Amyotrophic Lateral Sclerosis: Diagnosis and Management for the Clinician.

This book provides a comprehensive overview of the diagnosis and management of amyotrophic lateral sclerosis (ALS). The book consists of 16 chapters divided into two parts: Diagnosis (Chapters 1-9) and Management (Chapters 10-16).

In their preface the editors, both physicians, discuss the results of a retrospective study, performed in 1990, of their own patients with ALS. The study revealed that a surprisingly high number of the patients (42%) had originally been diagnosed with an incorrect neurological or medical condition, with ALS not even being considered. Those who were correctly diagnosed were often offered little information about the disease, treatment options, drug studies, or help with medical planning. This book is a step toward increasing awareness and knowledge of ALS presentation, diagnosis, and management.

Chapters 1 through 4 set the foundation for a discussion of the ALS diagnosis. Topics covered include the epidemiology of ALS and familial ALS (FALS) in the U.S. and globally; theories of the etiology of ALS; the definitions of terms necessary for the discussion of ALS and related disorders; and a classification system to aid clinicians in differential diagnosis. In Chapter 4 the typical and atypical signs and symptoms at the early and more advanced presentation of ALS are described, and the World Federation of Neurology's proposed diagnostic criteria for ALS are presented.

Chapters 5 and 6 comprise Parts 1 and 2 of a discussion of the differential diagnosis of ALS. Chapter 5 begins with a discussion of the difficulty of making the ALS diagnosis due to variations in clinical presentation, similarity of ALS to other conditions, failure on the part of physicians to consider ALS in the differential diagnosis, and lack of familiarity with the clinical presentation of ALS. Also discussed are the major neurological syndromes and diseases that present similarly to and are often confused with ALS. Particularly helpful for clinicians will be the sections throughout the chapter providing tips for avoiding false positive and false negative diagnoses. Chapter 6 deals with the clinical features, pathogenesis, laboratory investigations, and treatments of ALS-like syndromes and ALS variants. Chapters 7 and 8 continue the focus on diagnosis with in-depth reviews of electrodiagnostic evaluation and the neuropathology of ALS respectively.

Part 1 concludes with a chapter providing stories of notable people with ALS. The chapter puts a human face on the disease. The stories of these celebrated people, past and present, in the worlds of sports, entertainment, government, and science, are at once informative, touching, and inspiring. They also serve to illustrate the variety of possible psychological and physical effects that may be associated with ALS.

With Chapter 10 the focus of the discussion moves from diagnosis to management. In this chapter current treatment modalities are presented, organized into sections on pharmacological, physical agents, nutritional, physical and occupational, speech, swallowing, and respiratory treatments.

Chapter 11 presents short and long-term considerations in the management of patients with ALS from assessment and diagnosis, through the increase in physical problems that arise as the disease progresses, to the terminal phase. In Chapter 12 the various psychological reactions to ALS on the part of both patients and physicians are discussed, along with suggestions for both psychosocial and medical support.

Chapter 13 looks at the rehabilitation management of patients with ALS in the progression of ALS through six stages and comprehensively describes common problems and potential treatments and accommodations. In Chapter 14 the expected pulmonary function problems are described, along with assessment and management options.

The ethical and legal problems that may be confronted by physicians working with patients with ALS are considered in Chapter 15. Among the topics discussed are the issues involved in telling patients the truth about diagnosis and prognosis, educating patients about treatments, including experimental treatments, long-term care planning, and enhancing autonomy.

The book concludes with an inspirational, educational, and very personal account of one family's experience with ALS. The chapter encompasses the stages of psychological adjustment to ALS, the reactions of friends and the medical community, and many other factors commonly experienced by families affected by a chronic illness.

The editors have compiled a book that is readable, comprehensive, eminently practical, and logically ordered. The editors state that they expect this book to be of interest to specialists who are asked to evaluate patients when ALS is suspected, as well as general practitioners and health care professionals who may encounter such patients. For such people, this book would indeed be an important addition to their professional libraries.

Most of the chapter authors are physicians, and the book is clearly oriented toward the medical management of ALS. The lay person would likely find much of the information and terminology daunting and confusing. However, because the book is so comprehensive and the information so practical, any professionals who work with patients with ALS and their families should read and share the information within it.

Although this book primarily addresses ALS, its usefulness to rehabilitation professionals is extended due to the breadth of other disorders also discussed and the excellent coverage of psychosocial issues common to other chronic diseases and conditions. Further, the chapters on notable people with ALS (Chapter 9) and on one family's experience with ALS (Chapter 16) should be read by all people who work with individuals with chronic and disabling conditions. This book is therefore recommended, particularly to those involved with medical rehabilitation, but also to all rehabilitation professionals who work with people with chronic illnesses.