Book Review

The Molecular and Genetic Basis of Neurological Disease

Molecular Basis of Neurology

N Engl J Med 1993; 329:587-588August 19, 1993

Article

The Molecular and Genetic Basis of Neurological Disease
Edited by Roger N. Rosenberg, Stanley B. Prusiner, Salvatore Dimauro, Robert L. Barchi, and Louis M. Kunkel. 1023 pp., illustrated. Boston, Butterworth-Heinemann, 1993. $225. ISBN: 0-7506-9069-0

Molecular Basis of Neurology
(Molecular Basis of Clinical Medicine.) Edited by P. Michael Conneally. 296 pp., illustrated. Boston, Blackwell Scientific, 1993. $39.95. ISBN: 0-86542-150-1

Over the past decade, molecular genetic investigations of neurologic disease have entered a veritable golden age. With the recent cloning of the genes for Huntington's disease, amyotrophic lateral sclerosis, and neurofibromatosis 1 and 2, among others, molecular-based studies of nervous-system disorders reached a new pinnacle. They not only serve as effective models for logically determining the etiologic basis of inherited maladies in humans, but also are beginning to confirm the momentous promise of molecular biology for eventually treating neurogenetic abnormalities. It is not surprising, then, that this field has progressed at a whirlwind pace. Even those who are deeply immersed in molecular neurologic research cannot easily keep up with the newest findings and state-of-the-art techniques. Moreover, many highly competent investigators in this field possess only a skeletal knowledge of the clinical, pathological, and therapeutic aspects of neurologic abnormalities, including those of the disease that they are dissecting on a molecular level.

The two books under review may be regarded as godsends not only to the molecular biologists conducting molecular genetic studies of neurologic disease, but also to the practicing physician. The longer, more comprehensive book edited by Rosenberg et al. provides at once a comprehensive review of the extraordinary achievements in the fields of molecular neurobiology and neurogenetics and outstanding descriptions of the clinicopathological features, methods of diagnosis, and available therapies for each disorder. The more compact book, edited by Michael Conneally, serves a similar purpose and is likewise excellently produced. In both works, the contributing-author lists read like a “who's who” in molecular neurogenetics. After using these two books as references during my day-to-day research in a laboratory dedicated to molecular-neurogenetic studies, I came to the conclusion that they are analogous to comprehensive computer data bases of molecular neurology, with one contained in a desktop terminal and the other in a portable lap-top unit.

To test the usefulness of the book by Rosenberg et al., I took advantage of the situation I found myself in when I first received it for review. I had just begun performing molecule-based experiments on a neurologic disorder of whose clinical and pathological features I had only peripheral knowledge -- Wilson's disease. I turned to the chapter by John Menkes in the section on “Metal Metabolism” and found a superbly written chapter that was comprehensive, up to date, and highly informative. On scanning and reading the other chapters in the book, I realized that for me as a researcher in molecular neurogenetics, this book is a necessary and invaluable asset. I was astonished at the breadth and scope of this work and the detail in each chapter. The editors are to be congratulated for compiling the papers of more than 100 contributing authors in 66 outstanding chapters, divided into 22 parts ranging from “General Topics” to “Membrane Excitability Disorders” to “Gene Therapy and Human Genome.” This monumental book fills an important niche by providing cutting-edge information that ties together the clinical and molecular aspects of neurologic disease. Each chapter is written by an uncontested authority in the field and is capable of informing the practicing neurologist about recent molecular breakthroughs in a very “user friendly” manner. Similarly, each chapter describes up-to-date clinical information in a highly palatable fashion for the interested molecular biologist or geneticist.

The book edited by Conneally may be the shorter of the two reviewed here, but it is equally rigorous in presenting information targeted at the neurologist who is practicing molecular medicine or the investigator in a molecular-neurobiology laboratory. The introduction maintains that this book was written to assist physicians who wish to assimilate new knowledge emanating from neurogenetic research for their patients' benefit. Like the Rosenberg book, Conneally's begins with a refresher on the basic principles of molecular genetics. This chapter (by Jeffrey Towbin) is extremely informative and superbly written. For the physician who wishes to understand basic as well as state-of-the-art concepts of molecular biology, the book is worth buying for this chapter alone. The general topics and diseases covered in this work represent a subset of those included in the longer book by Rosenberg et al. and range from hereditary amyloidosis to the fragile X syndrome, in a total of 13 chapters. Each chapter begins with clinical aspects of the disease, then discusses recent developments in molecular approaches to the disorder -- all of which will benefit physicians and investigators alike.

Though there is some overlap between these two books in subject matter and authorship, both clearly deserve places on the bookshelf. As is always the case with textbooks, some of the material is bound to be outdated by publication time. However, both Rosenberg et al. and Conneally have done a highly commendable job of providing timely information. The book by Conneally can be effectively used as a highly informative, reliable, and compact source of information on recent developments in molecular neurogenetics. That by Rosenberg et al. is certain to become an essential neurology textbook that will serve splendidly to bridge the clinical and molecular aspects of neurologic disease for the investigator and the physician.

Rudolph E. Tanzi, Ph.D.
Massachusetts General Hospital, Boston, MA 02114