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Clinical pediatric neurology: a signs and symptoms approach
Book Reviews
problems of the newborn, serving both as a resource and as a stimulus for future research endeavors.
Neurology of the Newborn, 4th Edition By Joseph J. Volpe, 912 pp., illustrated. Philadelphia: WB Saunders Company, 2001, $105.00 ISBN 0-7216-8448-3. The fourth edition of this classic remains an extraordinary work of scholarship. The book has been completed, updated, and includes many new tables, figures, and more than 2000 new references. The organization of the 24 chapters is identical to that in the previous issues. However, tables and figures have been placed with the relevant text on the same page. Moreover, all of the tables have been reformatted, which makes for easy reading and comprehension. The strength of this book is the strong clinical emphasis that is incorporated from lessons learned from basic and clinical research. The book captures many of the recent advances that have occurred over the past 5 to 7 years since the last edition. Some of the additions are obvious whereas others are subtle. For example, in Chapter 4, it is pointed out that retesting the infant after failure of a hearing screening is best performed at approximately 2 to 6 weeks (previous recommendations were 2 to 4 months). A more obvious example that I found to be noteworthy is an expanded discussion on near-infrared spectroscopy to reflect the progress made with this technique. Description of little-used techniques, such as transillumination and the Ladd intracranial pressure monitor, are omitted. The important role of glucose in modulating perinatal brain injury is succinctly summarized in a new table. In the chapter on the biochemical and physiologic aspects of hypoxic-ischemic encephalopathy the importance of the reperfusion period and mode of cell death (necrosisapoptosis), the critical role of free radicals including nitric oxide, and the emerging importance of inflammation-cytokines-in the genesis of brain injury are highly relevant additions. Potential interventions for preventing or ameliorating perinatal hypoxic-ischemic brain injury is incorporated in a new table. The pathogenesis of periventricular leukomalacia has been expanded to include the intrinsic vulnerability of the oligodendrocyte, as well as the role of intrauterine infection and glutamate toxicity. In this regard, the biochemical mechanisms of oligodendroglial death with hypoxia-ischemia is an outstanding new section. The discussion of the relation of posthemorrhagic ventricular dilatation to brain injury includes new experimental data supporting the potential role of elevated free radicals in the genesis of white matter injury. In this regard, a potential role for glutathione depletion and oxidative stress in the genesis of hypoglycemic induced brain injury is presented. The description of potential new clinical interventions include the role of excitatory amino-acid antagonists in the treatment of nonketotic hyperglycemia, a new treatment for Zellweger syndrome (i.e., sodium4-phenylbutyrate), and the use of nasal CPAP for congenital muscular dystrophy. Newly described clinical conditions include rare examples of neonatal-onset fatty acid disorders, carbohydrate-deficient glycoprotein syndromes, and congenital isolated pharyngeal dysfunction. Recognition that there may be an additional type O lethal form of spiral muscular atrophy and differentiating congenital muscular dystrophy into the “classic,” “pure” merosin-positive and merosin-negative (which includes white matter involvement) subtypes are important subtle additions to the text. The management of the pregnant mother with HIV infection has been updated to reflect the rapid progress made in this field. There are numerous new MRI figures that illustrate the neuropathology of diseases, such as bilirubin encephalopathy, nonketotic hypoglycemia, biotinidase deficiency, and the evolution of Menkes disease, to name but a few. Indeed, there are many more important additions in every chapter that I will leave to future readers to discover. The book is so comprehensive, it is beyond criticism. However, I anticipate the next edition will include a section on the genesis of cognitive and neurobehavioral problems now noted at long-term follow-up in up to 50% of very-low-birth-weight survivors, irrespective of the presence or absence of neuroradiologic abnormalities. This book remains a treasure for those interested in neurologic
258
PEDIATRIC NEUROLOGY
Vol. 25 No. 3
Dallas, TX
Jeffrey M. Perlman, M.D. Department of Pediatrics University of Texas SW Medical Center
Clinical Pediatric Neurology: A Signs and Symptoms Approach By Gerald M. Fenichel, M.D. 405 pp., illustrated. Philadelphia: WB Saunders Company, 2001. $80.00 ISBN: 0-7216-9234-6. If you intend to write an accessible review covering a specialized area of medicine, this book should be the paradigm. It is the sort of book that is widely sought but seldom found. Fenichel’s survey of child neurology is concise, accurate, provides extensive coverage of the field, and is salted with “pearls of wisdom.” Wouldn’t this be an infinitely better choice for students and residents than most of the paperbound review books and clinical handbooks that are always in their jacket pockets? Perhaps the publisher, WB Saunders, needs to convert this text to the Palm OS so the information can be downloaded into the ever-present Palm Pilots. Senior physicians will also find much to like in this textbook, which offers mini-refreshers on uncommon neurologic disorders combined with snippets of advice from one of the country’s most experienced clinicians. Although it is subtitled A Signs and Symptoms Approach, this subtitle refers to the organization of the chapters and does not imply a concentration on signs and symptoms to the exclusion of diagnostic tests and therapeutic measures. The chapters begin with “Paroxysmal Disorders” and “Altered States of Consciousness” and continue through “Headache” and “Psychomotor Retardation and Regression.” There are eight chapters titled with various types of motor problems, chapters titled “Disorders of Ocular Motility,” “Disorders of the Visual System,” “Disorders of Cranial Volume and Shape,” and a chapter that departs from the signs and symptoms theme and is called “Lower Brainstem and Cranial Nerve Dysfunction.” It is not always clear which chapter will contain a given subject, although assignment is said by the author to be based “on the usual initial clinical feature.” Don’t worry about it. The index is excellent and always sent me to the right page. There is little duplication because the author limits each disorder to a single location and then ruthlessly refuses to discuss the topic anywhere else. Some of the chapter titles seem awkward and subject choices seem a bit forced (mitochondrial diseases in a chapter titled “Cramps, Muscle Stiffness and Exercise Intolerance,” for example), but who pays much attention to chapter titles, anyhow? Each topic has an introductory section that defines the topic and may give a chromosomal location or laboratory signature, a section describing the clinical features, another one on diagnosis, and a final portion describing therapy and management. The references are skimpy but up-to-date. They generally refer to clinical series or recent genetic studies. As expected for this type of book, there are frequent tables listing diseases in various categories. Included in these are the causes of encephalopathies lists, the brain tumor list, causes of hypotonia list, causes of facial weakness list, and so on—you get the idea. In addition, there are first-class pictures depicting clinical findings, showing biopsies, and diagramming pathophysiologic mechanisms. The illustrations are excellent and add to the reader’s understanding of the text. A unique feature of this book is Fenichel’s personal guidance, much of which is in separate sections at the beginning of chapters, labeled “Approach to the Patient.” For example, in “Movement Disorders” it states: “Movement disorders cannot be adequately described; they must be seen. If abnormal movements are not present at the time of examination, the parents should be instructed to videotape the movements at home.” In the chapter “Psychomotor Retardation and Regression,” “Telling Parents” advises: “It is not possible to make bad news sound good or even half bad. The goal of telling parents that their child will be mentally
© 2001 by Elsevier Science Inc. All rights reserved. 0887-8994/01/$—see front matter
retarded or otherwise neurologically impaired is that they hear and understand what you are saying.” Good advice like this is cheap at any price. It is this type of good judgment that permeates Clinical Pediatric Neurology and sets it apart from many of the small books crammed with anonymous lists that the house staff carry in their pockets. Add to this a fluid, easily understood English, good binding, glossy paper, and easy-to-read typefaces and this volume is a bargain. The book is not perfect. Some of the sections are uninspired. The discussion of antiepileptic drugs repeats FDA indications (which only have a tangential relationship to clinical practice) and does not offer much insight into the current utility of the newer agents. Collecting all the disorders primarily seen in newborns and infants in one chapter would have made the book more useful as would a more complete list of references. Nevertheless, although not perfect, this book is awfully good.
Lexington, KY
Robert J. Baumann, M.D. Professor of Neurology and Pediatrics University of Kentucky
Operative Techniques in Pediatric Neurosurgery Edited by A. Leland Albright, Ian F. Pollack, and P. David Adelson. 288 pp., illustrated. New York: Thieme, 2001. $179.00 ISBN 0-86577-846-9. Operative Techniques in Pediatric Neurosurgery, by Albright et al., is an adjunct to their more inclusive textbook, Principles and Practices of Pediatric Neurosurgery (1999). This book is presumed to be a quick reference for neurosurgeons, as well as an introductory text for nonsurgeons. According to the preface, the contributing authors are chosen for their experience and expertise in various operative techniques, recognizing that other approaches may be taken to achieve the same end. Emphasis is placed on “ ‘little’ techniques [that] may contribute importantly to the success of an operation.” The material is presented in a logical fashion, with major sections for
congenital/developmental disorders, trauma, neoplasms, vascular disease, and functional disorders. Under these broad headings, specific surgeries are addressed, including CSF shunting, cranial expansion, myelomeningocele and brachial plexus repairs, tumor and AVM resection, and epilepsy surgery. Chapter lengths are such that most are readily consumed within minutes but supply sufficient detail for the majority of readers. A plethora of intraoperative photographs and diagrammatic cartoons illustrate the narrative, although the quality varies among chapters. Helpful “pearls” and an editor’s commentary are found at the end of each chapter for emphasis or clarification of specific points. The section most helpful to the neurologist is that dealing with functional disorders. In it, specifics of preoperative evaluation and operative goals are discussed. Furthermore, the text delineates the surgeon’s role in a multidisciplinary approach to operable neurologic disorders. Although most authors suggest practical operative techniques to avoid complications, several do not address postoperative management and complications or do so in a cursory manner. Another concern is, that although more recent techniques, such as neuroendoscopy and intrathecal baclofen pump placement, are discussed in some detail, others are not mentioned (electronic valves in ventriculoperitoneal shunts or vagus nerve stimulation). Operative Techniques in Pediatric Neurosurgery is a straightforward book that serves as a quick reference to the neurosurgeon. More importantly, it fills a niche by introducing the neurologist to pediatric neurosurgery. Although the pediatric neurologist and neurosurgeon often share patients, most neurologists have a cursory knowledge of surgical interventions. This work provides a cogent discussion of these, so that the nonsurgeon may more immediately understand and appropriately utilize the surgeon’s services.
Loma Linda, CA
Chalmer McClure Division of Pediatric Neurology Loma Linda University Children’s Hospital
Book Reviews 259
problems of the newborn, serving both as a resource and as a stimulus for future research endeavors.
Neurology of the Newborn, 4th Edition By Joseph J. Volpe, 912 pp., illustrated. Philadelphia: WB Saunders Company, 2001, $105.00 ISBN 0-7216-8448-3. The fourth edition of this classic remains an extraordinary work of scholarship. The book has been completed, updated, and includes many new tables, figures, and more than 2000 new references. The organization of the 24 chapters is identical to that in the previous issues. However, tables and figures have been placed with the relevant text on the same page. Moreover, all of the tables have been reformatted, which makes for easy reading and comprehension. The strength of this book is the strong clinical emphasis that is incorporated from lessons learned from basic and clinical research. The book captures many of the recent advances that have occurred over the past 5 to 7 years since the last edition. Some of the additions are obvious whereas others are subtle. For example, in Chapter 4, it is pointed out that retesting the infant after failure of a hearing screening is best performed at approximately 2 to 6 weeks (previous recommendations were 2 to 4 months). A more obvious example that I found to be noteworthy is an expanded discussion on near-infrared spectroscopy to reflect the progress made with this technique. Description of little-used techniques, such as transillumination and the Ladd intracranial pressure monitor, are omitted. The important role of glucose in modulating perinatal brain injury is succinctly summarized in a new table. In the chapter on the biochemical and physiologic aspects of hypoxic-ischemic encephalopathy the importance of the reperfusion period and mode of cell death (necrosisapoptosis), the critical role of free radicals including nitric oxide, and the emerging importance of inflammation-cytokines-in the genesis of brain injury are highly relevant additions. Potential interventions for preventing or ameliorating perinatal hypoxic-ischemic brain injury is incorporated in a new table. The pathogenesis of periventricular leukomalacia has been expanded to include the intrinsic vulnerability of the oligodendrocyte, as well as the role of intrauterine infection and glutamate toxicity. In this regard, the biochemical mechanisms of oligodendroglial death with hypoxia-ischemia is an outstanding new section. The discussion of the relation of posthemorrhagic ventricular dilatation to brain injury includes new experimental data supporting the potential role of elevated free radicals in the genesis of white matter injury. In this regard, a potential role for glutathione depletion and oxidative stress in the genesis of hypoglycemic induced brain injury is presented. The description of potential new clinical interventions include the role of excitatory amino-acid antagonists in the treatment of nonketotic hyperglycemia, a new treatment for Zellweger syndrome (i.e., sodium4-phenylbutyrate), and the use of nasal CPAP for congenital muscular dystrophy. Newly described clinical conditions include rare examples of neonatal-onset fatty acid disorders, carbohydrate-deficient glycoprotein syndromes, and congenital isolated pharyngeal dysfunction. Recognition that there may be an additional type O lethal form of spiral muscular atrophy and differentiating congenital muscular dystrophy into the “classic,” “pure” merosin-positive and merosin-negative (which includes white matter involvement) subtypes are important subtle additions to the text. The management of the pregnant mother with HIV infection has been updated to reflect the rapid progress made in this field. There are numerous new MRI figures that illustrate the neuropathology of diseases, such as bilirubin encephalopathy, nonketotic hypoglycemia, biotinidase deficiency, and the evolution of Menkes disease, to name but a few. Indeed, there are many more important additions in every chapter that I will leave to future readers to discover. The book is so comprehensive, it is beyond criticism. However, I anticipate the next edition will include a section on the genesis of cognitive and neurobehavioral problems now noted at long-term follow-up in up to 50% of very-low-birth-weight survivors, irrespective of the presence or absence of neuroradiologic abnormalities. This book remains a treasure for those interested in neurologic
258
PEDIATRIC NEUROLOGY
Vol. 25 No. 3
Dallas, TX
Jeffrey M. Perlman, M.D. Department of Pediatrics University of Texas SW Medical Center
Clinical Pediatric Neurology: A Signs and Symptoms Approach By Gerald M. Fenichel, M.D. 405 pp., illustrated. Philadelphia: WB Saunders Company, 2001. $80.00 ISBN: 0-7216-9234-6. If you intend to write an accessible review covering a specialized area of medicine, this book should be the paradigm. It is the sort of book that is widely sought but seldom found. Fenichel’s survey of child neurology is concise, accurate, provides extensive coverage of the field, and is salted with “pearls of wisdom.” Wouldn’t this be an infinitely better choice for students and residents than most of the paperbound review books and clinical handbooks that are always in their jacket pockets? Perhaps the publisher, WB Saunders, needs to convert this text to the Palm OS so the information can be downloaded into the ever-present Palm Pilots. Senior physicians will also find much to like in this textbook, which offers mini-refreshers on uncommon neurologic disorders combined with snippets of advice from one of the country’s most experienced clinicians. Although it is subtitled A Signs and Symptoms Approach, this subtitle refers to the organization of the chapters and does not imply a concentration on signs and symptoms to the exclusion of diagnostic tests and therapeutic measures. The chapters begin with “Paroxysmal Disorders” and “Altered States of Consciousness” and continue through “Headache” and “Psychomotor Retardation and Regression.” There are eight chapters titled with various types of motor problems, chapters titled “Disorders of Ocular Motility,” “Disorders of the Visual System,” “Disorders of Cranial Volume and Shape,” and a chapter that departs from the signs and symptoms theme and is called “Lower Brainstem and Cranial Nerve Dysfunction.” It is not always clear which chapter will contain a given subject, although assignment is said by the author to be based “on the usual initial clinical feature.” Don’t worry about it. The index is excellent and always sent me to the right page. There is little duplication because the author limits each disorder to a single location and then ruthlessly refuses to discuss the topic anywhere else. Some of the chapter titles seem awkward and subject choices seem a bit forced (mitochondrial diseases in a chapter titled “Cramps, Muscle Stiffness and Exercise Intolerance,” for example), but who pays much attention to chapter titles, anyhow? Each topic has an introductory section that defines the topic and may give a chromosomal location or laboratory signature, a section describing the clinical features, another one on diagnosis, and a final portion describing therapy and management. The references are skimpy but up-to-date. They generally refer to clinical series or recent genetic studies. As expected for this type of book, there are frequent tables listing diseases in various categories. Included in these are the causes of encephalopathies lists, the brain tumor list, causes of hypotonia list, causes of facial weakness list, and so on—you get the idea. In addition, there are first-class pictures depicting clinical findings, showing biopsies, and diagramming pathophysiologic mechanisms. The illustrations are excellent and add to the reader’s understanding of the text. A unique feature of this book is Fenichel’s personal guidance, much of which is in separate sections at the beginning of chapters, labeled “Approach to the Patient.” For example, in “Movement Disorders” it states: “Movement disorders cannot be adequately described; they must be seen. If abnormal movements are not present at the time of examination, the parents should be instructed to videotape the movements at home.” In the chapter “Psychomotor Retardation and Regression,” “Telling Parents” advises: “It is not possible to make bad news sound good or even half bad. The goal of telling parents that their child will be mentally
© 2001 by Elsevier Science Inc. All rights reserved. 0887-8994/01/$—see front matter
retarded or otherwise neurologically impaired is that they hear and understand what you are saying.” Good advice like this is cheap at any price. It is this type of good judgment that permeates Clinical Pediatric Neurology and sets it apart from many of the small books crammed with anonymous lists that the house staff carry in their pockets. Add to this a fluid, easily understood English, good binding, glossy paper, and easy-to-read typefaces and this volume is a bargain. The book is not perfect. Some of the sections are uninspired. The discussion of antiepileptic drugs repeats FDA indications (which only have a tangential relationship to clinical practice) and does not offer much insight into the current utility of the newer agents. Collecting all the disorders primarily seen in newborns and infants in one chapter would have made the book more useful as would a more complete list of references. Nevertheless, although not perfect, this book is awfully good.
Lexington, KY
Robert J. Baumann, M.D. Professor of Neurology and Pediatrics University of Kentucky
Operative Techniques in Pediatric Neurosurgery Edited by A. Leland Albright, Ian F. Pollack, and P. David Adelson. 288 pp., illustrated. New York: Thieme, 2001. $179.00 ISBN 0-86577-846-9. Operative Techniques in Pediatric Neurosurgery, by Albright et al., is an adjunct to their more inclusive textbook, Principles and Practices of Pediatric Neurosurgery (1999). This book is presumed to be a quick reference for neurosurgeons, as well as an introductory text for nonsurgeons. According to the preface, the contributing authors are chosen for their experience and expertise in various operative techniques, recognizing that other approaches may be taken to achieve the same end. Emphasis is placed on “ ‘little’ techniques [that] may contribute importantly to the success of an operation.” The material is presented in a logical fashion, with major sections for
congenital/developmental disorders, trauma, neoplasms, vascular disease, and functional disorders. Under these broad headings, specific surgeries are addressed, including CSF shunting, cranial expansion, myelomeningocele and brachial plexus repairs, tumor and AVM resection, and epilepsy surgery. Chapter lengths are such that most are readily consumed within minutes but supply sufficient detail for the majority of readers. A plethora of intraoperative photographs and diagrammatic cartoons illustrate the narrative, although the quality varies among chapters. Helpful “pearls” and an editor’s commentary are found at the end of each chapter for emphasis or clarification of specific points. The section most helpful to the neurologist is that dealing with functional disorders. In it, specifics of preoperative evaluation and operative goals are discussed. Furthermore, the text delineates the surgeon’s role in a multidisciplinary approach to operable neurologic disorders. Although most authors suggest practical operative techniques to avoid complications, several do not address postoperative management and complications or do so in a cursory manner. Another concern is, that although more recent techniques, such as neuroendoscopy and intrathecal baclofen pump placement, are discussed in some detail, others are not mentioned (electronic valves in ventriculoperitoneal shunts or vagus nerve stimulation). Operative Techniques in Pediatric Neurosurgery is a straightforward book that serves as a quick reference to the neurosurgeon. More importantly, it fills a niche by introducing the neurologist to pediatric neurosurgery. Although the pediatric neurologist and neurosurgeon often share patients, most neurologists have a cursory knowledge of surgical interventions. This work provides a cogent discussion of these, so that the nonsurgeon may more immediately understand and appropriately utilize the surgeon’s services.
Loma Linda, CA
Chalmer McClure Division of Pediatric Neurology Loma Linda University Children’s Hospital
Book Reviews 259