Brain, Vol. 122, No. 2, 370-371,
February 1999
© 1999 Oxford University Press
Book Reviews |
DISEASES OF THE NERVOUS SYSTEM IN CHILDHOOD.
By Jean Aicardi.1998. Cambridge: Cambridge University Press.Price £150. ISBN 1-898-68316-6..
University Hospital of Wales, Cardiff, UK
The practice of the neurology of childhood is a particularly fascinating and demanding occupation. Maturational and developmental changes in the anatomy, physiology and biochemistry of all parts of the nervous system influence the possible historical details and physical findings. The recognition of the early manifestations of disorders more readily diagnosed when fully developed is especially challenging. It is essential to have a comprehensive text with which to tackle the problems of children whose neurological signs are static, but very debilitating; those with deteriorating conditions; and those, such as the ones with epilepsy, for whom treatment can lead to improvement, if not always cure.
Jean Aicardi's encyclopaedic knowledge of the disorders which might cause neurological problems in children was recognized long before he produced the first edition of his definitive text. This second edition has been thoroughly updated. Although written almost exclusively by Aicardi, there are chapters on metabolic diseases and cerebral palsy to which Ogier and Bax have contributed. The section on developmental and neuropsychiatric disorders is by Christopher Gillberg. The layout and chapter headings remain the same, with sections on foetal and neonatal neurology; malformations, chromosomal abnormalities, neurocutaneous syndromes and skull malformations; neurological consequences of prenatal, perinatal and early postnatal interference with brain development; metabolic and heredodegenerative disorders; postnatal extrinsic aggressions (infections and injury); tumours and vascular disorders; paroxysmal disorders; affections of the ocular, visual, auditory and vestibular systems; neuromuscular diseases; neurological manifestations of systemic diseases; and neuropsychiatry.
The major strengths are the tables and the references. The former complement the text admirably. For example, in Chapter 1, a brief statement on inherited metabolic and neurodegenerative disorders with foetal onset is accompanied by a tabulated list of the causes, each with at least one reference. Thus, while giving the principles of the problem, and a guide to sources of further reading, the text is not overcrowded with detailed descriptions of each of the enormous number of conditions considered. The tables are particularly helpful in summarizing causes of disorders which even the paediatric neurologist may encounter only rarely, and in taking an individual finding, such as corpus callosum agenesis, and itemizing the syndromic forms. Some, for instance that listing the 36 main causes of facial weakness in childhood, are remarkably comprehensive: one wonders which rare aetiologies can have been omitted. In total, an additional 19 tables appear in this second edition. They amplify information on foetal neurology, malformations of the CNS, hydrocephalus and non-traumatic pericerebral collections, heredodegenerative disorders, infectious diseases, space-occupying lesions, seizure disorders, other paroxysmal disorders, disorders of the visual system, peripheral neuropathies, primary muscle diseases and specific learning disorders. Thus, they indicate the diversity of the updating which has taken place.
Due acknowledgement is made of advances in neurometabolic and genetic fields, and in, for example, the neuronal ceroid lipofuscinoses, better definition of the clinical phenotypes. Inevitably, genetic linkage is mentioned in places where, more recently, the gene has been identified.
Neuroimaging has become much more sophisticated since the first edition was produced in 1992. This edition contains more illustrations; they are larger and clearer, and MRIs replace some CTs. There are no pictorial examples of SPECT or PET in the chapter on epilepsy, though these forms of investigation are described. Some illustrations of fMRI and MRS and the role of these tests might be considered for the next edition: they are referred to only very briefly in this one. There are areas in paediatric neurology in which particularly major advances have been made in recent years. Aicardi produces a succinct presentation of the recent clinical and genetic information on limb girdle muscular dystrophies. He tabulates a classification of peroxisomal disorders. Information is given on the biochemical background, pathogenesis, genetic background, neuropathology and clinical features of the various types of the carbohydrate-deficient glycoprotein syndrome. The newer anti-epileptic drugs are included in his recommendations for the treatment of epilepsy. In future editions, the role of molecular genetics will undoubtedly be given more prominence.
The wealth of references is unrivalled by any other text on child neurology. Each chapter is followed by those relevant to that section, making access easy. The references have been substantially updated, without loss of important previous publications. The indexing is comprehensive and accurate. The child neurologist who has correctly identified the physical signs and made a diagnosis with the help of Aicardi should have no problem with accessing additional information about even the rarest of conditions; but management, and, in particular, the choice of drug, from those listed for specific conditions, is not always as well delineated.
This second edition is easier to handle than its predecessor. It is slimmer, but taller and wider. There are fewer pages, but each contains many more words, which, although in smaller print, remain entirely legible with ease. The presentation is logical, clearly stated and easy to read. There are no problems with finding one's way around. It compares extremely favourably with other texts on child neurology, and is indisputably my favourite. Although the price is certainly not low the book is excellent value for money. It is doubtful whether practising paediatric neurologists would be satisfied with mere access to library copies: most will wish to have their own. However, the library should be persuaded to obtain this edition so that adult neurologists and general and community paediatricians have a wide ranging and accurate reference book available.
The current saying amongst paediatric neurologists is: `if it is not in Aicardi, it does not exist'! A greater compliment would be difficult to find.
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