Pichet Termsarasab - Stiff-Person Syndrome and Related Disorders: A Comprehensive, Practical Guide

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Stiff-person syndrome (SPS), originally called stiff-man syndrome, is actually a group of disorders in which knowledge and understanding have been evolving rapidly in the past few decades. As one can appreciate from the name transition, this group of disorders affects men and women, thus the name change from stiff-man syndrome to stiff-person syndrome. With the rapid evolution in the neuroimmunology field, various different antibodies, in addition to anti-glutamic acid decarboxylase (anti-GAD), with their associated phenotypes have been increasingly recognized. Hence, the name stiff-person spectrum disorders (SPSD) has emerged and is the most inclusive, because it includes various phenotypes in this group of disorders. Throughout this book, we use the term SPSD to clearly represent the entire group of SPS, not only classic SPS but also its variants. The relationship between antibodies and SPSD phenotypes is complex. Each individual antibody, such as the anti-GAD antibodies, may be associated with several SPSD phenotypes and other neurological phenotypes.

Stiff-Person Syndrome and Related Disordersis the first book dedicated to this group of disorders. It serves as a single go-to resource that collects important knowledge about SPSD, from original descriptions to state-of-the-art information. Despite extensive knowledge accumulating in SPSD, the existing publications are mainly original papers, review articles, or book chapters. The authors clinical experiences are supplemented in this book, since knowledge in many areas in SPSD remains limited and expert opinions remain a useful resource. The book starts with a historical review of the evolution of SPSD. In the subsequent chapters, clinical overview including clinical phenomenology of SPSD, differential diagnoses, and clinically relevant basic science aspects, including neurochemistry and neuroimmunology, are discussed. Then, clinical phenotypes of SPSD and other associated phenotypes are detailed based on the antibody type, including anti-GAD and other antibodies. The progressive encephalomyelitis with rigidity and myoclonus syndrome is unique and highlighted in a dedicated chapter. With the complexity between the clinical phenotypes and immunophenotypes (types of antibodies), a summary of the practical approach is then discussed. This chapter is followed by chapters on treatment, as well as emergencies in SPSD in which significant morbidity or mortality may ensue if not identified and treated promptly. SPSD in the pediatric population and a closely related but different group of disorders, namely, peripheral nerve hyperexcitability syndromes and startle syndromes, are also detailed in individual chapters in this book.

Stiff-Person Syndrome and Related Disorderswould be of interest to neurologists as well as multiple neurologic subspecialists, including those specializing in movement disorders, neuromuscular disorders, epilepsy, and neuroimmunology. Trainees in neurology and any of the above fields will find this book enriching. In addition to neurologists, internists, rheumatologists, psychiatrists, and physiatrists, physical and occupational therapists may find this book useful as they may encounter patients with SPSD. This book will also serve as a resource for basic science researchers, including electrophysiologists, neurochemists, immunologists, or pathologists. Many challenges and gaps in the field of SPSD are identified from the currently available data, and we hope that this book will stimulate further research and studies to fill the gap in this field.

We would like to thank all of our patients. We would like to express thanks to all our mentors and colleagues. Their teaching and support helped all of us to be neurologists, clinicians, and writers. These include Kanokwan Boonyapisit, Catherine Cho, Robert B. Daroff, Steven J. Frucht, Paul E. Greene, John Leigh, Chanin Limwongse, David C. Preston, Teeratorn Pulkes, Tumtip Sangruchi, Barbara Shapiro, Kristina Simonyan, David Swope, Oscar M. Reinmuth, Winona Tse, Supoch Tunlayadechanont, Ruth H. Walker, and Asa J. Wilbourn.

We also would like to thank Richard Lansing, Editorial Director, Clinical Medicine, Springer, and Diane Lamsback, Developmental Editor, for their support and help in getting this book successfully published.