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Channelopathies of the nervous system
  1. Michael Hanna

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    Edited by M R Rose and RC Griggs (Pp 347, £60.00). Published by Butterworth Heinemann, Oxford, 2001. ISBN 0-7506-4507-5

    Over the past few years there has been an explosion of knowledge regarding a group of diseases that have become known as the channelopathies. Like many new chapters of medical discovery it always seems obvious in retrospect. After all, ion channels are one of the most critical structures for normal neural activity. This cascade of new knowledge has now firmly established that dysfunction of both ligand gated and voltage gated ion channels may cause human diseases. The dysfunction either may be caused by autoimmune attack, such as myasthenia gravis, or may be the result of mutations in ion channel genes, such as the skeletal muscle channelopathies. In the main, channelopathies are disorders of excitable tissues and the nervous system is of course particularly affected.

    It is therefore timely that Channelopathies of the nervous system should be published to provide a snap shot of current knowledge in this area. The editors state that their aim is “to inform both clinicians and neuroscientists as to the state of the art of neurological channelopathies, both clinically and scientifically”. I think this has been achieved through the contributions of 34 recognized authorities in various subfields of neurological channelopathies.

    The foreword is particularly informative and sets the scene very well for what is to follow. In the preface the editors acknowledge that the ultimate importance of channelopathies and of ion channel dysfunction remains to be determined. It is certainly true that all the channelopathies defined to date are relatively rare diseases. However, there is of course enormous expectation that ion channel dysfunction will be important in the most common paroxysmal disorders: epilepsy and migraine. This remains unproved.

    The layout of the book is logical and generally user friendly. Each chapter stands more or less alone and as expected for a multiauthor text the styles vary. The book is divided into eight main parts: basic science, assessment of channel function (in vitro and in vivo), channel gene expression, genetic and acquired neuromuscular channel disorders, central nervous system disorders, toxin induced channel disorders, and potential channel disorders. Recent genetic discoveries indicate that proximal myotonic myopathy and Schwartz-Jampel have in fact both bitten the dust as potential channelopathies! I found the chapters on the central nervous system disorders especially readable although already out of date in what is such a rapidly expanding area.

    This is one of the first texts on this subject and I can recommend it to interested neurologists and neuroscientists.

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