Book Review

Handbook of Amyotrophic Lateral Sclerosis

N Engl J Med 1993; 328:1721-1722June 10, 1993

Article

Handbook of Amyotrophic Lateral Sclerosis
Edited by Richard Alan Smith. 783 pp., illustrated. New York, Marcel Dekker, 1992. $195. ISBN: 0-8247-8610-6

These are exciting times for investigators of neuromuscular diseases. With the explosion of information about the human genome, we are advancing on the mysteries of many diseases, such as Duchenne's muscular dystrophy, that were formerly thought to be unsolvable. Exciting advances in research on amyotrophic lateral sclerosis include the identification of the locus for the gene in familial cases, the identification of unique electrophysiologic features that herald a more benign prognosis for some patients, and the recognition of immunologic abnormalities that may identify a treatable form of the disease. Of particular interest is a new approach to therapy that uses genetically engineered nerve growth factors. As knowledge expands, the likelihood is increasing that amyotrophic lateral sclerosis is a clinical syndrome with several different causes. Therefore, it is essential for clinicians and investigators to understand the clinical syndromes and the pathogenic possibilities.

These advances have prompted several meetings and books. The Handbook of Amyotrophic Lateral Sclerosis is a new one on this list. It is divided into two sections, one on diagnosis and management and the other on research strategies. Stephen Hawking, arguably the most famous person of our time with amyotrophic lateral sclerosis, recounts a history of his own symptoms. Hawking's life has been nothing short of amazing. To become one of the greatest thinkers of this century, he has had to overcome daunting handicaps daily, and he has been appropriately honored for his physical achievements. Hawking's conclusion gives his key to survival: he emphasizes living with amyotrophic lateral sclerosis rather than dying of the disease -- quite different approaches.

The handbook contains 31 chapters contributed by 52 authors. The typeface is easy to read, but my version had several pages in which the bottom lines were of a different font and had different spacing. In addition, one of the authors consistently misspelled the often-used term “fasciculation,” which I found distracting. The micrographs and radiographs are well reproduced. As in most multiauthored textbooks, the contributions are of different quality and quantity. For example, there are exhaustive and authoritative reviews of epidemiology, pathology, neurobiology of the motor neuron, and neurochemistry, some with only tangential references to amyotrophic lateral sclerosis. Other contributions are more succinct, with discussions limited to amyotrophic lateral sclerosis.

There are several particularly noteworthy chapters. Forbes Norris reviews his experience acquired in more than three decades of caring for patients with amyotrophic lateral sclerosis in what may become a classic essay that can be appreciated by all physicians, regardless of their level of experience. Smith has also included several chapters that set this book apart from others on amyotrophic lateral sclerosis. Practical chapters address legal issues, ethical aspects of experimental treatments, and the pitfalls of experimental-treatment programs. David Greenblatt's chapter on caring for patients with amyotrophic lateral sclerosis is especially poignant reading for physicians who do not care for terminally ill patients.

The title is somewhat misleading, because it implies a more basic, “how-to-manage” or hands-on approach. But several important and practical clinically relevant issues have been omitted. For instance, there is no mention of the management of respiratory insufficiency. Other omissions include discussions of physical therapy, support groups, mechanical aids to preserve mobility, and the medications available to manage sleep, excessive salivation, and constipation. These issues, not commonly covered in scientific reviews, are usually addressed in clinical handbooks. As a state-of-the-art overview of the scientific issues, this book would have been improved by the inclusion of a more detailed discussion of recent therapeutic programs, such as those involving branched-chain amino acids, dextromethorphan (discussed only in terms of pharmacokinetics), and immunosuppression.

In summary, Smith has probably achieved his goal of providing an overview of amyotrophic lateral sclerosis. The book can be recommended to all those interested in neuromuscular disease. However, several other books present a better overview of the scientific status of amyotrophic lateral sclerosis, and the book by the late James Caroscio, Amyotrophic Lateral Sclerosis: A Guide to Patient Care (New York: Thieme, 1986), provides a more comprehensive approach to the practical problems of caring for patients with the disease.

Dale J. Lange, M.D.
Columbia-Presbyterian Medical Center, New York, NY 10032