Clinical Neurology for Psychiatrists, 5th Ed.
Book Reviews Muscle Disorders in Childhood, 2nd Ed. By V. Dubowitz. 549 pp., illustrated. Philadelphia: W.B. Saunders, 1995, $95.00. ISBN 0-7020-1437-0. Dr. Dubowitz has drawn from his vast personal experience and appropriate past and current literature to compose Muscle Disorders in Childhood, Second Edition. The result is a meticulously written, complete treatise of neuromuscular disorders of infants and children that is beautifully illustrated. The book represents his devotion to the care of children with neuromuscular diseases. There are 15 chapters. The first sets the stage for the clinical conditions to be discussed by underscoring the approach and diagnostic tests, particularly needle muscle biopsy, used to evaluate infants and children with lower motor neuron disorders. Chapter 2 on muscular dystrophies beautifully illustrates the clinical presentations of the muscular dystrophies in children. A historical accounting of recent molecular genetic advances, including cloning of the dystrophin gene and isolation of dystrophin, are thoughtfully provided. Animal models are discussed. Treatment approaches are reviewed. The chapters on congenital and metabolic myopathies highlight the advances made in understanding the genetic bases for these conditions in a clear manner and provide excellent tables summarizing current data. Clinical photographs and muscle biopsies clearly elucidate the cardinal findings of each condition. The metabolic myopathies are divided into glycolytic, lipid, channel, and mitochondrial disorders. A short chapter on endocrine myopathies includes conditions secondary to thyroid disorders, disorders of the parathyroids and osteomalacia, renal rickets, disorders of the pituitary and adrenals, and nutritional myopathies. The copiously illustrated chapter on spinal muscular atrophies provides much useful clinical detail. The spinal muscular atrophies are divided into the three commonly recognized types based on initial presentation and rate of progression. Discussion about the mapping of all three types to chromosome region 5q11.2-13.3 is current. Since preparation of this chapter, two candidate genes, SMN and NAIP, in the chromosomal region have been published in Cell, indicating the rapid advances in molecular genetics of the neuromuscular diseases. Myasthenia gravis is presented in a separate chapter. To help organize treatment of myasthenia gravis, a flow chart adapted from Dr. NewsonDavis is utilized. The congenital myasthenic syndromes are summarized in two helpful tables. The hypotonic infant is discussed in terms of neuromuscular and nonneuromuscular syndromes. Connective tissue disorders and the PraderWilli syndrome are well presented. Sequential photographs of patients with Prader-Willi syndrome illustrate the depth of the author's study. The chapter on inflammatory myopathies focuses on the clinical aspects and treatment of dermatomyositis. The author's approach to treatment is outlined. A chapter on contractures clearly denotes the clinical focus of the book. This clinical problem is discussed by reviewing the multiple causes of contractures in infants from arthrogyryposis to fibrodysplasia ossificans progressive to club foot. These conditions do not fit classical muscle and nerve disorder classifications, but they are very important for clinicians who care for children with neuromuscular conditions. The book concludes with a review of the current knowledge of molecular genetics of all known neurnmuscular diseases presented in a useful, table format. Dr. Dubowitz is to be congratulated on this complete compendium of neuromuscular disorders of infants and children. His first-person-singular writing personalizes the book and makes for delightful and informative reading. The references are appropriate and current. Muscle Disorders in Childhood, Second Edition will be well appreciated by clinicians who diagnose and treat infants and children with neuromuscular disorders.
Stephen A. Smith, M.D. University of Minnesota Medical School
© 1996 by Elsevier Science Inc. All rights reserved. PII S0887-8994(96)00017-3 0887-8994/96/$15.00
By David M. Kaufman. 669 pp., illustrated. Philadelphia: W.B. Saunders, 1995. $79.00. ISBN 0-7216-5829-6. Pediatric and medicine/pediatric residents frequently have difficulty in performing a skilled neurologic examination on adolescents and young adults. To improve their performance, they often resort to textbooks which promise to impart the ability to perform a complete neurologic examination in a few minutes. Resorting to such textbooks has the predicted outcome--neither the resident nor the patient benefits from such a limited approach. Unfortunately, the resident tends to leave his/her training with limited skills in the neurologic examination as well as a poor foundation for improvement. This text fills a real didactic notch for these residents. In a straight-forward fashion, without overwhelming detail and utilizing simple diagrams, the neurologic examination including cortex, midbrain, cerebellum, and brainstem, is described verbally and schematically (with simple drawings of the nervous system). The book is relatively easy to read, contains a wealth of information in regard to the examination, and would be extremely useful for psychiatrists and pediatric and medicine/pediatric residents. The book is divided into two sections, one covering classical and anatomic neurology and the other major neurologic symptoms, such as seizures, headaches, and cerebral vascular disease. Following each chapter, there is a question and answer section with varying degrees of difficulty. In the introduction, the author states a belief that the question and answer section is an intricate part of the learning process, allowing each student to gauge the depth of his/her understanding. The chapter references are relatively modern with many 1993 and 1994 notations as well as earlier seminal contributions from the literature. The one flaw is that psychiatric disorders, such as psychogenic seizures, receive relatively limited discussion, presumably because of the author's experience. 1 would highly recommend this introduction to neurology to any firstor second-year resident in virtually any specialty where the neurologic examination is important. I like the book, and even the most experienced neurologist will get an enjoyable review and may even find an occasional pearl. I did not know that dystonia patients can frequently retrowalk without dystonia.
Oklahoma City, OK
Sanford Schneider, M.D. Presbyterian Health Foundation Chief, Child Neurology University of Oklahoma School of Medicine
Metabolic Myopathies By David Hilton-Jones, Marian Squier, Doris Taylor, and Paul Matthews. 281 pp., illustrated. London: W.B. Saunders, 1995. $70.50. ISBN 0-07020-1607-1. This compact volume represents number 29 in the series Major Problems in Neurology edited by professors Warlow and van Gijn and results from the combined efforts of a team of four specialists from the areas of clinical neurology, neuroscience, neuropathology, and neurogenetics. It is a comprehensive guide to the diagnosis and management of metabolic myopathies, intended for a wide audience of neurologists, pediatricians, clinical biochemists, and pathologists. The chemistry works well and the fruit of their labor is an eminently readable and informative guidebook. The volume is divided into 13 chapters, which encompass clinical evaluation of the patient suspected of having a neuromuscular disorder, a primer on exercise physiology (which even this reviewer can understand), and chapters on disorders of carbohydrate metabolism, mitochondria, endocrine glands, toxic/nutritional factors, malignant hyperthermia, and genetics, as these apply to metabolic myopathies. The guide consists of only 281 pages, so that each chapter is allocated an average of only 22 pages (including 2-6 pages of references). It is precisely this conciseness which makes this book so useful; one can always refer to Engel or to Dubowitz for more comprehensive coverage. The information is pre-