Human Motor Neuron Diseases. Advances in Neurology, Vol. 36

TINS -July 1983 Human Motor Neuron Diseases. A d v a n c e s in N e u r o l o g y , V o l . 3 6

edited by Lewis P. Rowland, Raven Press, 1982. $117.80 (xiv + 577pages) ISBN 0 890 04737 5 Selective alteration in function or loss of particular sets of neurons characterizes a number of neurological disorders. Some of these may reflect the cellular preferences of neurotropic viruses, as in acute anterior poliomyelitis. Others may depend upon the selective vulnerability of certain neurons to neurotoxins. Yet others are the consequence of the metabolic differences between groups of cells in the nervous system that may be selectively affected by inborn errors in metabolism or as the consequence of vitamin deficiency. The motor neuron diseases constitute a group of disorders in which the motor neurons - lower, upper, or both - are selectively affected by the disease process. In the past, poliomyelitis was the most devastating example. Now it is amyotrophic lateral sclerosis (ALS). ALS is one of the most wretched conditions that neurologists see. Inexorable progression of motor paralysis with total retention of mental faculties leads inevitably to death, usually within five years of the onset of symptoms, frequently less. When the bulbar muscles are affected, difficulties in swallowing that lead to problems in feeding and consequent inanition, and to choking with the aspiration of saliva and food into the respiratory passages, are particularly unpleasant for the patient. Human Motor Neuron Diseases, edited by 'Bud' Rowland, surveys, we are told on the inside cover, recent insights into the pathogenesis of these disorders and 'offers clues as to the cause of amyotrophic lateral sclerosis'. This statement is perhaps somewhat over-enthusiastic but Rowland has assembled most of the workers active in the field and has produced an authoritative and reasonably comprehensive coverage of this important area. The book arose from an international conference organized by the Muscular Dystrophy Association of America, held in 1981 in Scottsdale, Arizona. The reviewer is no great enthusiast of the current vogue of producing books out of conference proceedings. They are usually dreary documentations of previously published work or work that is about to be published more fully elsewhere (it is not only the publishing companies that are at fault). This book is an exception. The wide coverage makes it difficult to review the contents in any detail. Rowland sets the stage by discussing the terminological difficulties in this highly disparate group of conditions and the problems in identification of individual clinical entities,

289 a question taken up specifically in relation to ALS by D. W. Mulder. Physiological aspects of normal motor neuron function receive scant attention but Eric St~tlberg contributes an interesting electrophysiological study of muscle reinnervation in ALS. Pathological aspects are dealt with more extensively with useful reviews on ALS by Trevor Hughes and Asao Hirano. Inherited motor neuron diseases are then discussed, including both those in which motor neuron involvement is the main disease feature and those in which it is part of a more widespread neuronal degeneration. The recent interesting finding that hexoseaminidase deficiency may present with a syndrome that mimics the KugelbergWelander type of hereditary spinal muscular atrophy or with a disorder that resembles ALS is reviewed by W. G. Johnson. This section is followed by one on acquired motor neuron diseases, with particular emphasis on heavy-metal intoxications. Virology is given justifiable prominence S u b s t a n c e P in t h e N e r v o u s S y s t e m . C i b a F o u n d a t i o n S y m p o s i u m 91

edited by Ciba Foundation 1982, Pitman, 1982. (Distributed in the USA by Ciba Pharmaceutical Company -Medical Education Administration.) £25.00/$35.00 (x + 349 pages) ISBN 0 2 72 79655 7 Conference volumes should provide an up-to-date account of the field, usually with a historical r6sum6 and some pointers to the future development of the area. In reality, the majority of conference proceedings are a cynical commercial and scientific exercise, collecting under a general title a range of disparate and thinly written chapters. If these volumes then offer at best an unrefereed publication, should the format be taken seriously and does it, given the long publication delays, offer any advantage to research scientists? The tremendous advantage that conference volumes can have over all other forms of scientific communication is brilliantly illustrated in the Ciba Foundation Symposium 91, Substance P in the Nervous System, and lies mainly within the extensively documented discussion period following each chapter. What makes the discussion period so fruitful is unclear, but it may well reflect the Ciba format of holding the meeting 'behind closed doors' without an audience other than the speakers themselves. The discussion serves several functions. It highlights, usually under the nonpartisan and headmasterly direction of the Chairman, A. S. V. Burgen, the various important points in the communication, stimulates comment from the audience and

in view of the longstanding but unproven suggestion that ALS could be due to a chronic neurotropic viral infection. The motion that ALS may sometimes occur as a late consequence of poliomyelitis still lingers on and receives comment. The experimental pathology of the motor neuron diseases provides some of the most interesting chapters and it is in this area that ideas such as ALS being a disorder of DNA are explored. ALS represents a major challenge for neurologists and experimental neuropathologists alike. Its solution will not be easy. The present situation is aptly summarized in the concluding section on 'Therapeutic Trials' which largely concentrates on methodological considerations for future attempts at treatment. P. K. THOMAS

Professorof Neurology, Departmentof Neurology, Royal Free Hospital School oJ Medicine, Pond Street, London NW3 2QG, UK. serves to review the communication, suggesting alternative interpretations of data, and in some cases even allowing the presentation of conflicting data. The early history of substance P and its subsequent isolation, sequencing and immunochemistry are covered in the opening chapters of the book and are followed by chapters covering all aspects of peptide localization, synthesis, breakdown, physiology and possible behavioural roles. Throughout the volume, however, one can detect a consistently contentious issue. Is substance P a neurotransmitter and is it involved in somatosensory sensation, particularly pain transmission? Substance P is found in some primary afferents which are thought to be involved in the peripheral neurogenic responses to injury. As the chapter by Lembeck and Gamse demonstrates, there is considerable evidence, including v, ork with synthetic peptide antagonists of substance P, that this may be a major function of the peptide. It therefore represents a very significant breakthrough in the organization of future research into the pharmacology of the peptide (a point clearly recognized by the organizers in their choice of the front-cover artwork). The peripheral process of sensory neurons has also been shown, in some cases, to collateralize and to terminate oo autonomic ganglion cells, providing a powerful model for the investigation of the slow EPSP thought to be produced by stimulationinduced release of the peptide. The central branch of the substance-P-containing sensory neuron has been attributed with the function of conveying pain messages from the periphery to the spinal cord, but as Wall