, Patrik Rogalla, Andreas Meisel, Klaus D. Wernecke, Ralph I. Rückert, Joachim M. Müller.

41. Extended Transcervical Thymectomy in the Treatment of Myasthenia Gravis: Sanjay G. Khicha, Larry R. Kaiser, and Joseph B. Shrager.

42. Status of the Thymectomy Trial for Nonthymomatous Myasthenia Gravis Patients Receiving Prednisone: John Newsom-Davis

About the Author

Henry J. Kaminski, MD is board-certified in Neurology, the Meta Amalia Neumann professor, and the chair of the Department of Neurology at The George Washington University.

Reviews

"The book covers the physiology of the neuromuscular junction and of the ACR structure with detailed illustrations. There are extensive dissertations on the immunopathogenesis of myasthenia gravis, on the different immunogenetic properties of different thymona types, and finally on the different clinical presentations and therapeutic options. An entire chapter is devoted to when thymectomy is necessary and how it is performed. The chapter on myasthenia gravis crisis is concise and informative. This is a well organized, comprehensive book with abundant up-to-date references. It belongs in the library of any neurologist involved in the care of neuromuscular junction disorders." -Doody's Health Sciences Book Review Journal
.,."a well rounded book. I think that neurologists in training and general neurologists wanting a refresher course could do a lot worse then read this text. I would certainly recommend it." - Brain: A Journal of Neurology
"Primarily this book will appeal to neurologists, clinical neurophysiologists, and neuroanaesthetists who will find it useful for their everyday clinical practice; researchers of neuromuscular junction disorders will also find the book interesting. This book is an up-to-date and valuable source of information on myasthenia and related disorders." -The Lancet
"Generally, the chapters are well-written, illustrations are appropriate, and the twenty-first century well represented in the references. The book is useful and up-to-date summary of all aspects of MG and its related disorders." -Muscle & Nerve
"This book is strongly recommended to clinicians and researchers dealing with neuromuscular disorders (and to thoracic surgeonsperforming thymectomy)." -Journal of Neurology, Neurosurgery, and Psychiatry

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