Book Reviews Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach Editors: H. Royden Jones, Jr, Darryl C. DeVivo, Basil T. Darras. 1323 pp, illustrated. Butterworth Heinemann, 2002. $285 ISBN: 0-7506-7190-4. Everything you need to know about neuromuscular disease is to be found in the 1323 pages of this large, well produced textbook. An international group of experts has produced an integrated and comprehensive review of current knowledge. A unique feature for a 21st century text, and one of which the editors are justifiably proud, is the large number of clinical vignettes included in almost all of the clinical chapters. Not since the era of Frank Ford’s text (Diseases of the Nervous System in Infancy, Childhood and Adolescence, 1937-1966) or the early editions of Standbury, Wyngaarden, and Fredrickson (The Metabolic Basis of Inherited Disease, 1960-1972) have authors and publishers of a major text been willing to devote so many column inches to case descriptions. Long after many of the pages containing detailed classifications, pathophysiologic theories, and proposed genetic mechanisms have been made obsolete by newer knowledge, this textbook, like its predecessors above, will be on clinicians shelves and in active use because of the clinical insights provided by the vignettes. Though brief, the vignettes help to convey a picture of the clinical disorder that tables of symptoms and lists of signs cannot match. They are often accompanied by a helpful discussion of the differential diagnosis or of the most efficient sequencing of diagnostic tests. Three useful introductory chapters cover Pediatric Electromyography, Muscle Biopsy and Nerve Biopsy and include tables with such useful information as nerve conduction reference values by age and the nerve biopsy findings for various disorders. The next five sections are organized by clinical area beginning with Infantile Hypotonia and Arthrogryposis followed by sections on Anterior Horn Cell Disorders (interestingly this includes chapters on Tetanus and Rabies), Peripheral Nerve Disorders, Neuromuscular Junction Disorders and Myopathies. There is then a catch-all section covering miscellaneous problems such as the critical illness neuromuscular disorders, malignant hyperthermia, cramps and rhabdomyolysis. Included in this section are chapters on Friedreich’s Ataxia, other spinocerebellar syndromes, and complex regional pain syndromes. A final section is devoted to therapy, including orthopedic interventions, intensive care, and rehabilitation. As a bonus this section includes a review of potential molecular therapies by George Karpati and a nice discussion of ethical issues by Russell Snyder. This book is well bound, has an easy to read type-face and is printed on high quality, shiny paper. I found the index to be complete. The chapters are extensively referenced as is to be expected in this type of text and I was pleased to see a large number of current references (but none more recent than 2001). There are abundant (and appropriate) tables, lists and illustrations including 13 pages with color plates. There are a number of particularly useful figures such as the one showing the risk of transmission of a mutation causing Duchenne’s muscular dystrophy for various family histories. Clinicians will also be pleased with the numerous pictures illustrating the typical appearance of children with different neuromuscular disorders. This book is certain to become the standard reference for pediatric neuromuscular disorders. It will enhance the collection of any medical or neurological library and child neurology groups will want to add it to their office or departmental book shelf. Students and residents should be encouraged to read the clinical vignettes even if they do not want to purchase their own copies.
Robert J. Baumann, MD Department of Neurology University of Kentucky
Disorders of Development and Learning, 3rd Edition Editor: Mark L. Wolraich. Hamilton, Ontario, Canada. BC Decker, 2003, 374 pp, illustrated, $79.95. ISBN: 1-55009-224-3. Why do pediatricians persist in lumping development, cognition, and behavior into the same category for subspecialty attention? Each of these subjects, if not wholly unrelated to the other two, can at least be studied in its own right. Why is it further stipulated that these topics must necessarily be approached from the neurological perspective? “Behavior Belongs in the
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Brain”, intones the title of a popular recent compendium in developmental/ behavioral pediatrics. Other parts of the body certainly develop; where is it written that they do not also behave or learn? Are child neurologists, as pediatric subspecialists, victims of our own hype? The point of this polemic is that disorders of development, behavior, and cognition can perfectly well be studied, diagnosed, treated and managed by pediatricians with special interest and training in these areas. Thus the rationale for this book, which is actually a collection of essays on selected topics in development, behavior, and cognition in children. The compilation is far from exhaustive, and the apparent randomness of the selection is a bit off-putting at first glance (why include Prader-Willi but not Angelman’s, myelodysplasia but not micrencephaly, etc.?) The reader soon realizes, however, that a sincere attempt has been made to focus on those disorders most commonly encountered in clinical practice. While nearly half the authors are nonphysicians, the book has been edited and organized by a well-known developmental pediatrician. It is thus surprising that the clinical approach is to such a large degree nonmedical. The book opens with five chapters devoted chiefly to assessments that the physician would most likely classify as laboratory measures, to be chosen only once a clinical diagnosis has been made. Such tests are frequently essential to developmental diagnosis, but so is the EEG to convulsive disorders; this does not mean that the diagnosis of epilepsy can be left to the electroencephalographer. Subsequent chapters, dealing with individual syndromes, are scholarly and informative, but few place any significant emphasis on the natural history of the disorder in question. The approach is foreign to those of us who explain our work to patients thus: “The reason your auto mechanic can fix your car is not that he is some sort of mechanical genius, but that he knows what happened to the last 100 cars that made that noise”. The book contains a wealth of information for reference. Especially useful are the numerous tables detailing milestones of normal development in various areas, as well as functional descriptions of the most common intelligence quotient tests. The discussion of clinical features of the various syndromes of the autism spectrum is particularly thorough, with tables of differential diagnosis and pharmacologic treatment that can be consulted with a case at hand. The chapter on speech and language disorders, written from the perspective of the speech pathologist, touches on nearly every important syndrome. One might wish it were organized to make a clearer distinction between the structural and neurological apparatus of speech on the one hand, and the neuropsychological apparatus of language on the other. For example, fluency disorders, an appropriate bridge between the two, should not be separated from language disorders by discussions of resonance and cleft palate, which are purely speech issues. The section on developmental language disorders omits reference to phonologic programming deficit and verbal auditory agnosia, but does include a detailed discussion of pragmatics and discourse. The chapter on learning disabilities provides a quick overview; more complete discussions of dyslexia, dysgraphia, and dyscalculia abound in the neurological and psychiatric literature. The book’s treatment of the concept of intellect is a bit traditional for this day and age. The chapter on developmental measures points out correctly that Binet and Simon designed the first intelligence quotient test as a predictor of school success, but doesn’t go on to explain that while such tests still do this job superbly, they are not good for much of anything else. In the chapter on disorders of mental development, the importance of adaptive behavior to the definition of mental retardation is treated almost dismissively. On the other hand, the author’s suggestion that the distinction between “educable” and “trainable” is no longer useful is open to question, since this distinction defines the mental age at which functional literacy may be reasonably expected. In the chapter on cerebral palsy, it is stated that “the physician should assume normal cognitive function”; however, overestimation of cognitive abilities can be just as harmful to the patient as underestimation. The physician’s job is to know, as precisely as possible, what is the extent of the patient’s intellectual capacities.
These few cavils (probably largely turf issues) aside, the book makes a substantive contribution to the developmental disabilities literature. It should provide a useful resource to the child neurologist who does not have a primary interest in these disorders. Boston, MA
Peter B. Rosenberger, MD Massachusetts General Hospital
© 2003 by Elsevier Inc. All rights reserved. 0887-8994/03/$—see front matter