Brain, Vol. 126, No. 7, 1715-1716,
July 2003
© 2003 Guarantors of Brain
doi: 10.1093/brain/awg149
Book Review |
MYASTHENIA GRAVIS AND RELATED DISORDERS
Department of Neurology, University of Newcastle upon Tyne, Newcastle upon Tyne, UK
MYASTHENIA GRAVIS AND RELATED DISORDERS
Edited by Henry J. Kaminski
2002. Totwa: The Humana Press
Price $125. pp. 396. ISBN 1-58829-058-1.
As a neurologist with an (un)healthy interest in neurological genetics, I was pleased to be asked to review the recently published book Myasthenia Gravis and Related Disorders. I hoped that it would bring me up to speed with current thinking for diseases that are not my specialist area of interest, but are important for my routine neurological practice. I was not disappointed.
Myasthenia Gravis and Related Disorders is a short, readable text which follows a standard format. Different chapters are written by different authors. Most of the well-recognised international experts have made a contribution, and although there are a few notable exceptions, their work is regularly cited throughout the text. There does appear to be an over-representation of the editor’s colleagues in Cleveland, Ohio, but overall I think it presents a balanced view of our contemporary understanding of this fascinating area.
The book begins with a series of chapters on the basic science of the neuromuscular junction (the structure and function of the neuromuscular junction and the acetylcholine receptor), followed by a detailed consideration of the immunopathogenesis of myasthenia gravis. The next section describes the clinical presentation of myasthenia gravis and its various forms, followed by chapters on the electrodiagnosis and immunological tests for disorders of the neuromuscular junction. The various management options are then discussed, including a pragmatic guide to the management of an acute myasthenic crisis with guidelines for managing patients on the intensive care unit. There is then a fascinating chapter on the role and practicalities of thymectomy. The Lambert Eaton syndrome and congenital myasthenia are considered towards the end of the book, along with a separate chapter on acquired neuromyotonia. There is a section on toxic and metabolic disorders of neuromuscular transmission, and the book ends with a chapter on the psychosocial consequences of myasthenia.
This book has a number of strong points. The first is the right balance between length and depth. The chapters are generally short and well written. There is sufficient detail to make each section an interesting and enlightening read, but not so much as to make it indigestible. This book could be easily read late at night after a busy day in the clinic. The book’s second strength is its style, which is remarkably consistent throughout. The authors were not afraid to give a firm opinion, which may or may not be qualified with a reference to published evidence. This approach might be considered unfashionable by some, and unacceptable by others—but I found it a refreshing change from many of the somewhat indigestible ‘evidence-based’ texts that are being published at the moment. Of course, the opinions expressed in this book are based upon evidence, but for a relatively rare disorder like myasthenia gravis, this evidence may be a personal case series acquired during a long and busy career, rather than 5000 patients assessed by 5000 independent observers! I do not want to create the impression that this book has been written by opinionated extremists—nothing could be further from the truth. I am sure that the editor has made sure that when an author expresses an opinion, he makes it clear that it is a personal view, and these views are usually made in the context of the available published literature. The frequency of personal communications and direct quotations is one example of this style, which, as a non-expert, I find very helpful and easy to put into clinical practice.
Specific sections that stand out are the succinct summary on the presentation and management of ocular myasthenia, and the balanced arguments for and against thymectomy in particular patient groups. The chapter on electrodiagnosis is beautifully clear, and the evidence in favour of newer treatments (intravenous immunoglobulin and mycophenolate mofetil) is presented. The chapter on thymus surgery was an eye-opener (I will never again think of thymectomy as a routine operation), and the sections on immunopathogenesis and serological investigations were both easy to understand and up to date, including the relevant recent data on antibodies to muscle-specific kinase (MuSK), which are found in a large proportion of patients with myasthenia gravis who do not have acetylycholine receptor antibodies. The final chapter on social and psychological consequences may not be everyone’s cup of tea, but it is a fitting end to a well rounded book. I think that neurologists in training and general neurologists wanting a refresher course could do a lot worse that read this text. I would certainly recommend it.
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