Book Review

Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach

N Engl J Med 2003; 349:1683-1684October 23, 2003

Article

Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach
Edited by H. Royden Jones, Jr., Darryl C. De Vivo, and Basil T. Darras. 1323 pp., illustrated. Philadelphia, Butterworth–Heinemann, 2003. $285. ISBN: 0-7506-7190-4

A large group of disorders affect the muscles, nerves, neuromuscular junctions, and motor neurons. These neuromuscular diseases are common in both children and adults, but because of their complex presentation their diagnosis and treatment can require not only clinical proficiency but also expertise in clinical phenomenology, electrophysiology, histopathology, molecular genetics, and protein chemistry. In children, in whom the use of extensive laboratory tests poses practical difficulties and ethical dilemmas, emphasis on a clinical approach remains fundamental, even in the present era of molecular science. For these reasons, this book on childhood neuromuscular diseases, which is aimed at clinicians and was edited by astute clinicians and scientists, is welcome. By combining their own clinical experience with that of an outstanding cast of authors, the editors have succeeded admirably in covering the clinical science of neuromuscular diseases in children while fulfilling the needs of scholars in the field.

Reviewing a book as good as this one is like critiquing a good wine; you need not only to evaluate its present strength but also to predict its longevity. Because a good book's longevity depends on improvements in future editions, it is refreshing to read in the introduction to this book that the editors humbly invite criticisms for the future. This is another sign of their sincere effort to produce a perfect book.

Most of the book's 65 chapters are very good or outstanding. Repetitions are numerous, but I found them didactic rather than distracting. The introductory chapter, a collective effort based on the editors' approach to clinical problems, offers superb reading and practical pearls emanating from the authors' clinical experience. The chapters on histology, electromyography, genetics, motor-neuron disorders, neuropathies, myasthenias, dystrophies, metabolic myopathies, channelopathies, and therapeutic principles, written by senior authors, are superb. They are succinct and contain numerous clinical pearls of wisdom. A few notable exceptions are the chapters on inflammatory myopathies, infectious myositis, and disorders of the ocular nerves and muscles, which are wordy and, though informative, not as authoritative as the others. Therapies are generally well covered, but the depth of the practical therapeutic guidelines useful to busy clinicians varies from excellent, as in the instances of chronic inflammatory demyelinating polyneuropathy and myasthenia, to weak, as in the instance of inflammatory myopathies, the most treatable group of myopathies.

It is unclear why the editors provide two chapters — although they are superb — on the Guillain–Barré syndrome (labeled the Guillain–Barré syndrome in one and acute motor axonal neuropathy in the other), two on poliomyelitis (acute poliomyelitis and vaccine-associated poliomyelitis), and three on mononeuropathies (one for the face, one for the upper extremities, and one for the lower extremities). If these chapters and some of the others were condensed, the second edition would be lighter (in weight, not in content) and more user-friendly for busy clinicians. A chapter on diagnostic testing (autoantibodies, muscle enzymes, ischemic exercise testing, and muscle imaging) would also be helpful.

The illustrations are excellent, but I found the duplication of figures, in black and white and in color, redundant and distracting, especially given that some of the color plates and the corresponding text appear on different pages. The tables are abundant, but some are so busy that they become unhelpful. The clinical vignettes are informative, didactic, and highly enjoyable. The authors deserve congratulations for including even the latest mutations (except for those involving desmin and, partially, perlecan), in spite of the rapid pace of molecular genetics.

This is an excellent book. My minor criticisms are meant only to improve future editions. Pediatric or adult neurologists, pediatricians in academic settings, and experts in neuromuscular diseases or in training for this specialty will find this book indispensable. Most important, this book comes with the assurance that what is presented is reliable, objective, and up to date.

Marinos C. Dalakas, M.D.
National Institutes of Health, Bethesda MD, 20892
dalakasm@ninds.nih.gov