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Handbook of ataxia disorders
  1. NICHOLAS WOOD

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    Handbook of ataxia disorders. Edited by thomas klockgether(pp 689, US$215). Published by Marcel Dekker Inc, New York, 2000. ISBN0-8247-0381-2.

    There is a constant debate as to the value of textbooks in general in this information technology age. This is perhaps most marked in the field of molecular genetics, which is moving at such a pace that a chapter could almost be redrafted or updated on a weekly or at least monthly basis given the rapidity of new developments. Nevertheless there is value in collating all the current information and attempting to put a field in context. I think that such is the intention of a volume such as this. Klockgether has amassed an impressive field of contributors to this large volume on ataxic disorders. The book spans some basic neuroanatomy and neurophysiology of the cerebellum through clinical approaches to ataxic patients and then several chapters describing the many disorders that can affect the cerebellum and its connections, thus producing ataxia. Perhaps unsurprisingly given the area of progress in this field most of the chapters are given over to the genetic forms of ataxia and a relatively few to the non-hereditary ataxias. This of course does not represent the true pattern seen in a general or even specialist neurology clinic. However, I see no way around this as our knowledge of the non-hereditary is rather scant at present. This of course is in marked contrast with the progress over the past 5-10 years and our understanding of the genetic ataxias where we have seen identification of numerous forms of dominant and recessive ataxias. In most of the major areas the genes are identified and we have now moved into the field of molecular biology to try and illuminate the pathogenic pathway. These experiments are brought up in the relevant chapters.

    A constant problem in a multiauthor book such as this is a non-uniformity of style or perhaps even worse, repetition from one chapter to the next. I am pleased to note that this has largely been avoided and apart from a rather brief general introduction to most of the chapters which could apply to some others each author does not labour the point on the dominant ataxias, for example. However, sufficient information is given in each chapter to allow the reader to delve into that chapter alone to understand SCA 1 or SCA 3 for example. I think this is testimony to both good authorship and good direction from the editor. However, there are one or two minor irritations. For example, the referencing system in chapter 1 is different from other chapters. Also, within this chapter there is a small error but it makes part of the chapter difficult to read. The abbreviation for long term depression and long term potentiation are given as the same and therefore that section is more difficult to understand than it might be. These are small quibbles. My only other disagreement with the book came in chapter 27 when the author describes most late onset cerebellar degeneration as being cases of MSA. I would agree that a significant minority fall under this category but I think that there are larger numbers of patients in whom no definitive diagnosis can be made and yet do not go on to produce the other features of MSA. Overall I enjoyed this book and I think that it has a place in the library of a neurologist. It will probably be most attractive to those neurologists who see a significant number of ataxias or those who wish to update themselves in the general sense. However, it is quite expensive and I think that this may limit its attractiveness to the generalist.

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