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Amyotrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice

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    Amyotrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice. Edited by andrew eisen and charles krieger. (Pp303, £45.00). Published by Cambridge University Press, Cambridge, 1998. ISBN 0 521 58103 6.

    From its very first sentence, this monograph on amyotrophic lateral sclerosis is anchored in its writers’ personal experience. Its contents are, they tell us, “based on 664 patients”—“all examined by one of the authors”. Professor Eisen and Dr Krieger have reviewed all aspects of the condition, from molecular mechanisms to bedside care. Their approach is essentially focused on clinical issues, but the book is also a useful introduction to the basic science. They review the diagnostic process from the standpoint of the clinic room, stressing both the rarity of conditions that mimic the disease and, in view of the gravity of the diagnosis, the vital need to seek out any potentially treatable disorder. The stamp of seasoned knowledge is particularly evident when the text turns to electrophysiology. Here, readers are offered precise advice and encouraged to perform techniques—such as recording corticomotoneuronal excitatory postsynaptic potentials—themselves.

    The personal approach that underpins the book sometimes leads the argument in unexpected directions. On only the third page, studies of sex hormones are discussed to explain the male predominance in young onset cases. In this and other areas “not shared- by conventional dictum-” as the authors’ preface has it, references are mainly to their own papers. In the chapter on therapy, which opens with a survey of trial methodology and reviews the underlying mechanisms of potential therapeutic agents, only four pages are spent on symptomatic treatment of the patient. This brief overview touches on, but hardly does justice to such topics as respiratory support and gastrostomy feeding—two interventions of established benefit. Moreover, it makes scant mention of the need for multidisciplinary care. For a summary of these issues, readers would find Mitsumotos’s Care and Management of the Patient with ALS much more useful.

    The superspecialist may quibble over the odd detail (there is no mention of the dual pattern of inheritance of the D90A superoxide dismutase mutation, a paradox apparently unique in inherited disease) and recent developments, such as the role of tau pathology in ALS-dementia overlap syndromes, come too late to be included. But this volume’s strengths—its clarity, brevity, and breadth of vision—are to be applauded. Where else would one find tidy one paragraph descriptions of the basis of magnetic resonance spectroscopy, or the difference between an odds ratio and a relative risk? I have already lent out my copy of this handy little one volume summation and recommend it as a launchpad for readers’ further investigations into ALS/MND.