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The molecular and genetic basis of neurological disease
144
Book Reviews/Electroencephalography
and clinical Neurophysiology
In conclusion, this software and the accompanying book offer a good and enjoyable way of exploring the properties of different ionic currents, and the way they interact and determine the electrical response of neurons. Yitzhak Schiller
Max-Planck-Institut fiir Medizinische Forschung, Heidelberg
(Germany)
Neurobiology, 3rd JCdn.- G.M. Shepherd (Oxford University Press, New York, 1994, 784 p., Price US $34.00 (paper), ,US $55.30 (cloth)) Neuroscience is a rapidly growing, multi-disciplinary field encompassing many subtopics and disciplines. The author succeeded in performing what seemed to be an almost impossible mission: writing a comprehensive updated and well written textbook of neurobiology within reasonable spatial constraints. The book refers to almost all major topics in neuroscience from the molecular biology of ionic channels and receptors through cellular electrophysiology and functional neuroanatomy to animal behaviour. It is written in a clear and lucid style with many accompanying illustrations and diagrams that complement the written text. The bibliography of the book is extensive and up to date; it will allow fast and easy access to further reading. Contrary to many textbooks, which are written in a dry and boring style, an especially attractive feature of this book is the delightful style of writing which makes reading a real pleasure. Each chapter can be read separately but, if read as a whole, the book takes the reader to a fascinating organised tour of neurobiology which allows the reader to get both a generalised integrated view of neurobiology and relatively detailed knowledge of many subtopics. Unfortunately, certain sections are too condensed. As a result, the naive reader will find it difficult to fully understand certain difficult subjects such as voltage clamping, the auditory end organ or the information processing in the retina. Despite this inevitable disadvantage, I strongly recommend this book as a textbook for undergraduate and medical students, or as a general reference book for neurobiology. In my mind, it is far superior to other textbooks of its kind.
Yitzhak Schiller
Max-Planck-Institut fir Medizinische Forschung Heidelberg
95 (1995) 143-146
Each chapter, which covers a different clinical genetic disorder or related group of disorders, tends to include a comprehensive abstract, brief but usually solid review of the clinical features of the disorder and an account of the clinical course. This is followed by a discussion of pathology and current recommended therapy. During this clinical section there is often some attempt to cover aspects of EEG and evoked potential data, but the comprehensiveness and accuracy of this coverage vary with the authors and syndromes covered. Obviously, the strength of each chapter in the volume lies in the longer and more expert sections on inheritance, epidemiology, genetics, and pathophysiology which follow the clinical review. Where applicable, interesting sections concerning data from animal models are also included. The references listed at the end of each chapter are numerous and comprehensive, providing a good immediate source of further information. Almost the entire gamut of inherited disorders that affect infants and children are covered in this book, making it of particular value to pediatric neurologists, developmental pediatricians and geneticists who see children who have developmental or progressive neurological disorders. Other disorders are of particular interest to the adult neurologist or internist who see adult patients, including those who have multiple sclerosis or Alzheimer disease. Peripheral neuromuscular disorders are also included. One chapter devoted to the genetic epilepsies provides a brief but effective review of definitions of seizures and the epilepsies and a useful review of some of the experimental models of genetic epilepsies that would be very appropriate for medical students and residents. Although it is quite comprehensive, the volume concentrates on major categories of disorders and does not detail some rarer entities. The information concerning each disorder is not presented in such a way that it would be expected to help the clinician make a diagnosis at the time he or she is evaluating a patient in the office or clinic. The usefulness of this volume is in helping the physician, once a clinical diagnosis has been entertained or achieved, to gain a broader understanding of the background genetic information and the rationale and status of modern diagnostic tests and current therapy. It especially gives food for thought about genetic therapies that are currently evolving and may be applicable in the near future. Jane F. Donat Division of Pediatric Neurology, Children’s Hospital, 4800 Sand Point Way NE, Seattle, WA (USA)
(Germany)
The molecular and genetic basis of neurological disease. - R.N. Rosenberg, S.B. Prusiner, S. Di Mauro, RL. Barchi and L.M. Kunkel (Eds.) (Butternorth-Heinemann, Stoneham, MA, 1993, 1023 p., Price us $225.00) This is a large and comprehensive multi-authored text which proposes to cover this “rapidly evolving field,” to use a term from the preface, and continually increasing number of disorders of the nervous system that are thought to have a genetic basis. Specifically, a major goal of this volume is to be “at the cutting edge of the interface between clinical and basic neuroscience.” The first several chapters comprise a review of basic principles of molecular genetics and gene therapy and include definitions of much modem terminology that provide a useful brief update for neurologists who are not experts in these areas. Most of the rest of the book covers a vast array of groups of disorders or individual disorders that have, or are suspected to have, a genetic basis. Although individual chapters are authored by over 100 different authorities, each expert in the particular areas covered, the many chapters are written in fairly similar styles.
Idiopathic generalized epilepsies. - A. Malafosse, P. Genton, E. Hirsch, C. Marescaux, D. Broglin and R Bernasconi (Eds.) (John Libbey, London, 1994, 560 p., Price &I45.00) This volume consists of 7 major parts: (1) Current approaches, (2) Familial neonatal and infantile convulsions, (3) Absence seizures and absence epilepsy, (4) Juvenile myoclonic epilepsy and related syndromes, (5) Photosensitivity, (6) Pathophysiology of convulsive seizures, and (7) Fundamental and therapeutic aspects. These sections encompass a total of 43 chapters. The result is an up-to-date overview of contemporary work in the field. Clinical and experimental aspects appear to be harmoniously blended but, with perusal of the work, it becomes evident that the main emphasis has been placed on the basic sciences of epileptology with the inclusion of genetics. This is not felt to be a drawback of this volume; it is pointed out in order to guide the prospective buyers and readers. The basic philosophy of this work can be exemplified by the discussion of a few chapters. Chapter 2 (“Clinical and electroencephalographic characteristics of idiopathic generalized epilepsies”) was entrusted to Joseph Roger with a group of coworkers from Marseilles and Barcelona _ an optimal choice for this pivotal chapter but with a space allotment
Book Reviews/Electroencephalography
and clinical Neurophysiology
In conclusion, this software and the accompanying book offer a good and enjoyable way of exploring the properties of different ionic currents, and the way they interact and determine the electrical response of neurons. Yitzhak Schiller
Max-Planck-Institut fiir Medizinische Forschung, Heidelberg
(Germany)
Neurobiology, 3rd JCdn.- G.M. Shepherd (Oxford University Press, New York, 1994, 784 p., Price US $34.00 (paper), ,US $55.30 (cloth)) Neuroscience is a rapidly growing, multi-disciplinary field encompassing many subtopics and disciplines. The author succeeded in performing what seemed to be an almost impossible mission: writing a comprehensive updated and well written textbook of neurobiology within reasonable spatial constraints. The book refers to almost all major topics in neuroscience from the molecular biology of ionic channels and receptors through cellular electrophysiology and functional neuroanatomy to animal behaviour. It is written in a clear and lucid style with many accompanying illustrations and diagrams that complement the written text. The bibliography of the book is extensive and up to date; it will allow fast and easy access to further reading. Contrary to many textbooks, which are written in a dry and boring style, an especially attractive feature of this book is the delightful style of writing which makes reading a real pleasure. Each chapter can be read separately but, if read as a whole, the book takes the reader to a fascinating organised tour of neurobiology which allows the reader to get both a generalised integrated view of neurobiology and relatively detailed knowledge of many subtopics. Unfortunately, certain sections are too condensed. As a result, the naive reader will find it difficult to fully understand certain difficult subjects such as voltage clamping, the auditory end organ or the information processing in the retina. Despite this inevitable disadvantage, I strongly recommend this book as a textbook for undergraduate and medical students, or as a general reference book for neurobiology. In my mind, it is far superior to other textbooks of its kind.
Yitzhak Schiller
Max-Planck-Institut fir Medizinische Forschung Heidelberg
95 (1995) 143-146
Each chapter, which covers a different clinical genetic disorder or related group of disorders, tends to include a comprehensive abstract, brief but usually solid review of the clinical features of the disorder and an account of the clinical course. This is followed by a discussion of pathology and current recommended therapy. During this clinical section there is often some attempt to cover aspects of EEG and evoked potential data, but the comprehensiveness and accuracy of this coverage vary with the authors and syndromes covered. Obviously, the strength of each chapter in the volume lies in the longer and more expert sections on inheritance, epidemiology, genetics, and pathophysiology which follow the clinical review. Where applicable, interesting sections concerning data from animal models are also included. The references listed at the end of each chapter are numerous and comprehensive, providing a good immediate source of further information. Almost the entire gamut of inherited disorders that affect infants and children are covered in this book, making it of particular value to pediatric neurologists, developmental pediatricians and geneticists who see children who have developmental or progressive neurological disorders. Other disorders are of particular interest to the adult neurologist or internist who see adult patients, including those who have multiple sclerosis or Alzheimer disease. Peripheral neuromuscular disorders are also included. One chapter devoted to the genetic epilepsies provides a brief but effective review of definitions of seizures and the epilepsies and a useful review of some of the experimental models of genetic epilepsies that would be very appropriate for medical students and residents. Although it is quite comprehensive, the volume concentrates on major categories of disorders and does not detail some rarer entities. The information concerning each disorder is not presented in such a way that it would be expected to help the clinician make a diagnosis at the time he or she is evaluating a patient in the office or clinic. The usefulness of this volume is in helping the physician, once a clinical diagnosis has been entertained or achieved, to gain a broader understanding of the background genetic information and the rationale and status of modern diagnostic tests and current therapy. It especially gives food for thought about genetic therapies that are currently evolving and may be applicable in the near future. Jane F. Donat Division of Pediatric Neurology, Children’s Hospital, 4800 Sand Point Way NE, Seattle, WA (USA)
(Germany)
The molecular and genetic basis of neurological disease. - R.N. Rosenberg, S.B. Prusiner, S. Di Mauro, RL. Barchi and L.M. Kunkel (Eds.) (Butternorth-Heinemann, Stoneham, MA, 1993, 1023 p., Price us $225.00) This is a large and comprehensive multi-authored text which proposes to cover this “rapidly evolving field,” to use a term from the preface, and continually increasing number of disorders of the nervous system that are thought to have a genetic basis. Specifically, a major goal of this volume is to be “at the cutting edge of the interface between clinical and basic neuroscience.” The first several chapters comprise a review of basic principles of molecular genetics and gene therapy and include definitions of much modem terminology that provide a useful brief update for neurologists who are not experts in these areas. Most of the rest of the book covers a vast array of groups of disorders or individual disorders that have, or are suspected to have, a genetic basis. Although individual chapters are authored by over 100 different authorities, each expert in the particular areas covered, the many chapters are written in fairly similar styles.
Idiopathic generalized epilepsies. - A. Malafosse, P. Genton, E. Hirsch, C. Marescaux, D. Broglin and R Bernasconi (Eds.) (John Libbey, London, 1994, 560 p., Price &I45.00) This volume consists of 7 major parts: (1) Current approaches, (2) Familial neonatal and infantile convulsions, (3) Absence seizures and absence epilepsy, (4) Juvenile myoclonic epilepsy and related syndromes, (5) Photosensitivity, (6) Pathophysiology of convulsive seizures, and (7) Fundamental and therapeutic aspects. These sections encompass a total of 43 chapters. The result is an up-to-date overview of contemporary work in the field. Clinical and experimental aspects appear to be harmoniously blended but, with perusal of the work, it becomes evident that the main emphasis has been placed on the basic sciences of epileptology with the inclusion of genetics. This is not felt to be a drawback of this volume; it is pointed out in order to guide the prospective buyers and readers. The basic philosophy of this work can be exemplified by the discussion of a few chapters. Chapter 2 (“Clinical and electroencephalographic characteristics of idiopathic generalized epilepsies”) was entrusted to Joseph Roger with a group of coworkers from Marseilles and Barcelona _ an optimal choice for this pivotal chapter but with a space allotment