Neurodegenerative diseases such as amyotrophic lateral sclerosis have always been a challenge to neurologists and to the scientific community in general. These idiopathic diseases target a particular neural system (the motor system, in the case of amyotrophic lateral sclerosis) and cause progressive neuronal death with no clear inflammatory or metabolic basis. Neurodegenerative diseases tend to occur both sporadically and, less frequently, in a pattern of autosomal dominant inheritance: 10 percent of cases of amyotrophic lateral sclerosis are familial.
Investigators previously considered neurodegenerative diseases to be abiotrophies, which are processes caused by accelerated aging. This concept led to a certain pessimism about our ability to understand and treat this disease. The mood has changed, and research on neurodegenerative diseases has never been more exciting, partly because mutant genes that cause the inherited forms of some neurodegenerative diseases have been identified. Intense activity in research on amyotrophic lateral sclerosis has been triggered by the recent identification of mutant copper-zinc superoxide dismutase type 1 (SOD-1) as the cause of 20 percent of cases of the inherited form of the disease. Most investigators believe that the mutant SOD-1 causes disease through a toxic gain of function. There are now reliable diagnostic criteria for amyotrophic lateral sclerosis, a host of treatment trials run by multicenter groups, and a drug that has been approved for its treatment by the Food and Drug Administration. Along with this interest has come a plethora of books on the subject and even a new journal dedicated to the disease. Despite the many existing publications, Eisen and Krieger have done a fine job with their overview of the field.
One reason for the success of this book is that it is not an edited collection with many contributors, replete with the problems common to such anthologies. Rather, it was organized and written by two neurologists who have had years of clinical and research experience in this field. As Rowland notes in the foreword, "the writing here is seamless, in contrast to multi-authored books."
The background of the authors ensures that issues relevant to neurologists interested in amyotrophic lateral sclerosis are discussed in a comprehensive and practical way. Readers will be impressed as well as educated by the data gathered from the authors' experience over a period of almost two decades with more than 600 patients with this disease. Among other things, the book details initial clinical presentations, describes other diseases that masquerade as amyotrophic lateral sclerosis, provides illustrative case reports, gives detailed examples of neurophysiologic and neuropathological findings, and provides mortality data. Also helpful is the information on treatment, which is accompanied by specific dosages and descriptions of personal experiences with the use of particular drugs. The authors manage to deal with issues in a fair and balanced way and provide their perspective on controversial issues, when appropriate.
This is a book for neurologists and medical students, especially those with a special interest in amyotrophic lateral sclerosis. Nonclinical neuroscientists who want to learn more about the disease will also find it educational. Although the book includes a discussion of basic-research topics relevant to amyotrophic lateral sclerosis, it emphasizes clinical research, particularly neuroimaging, neurophysiology, and trials of therapy rather than more fundamental neuroscientific issues. For example, there is no description of the neurobiology of the motor neuron and only a relatively brief description of SOD-1 (with no description of the biochemical, molecular biologic, or structural features of the enzyme), whereas more than 40 pages -- the longest chapter in the book -- cover clinical neurophysiology.
The book is relatively up to date, although more recent developments in this rapidly changing field are unavoidably omitted; for example, breakthroughs in the understanding of spinal muscular atrophy are not discussed. In addition, one could take issue with the inadequacy of the citations for a few topics; for instance, no references are cited in the discussion of the "wasted mouse" model. However, these are minor criticisms that do not detract from the success of this treatise on a disease that has never ceased to fascinate, inspire, and challenge.
Reviewed by Raymond P. Roos, M.D. Copyright © 2000 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
--This text refers to the
Hardcover
edition.
