Amyotrophic Lateral Sclerosis: Diagnosis and Management for the Clinician.

Belsh JM, Schiffman PL, eds. Armonk, NY 10504, Futura Publishing Co Inc, 1996, hardback, 389 pp, $90.

The purpose of this book is to provide a detailed overview of amyotrophic lateral sclerosis (ALS), with particular emphasis on diagnosis and management. The book was prompted by an extensive study by the editors showing that the most likely reason for the frequent misdiagnosis in ALS is physicians' lack of familiarity with the disease and failure to consider differential diagnoses.

The book is written by 27 contributors, mostly physicians, but it is not only for physicians. As the editors explain, and as is supported in their text, this book is of interest not only to specialists who evaluate patients with ALS, but also to the general practitioner and any health care professional who works with patients with ALS.

The book addresses its audience at a very appropriate level so that if you knew absolutely nothing about this disease, you could begin and finish with this book and come away with a thorough understanding of ALS. The book is comprehensive and well supported, with numerous and current references in each chapter.

Part I, which contains the first nine chapters, deals with diagnosis. Chapters 1 and 2 deal with the etiology of ALS and a historical perspective on ALS. Chapter 3 not only defines ALS, but goes to the basic level of definitions such as upper and lower motoneurons. It is a well-written and easy-to-follow chapter, with helpful diagrams and supporting tables.

Chapter 4, on the clinical presentation of ALS, does a wonderful job of presenting the symptoms and signs (including sensory and cranial nerve signs) grouped by specific body regions such as upper and lower extremities or neck and trunk. There is a heavy emphasis on the importance of the neurological examination, and the fact that the diagnosis of ALS rests on clinical findings. The authors explain that although medical history and laboratory tests are key for diagnosis of other diseases, the presence of certain abnormal neurological signs and the absence of others help confirm the ALS diagnosis. Not only are the signs and symptoms and test results for what represents ALS covered in clear detail and supported in wonderful table summaries, but the symptoms and signs that are not common to ALS are covered. This constantly reminds the reader "what is not" ALS.

Chapters 5 and 6 elaborate on the differential diagnosis of ALS, looking at the non-motoneuron diseases, spinal muscular atrophies, and ALS-like syndromes. There are also fine tables and outlines that clearly explain the potential pitfalls in making a clinical diagnosis of ALS, and tips for avoiding false negative diagnoses as well as false positive diagnoses.

Chapter 7 is a well-explained and well-illustrated chapter on electrodiagnostic evaluation of the patient with suspected ALS. It begins with the historical aspects and includes tables with criteria for electrodiagnostic confirmation of ALS and steps in the electrodiagnositic examination. Standard motor and sensory nerve conduction studies, the types of lesions, and the expected results are discussed. There are also tables of muscles to test in the upper and lower extremities and fine diagrams for electrode placement. Again, differential diagnosis is also discussed here and provides good comparative information. Chapter 8, on neuropathology, is extensive and up-to-date, with electron micrographs comparing normal and abnormal spinal cord and brain-stem samples.

Chapter 9 is an excellent narrative on notable people with ALS such as Lou Gehrig and Jacob Javits. It provides a sensitive picture of patients with ALS and the obstacles they face. Lou Gehrig's narrative is powerful and well documented and shows the problems of misdiagnosis that plague anyone with this disease.

Part II of the book (seven chapters) covers management. Chapters 10 and 11 deal with concepts and management in the treatment of patients with ALS, discussing the multidisciplinary approach. They cover pharmacological, speech, nutritional, physical therapy, and occupational therapy. They also discuss neck and head splints, nonverbal communication, bowel and bladder problems, financial issues, and even hospices. Chapter 12 is on the neurobehavioral aspects of ALS and deals with the patient's, family's, and physician's reactions to the diagnosis; support needs; the cognitive problems associated with ALS; the dying process; and quality-of-life issues.

Chapter 13 discusses the rehabilitation management for the patient with ALS and the six stages of ALS from a rehabilitation perspective. Tables identify appropriate treatment for each stage. There is even a finely tuned table for treating bulbar signs. Adaptive aids are also discussed in detail. The suggested activities and types of exercise are well described and appear to be appropriate for each stage. The programs described emphasize active participation of the patient as a full partner in the therapeutic process as well as in planning.

Chapter 14 extensively discusses pulmonary function and respiratory management, beginning with a review of the pulmonary system. Chapter 15 discusses the many ethical and legal issues of ALS, including truth telling, treatment options, advance directives, terminal patient care, and right-to-die issues.

Chapter 16, the final chapter of this powerful and extensive book, describes a family's experience with ALS, written from the patient's perspective. This chapter ties all the reading together and forms an effective closure.

This book would definitely be an asset to any health care professional working with patients who have ALS. Of particular relevance to physical therapy: the use of the neurological examination in identifying the signs of this condition, the well-written chapter on rehabilitation management, and the discussion of the neuropathology of this condition. The emphasis on differential diagnosis--what is and what is not ALS--may be of particular importance for any physical therapist who works with direct access.

The only features that would have made this document more useful would have been an extensive glossary and an appendix summarizing diagnostic tests for ALS and their expected results.

This is a well-written and very powerful document on ALS, and I highly recommend this book for any health care professional working with patients who have ALS. It should be a standard reference in any medical library or neurology setting.

Barbara S Oremland, PT
University of Louisville
Louisville, Ky

Ms Oremland is Assistant Professor in the physical therapy program at the University of Louisville and is also a consultant with the university's Movement Disorders Clinic. She teaches general medicine neurology and adult neurology courses.