348 Book Reviews

more available paperbacks, such as the present volume.

For the uninitiated a glossary is essential and is here placed at the beginning. Unfortunately, some important and often-used terms (such as transgenic, mini- and microsatellites) are not included, and some other definitions (for ex- ample, alternative splicing) seem unnecessarily complicated. There then follows the now obliga- tory chapter on Principles, which is well written but would have benefited from more illus- trations.

The following 13 chapters deal with specific disorders or groups of disorders. Those which will be of greatest interest to readers of Neuro- muscular Disorders include hereditary amyl- oidosis (Benson), Duchenne muscular dystrophy (Worton & Gillard), myotonic dystrophy (Roses & Margaret Pericak-Vance), neurofibromatosis (Wallace & Collins), Charcot-Marie-Tooth disease (Vance & Bird) and mitochondrial myopathies (Harding & Holt). The authors are all experts in their respective fields and, therefore, write with authority. The relationship between clinical phenotype and molecular findings, so often neglected in many recent reviews, is nicely presented, particularly in the case of Duchenne muscular dystrophy and ,myotonic dystrophy, the chapter on the former being especially comprehensive.

Each chapter concludes with references but oddly these are rarely cited in the text which the discerning reader may find frustrating. And some of the contributions have already been eclipsed by recent research findings. There does not seem to be any simple answer to this latter problem in a subject which is attracting so much attention from molecular geneticists and which is advancing so quickly.

A regular journal dedicated entirely to reviews (such as the Trends series) is one possibility. Alternatively, for those less informed about the details, which may still include many clinical neurologists, a good single-authored text on a specific disorder or groups of related disorders (such as Harper's Myotonic Dystrophy, Saunders) has much to recommend it. But then perhaps I am biased!

Meanwhile, the present volume has much in its favour and should have a place on the shelf of any neurologist with interests in these disorders.

ALAN EMERY

The Medical School University of Edinburgh

The Genetic Basis of Common Diseases. Edited by RICHARD A. KING, JEROME |. ROTTER and ARNO G. MOTULSKY. Oxford Monographs on Medical Genetics No. 20. Published 1992 by Oxford University Press, Oxford. ISBN 0 19 505482 2, 978 pp. Price £95.00.

This text follows the tradition of excellence of the Oxford Monographs on Medical Genetics series but differs from the others in the series being an edited multi-author text.

The first four chapters are general background covering the approach to the genetic bases of common diseases, including the concept and types of genetic susceptibility, familial aggre- gation, twin studies and genetic mechanisms; molecular genetics and recombinant DNA technology; human gene mapping, linkage and association; the methods and interpretation of the analysis of genetic data including twin studies, testing modes of inheritance and risk estimation for genetic counselling.

If there is a criticism of this portion of the text, it would be that more detail could be given to the concepts and appropriate use, with examples of linkage, disease association and segregation analysis for persons less familiar with these concepts (i.e. non-geneticists) in the analysis of genetic data.

The remaining 42 chapters cover disorders of a specific system of the body such as structural heart disease, a particular disorder such as rheumatoid arthritis, or a group of disorders such as inflammatory bowel diseases. Each chapter starts with a section on the definition and diagnosis of the disease or disorder(s), then considers the relevant genetic and non-genetic epidemiological factors, the evidence for the biological basis of genetic susceptibility and then has a short section on clinical considerations, such as counselling and therapy, concluding with an extensive reference list.

The chapters/topics of strictly neurological interest include epilepsy, multiple sclerosis, Parkinson's and Alzheimer's diseases. The chapter on epilepsy usefully reviews the results of different family studies of recurrence risks for the various types of epilepsy and includes a section on the teratogenic effects of anticonvulsant drugs in pregnancy. The chapter on Alzheimer's disease is particularly well written with recurrence risk data based on the age of onset and a critical appraisal of recent findings in relation to amyloid. The inclusion of a chapter on Parkinson's disease might seem slightly un-

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usual as there is little evidence for a significant genetic contribution to its aetiology. Other chapters/topics of potential "neurological" interest include schizophrenia, the major affect- ive disorders, alcoholism and panic disorders.

The authors indicate that future edition(s) are planned. This would be essential in order to keep the text up to date and relevant due to the rapid rate of progress in many of the diseases covered, as witnessed, for example, by the recent report of genetic susceptibility to multiple sclerosis with the myelin basic protein gene.

In summary, this text is welcome as it provides a comprehensive review of the current state of understanding of the genetic basis of many of the common diseases, but is unlikely to be one which an individual would have in his or her own personal library, but is an essential reference to have in a departmental library.

R. F. MUELLER Department of Clinical Genetics

St James's University Hospital Leeds

Guillain-Barr6 Syndrome. GARETH J. PARRY. Published 1993 by Georg Theime, Stuttgart. ISBN 3 13 783601 8, 200 pp. Price DM 90.

This review of the Guillain-Barr6 syndrome starts with a history of the disorder, which explains the interest among neurologists out of proportion to its incidence. A detailed account of the clinical features is given, and the importance of the autonomic disorders is emphasized as these may result in the most life-threatening complications. For example, there may be abnormalities of blood pressure, cardiac rhythm, and inappropriate excretion of antidiuretic hormone. Atypical forms are considered, including the unresolved relationship of the Miller-Fisher syndrome.

The diagnosis of the Guillain-Barr~ syndrome is usually, in the first instance, a clinical one confirmed by the well-known albu- minocytological dissociation in the CSF; but there can be difficulties. For instance, it seems likely that the Chinese paralytic syndrome, pos- sibly due to a virus, may be more widespread than at first thought. This was certainly originally presented as a kind of epidemic of the Guillain-Barr6 syndrome.

Particular importance is given to the results of electrophysiological tests, and this is likely to be of special help to those unfamiliar with such

investigations. Such tests can be used in diag- nosis, in assessing the progress of the disease, and in prognosis. Certainly anyone involved in research into the polyneuropathies will find this chapter, and the references, of value.

In a disease in which the majority will make a complete recovery, supportive treatment, such as mechanical ventilation and the management of autonomic instability will be vital. It is rightly stressed that success depends on attention to detail; and of even greater importance is the ability to anticipate complications and prevent them. When it comes to more specific treatment there is more controversy. Although the syn- drome is almost certainly an autoimmune disease, steroids and other immunosuppressants are not recommended. Plasmaphoresis can be helpful, especially if started early, but intra- venous infusions of human immunoglobulin may be the treatment of choice when the condition is severe.

Epidemiological studies show that the Guillain-Barr6 syndrome is associated with all kind of infections, and other disorders. The mechanism is uncertain but some form of im- mune response seems likely. The pathology is reviewed, and there seems to be no doubt that there is a macrophage-mediated demyelination, particularly of the nerve roots. The controversy over the respective roles of cell-mediated and humeral immunity is given special attention. It is possible that both mechanisms may operate in different cases. A final chapter contrasts chronic inflammatory demyelinating polyradiculo- neuropathy with the Guillain-Barr6 syndrome.

The summaries at the end of each chapter are helpful, and the references are comprehensive. This book can be recommended to anyone interested in this syndrome. It is a mine of information, and will, undoubtedly, help those involved in the management of patients with peripheral neuropathies.

NElL GORDON Huntlywood

Wilmslow, Cheshire

Brown-S6quard: A Visionary of Science. MICHAEL J. AMINOFF. Published 1993 by Raven Press, New York. ISBN 0 88167 956 9, 223 pp. Price $85.

Until now little has been written of Charles Edouard Brown-S6quard, one of the luminaries of French neurology in the last century. In an era