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Neuromuscular diseases: from basic mechanisms to clinical management. Monographs in clinical neurosciences. Volume 18
  1. MICHAEL G HANNA

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    Neuromuscular diseases: from basic mechanisms to clinical management. Monographs in clinical neurosciences. Volume 18. Edited by: f deymeer (Pp 196, US$164.50). Published by Karger, Basel, 2000 ISBN 3 8055 702

    The monographs in clinical neurosciences series is an established forum designed to bridge the gap between developments in basic neuroscience and clinical practice. It is suggested that this volume, devoted to selected neuromuscular diseases, “will be of great value to neurologists and neuroscienctists”. Certainly, there can be little argument that basic science advances in these disorders, particularly in the area of molecular genetics, have been impressive in recent years. Such monographs which attempt to give a snap-shot of the current “state of the art” are therefore welcome. Importantly, many of the genetic advances are now available to the practising neurologist in the form of improved DNA based diagnosis and have therefore changed clinical practice and benefited patients. For example, muscle biopsy is often now unnecessary when certain muscle diseases can be diagnosed genetically. On the other hand, the hope that these advances will result in effective therapies for people with these disorders remains to be fulfilled. I was therefore pleased to see that in addition to chapters which neatly outline the genetic testing now available, there are chapters devoted to the thorny issue of myoblast transfer and to novel approaches to therapy in muscular dystrophies.

    This volume contains 12 chapters, each written by recognised authorities. The following diseases, with clinical and genetic or immunological data, are covered; the sarcoglycanopathies, fascioscapulohumeral dystrophy, myotonic dystrophy, muscle channelopathies, congenital myasthenic syndromes, myasthenia gravis, inherited peripheral neuropathies, acute inflammatory neuropathies, spinal muscular atrophies, and familial amyotrophic lateral sclerosis. Inflammatory muscle diseases, chronic inflammatory neuropathies, mitochondrial diseases, and metabolic muscle diseases are notably absent. However, the editor does not claim this to be a comprehensive review of all neuromuscular disorders. The format of each chapter varies considerably. Although this is partly a reflection of a multiauthor text, it is also a reflection of the degree to which basic science advances have impinged on clinical practice in the different areas considered.

    So who will this book appeal to? Certainly, it would be of interest to all clinical neurologists, but particularly to those running specialist neuromuscular clinics. I think that it would also be valuable to neurology trainees developing an interest in this area.

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