Functional Recovery in Neurological Disease

on chromosome Xp2 1. Rowland's chapter provides an exciting introduction to what proves to be a disappointing volume. Many of the chapters which review current advances in a particular disease are too brief and not well written. For example, a chapter on the construction of a complete restriction map of human chromosome 19 describes, in less than three pages, the strategy used to accomplish such a feat. Other chapters, such as the chapter on the inherited neuropathies and the chapter on the inherited ataxias, present information which is interesting, but reflects the poor understanding of the nature of many of these disorders. Much of the work presented focuses on the identification of markers for neurological and neuromuscular disease. Many of the authors use pedigree analysis and the isolation of restriction fragment length polymorphisms (RFLP) to map and screen for disease. For example, Helmut Zander discusses his search for markers for multiple sclerosis in families in his native West Germany. The last chapter, by S. Chamberlain, C. Worrall, and R. Williamson, describes, in less than four pages, their attempts to map the locus for Friedreich's ataxia (FA); most of this chapter focuses on background information and not on their work, which has ruled out chromosomes 19 and 21 as possible locations of the FA locus. There are a few bright spots in this volume. A chapter authored by J.E. Fox and L.E. Rosenberg describes their discovery of a RFLP at the ornithine transcarbamylase (OTC) locus, using OTC cDNA probes; in addition, they nicely show how this RFLP can be used in the prenatal diagnosis of OTC deficiency, as well as in carrier detection and carrier exclusion. Two other excellent chapters in this book were both authored by Kay Tanaka, whose primary work has been the study of isovaleric acidemia. In these chapters, Tanaka clearly discusses the biochemical and enzymatic basis for this disorder. He then delves into his own research, which has shown that this disorder is due to a deficiency of isovaleryl CoA dehydrogenase activity. After describing the purification and characterization of five different acyl-CoA dehydrogenases, Tanaka brings us to his current focus on the molecular cloning of these five enzymes. This volume is intended for physicians, scientists, and students interested in the field of genetics and neurology. Pedigree analysis and restriction fragment length polymorphisms provide both simple and powerful tools in the study and diagnosis of genetic disorders. Inexcusably, this volume omits advances in other important areas such as nerve regeneration, the use of transgenic mice to study multiple sclerosis, and the cloning and characterization of receptors for neurotransmitters. The book presents an overview of neurological and neuromuscular disorders. Each chapter provides numerous references so that the reader can pursue relevant literature; however, this book focuses almost entirely on gene mapping and RFLPs and devotes no time to other advances in this field engendered by the development of recombinant DNA technology. FABIAN CHEN Medical Student Yale University School ofMedicine