The objective of the second edition of Myasthenia Gravis and Related Disorders is identical to the first, to provide the clinician and the scientist a common source for understanding the complex disorder, Myasthenia Gravis (MG). Although the first edition appeared in 2003, there have been surprising advances in the field that recommend a new publication. The basic sciences continue to refine acetylcholine receptor structure and the dysregulation of the immune system that modulates MG; identification of the initial triggers still appears far away. MG remains a challenging disorder to recognize and treat, with patients frequently complaining of delays in diagnosis, complications of treatment, and poor response to therapies. Muscle-specific kinase antibody-associated MG has been clinically defined and the pathogenic nature of the antibodies has gained considerable support. New mutations as causes of congenital myasthenias have been discovered. Some incremental, therapeutic advancement has occurred and these are discussed. Since the first edition, the controversy of whether thymectomy benefits in the light of modern immunosuppressive therapies has motivated the implementation of an NIH-funded trial. Robert Paul and colleagues have expanded their original discussion of the most challenging aspects of MG, its impact on patients’ psychological makeup. This subject is commonly neglected in MG texts. The new edition has been supplemented with chapters which discuss rigorous clinical assessments of patients for research trials and the epidemiology and genetics of MG.
Related to MG by clinical presentation or pathophysiology are Lambert-Eaton syndrome, congenital myasthenias, and toxic neuromuscular junction disorders. A chapter discusses neuromyotonia because of its similarity in autoimmune pathology to MG, and the occasional overlap of neuromyotonia with MG. These diseases are increasingly being defined at a molecular level. We do not use ‘‘myasthenic’’ when referring to the Lambert-Eaton syndrome. This is done in deference to Vanda Lennon and the late Edward Lambert, who prefer this terminology.
The second edition retains the ‘‘personal approach’’ of the authors regarding treatment. A challenge for MG investigation is its rarity, variability in clinical course, and heterogeneous nature, i.e., thymoma-associated, age-related differences, and clinical phenotypes (ocular vs. bulbar vs. generalized). Therefore, clinical trials are few, and robust evidence base is lacking and expert opinion often the bulwark of treatment recommendations. The individual opinion is particularly evident in the thymectomy, ocular myasthenia, and the treatment chapters.
I thank all the contributing authors, and am indebted, once again, to Humana Press for making the book a reality. Appreciation is extended to the National Eye Institute, the National Institute of Neurological Disorders and Stroke, and the Myasthenia Gravis Foundation of America that have supported my research as well as the work of many of the contributors. I also thank the Muscular Dystrophy Association for their support of neuromuscular research. To my patients who have given me more than I could ever return. Thank you.
Henry J. Kaminski, MD
