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Amyotrophic lateral sclerosis and other motor neuron diseases (Advances in neurology volume 56). Edited by Lewis P. Rowland. MD New York. Raven Press 1991 560 pp

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Amyotrophic Lateral Sclerosis a n d Other M o t o r
Neuron Diseases (Advances in Neurology, Volume 56)
Edited by Lezim P. Roubnd. M D
Neil York. Raien PveJs. 1991
560 pp4rfhrtratrd. $ 1 3 5
Onc should greet a collection of papers from a symposium
with skepticism. Is this really “essential reading for all clinicians and investigators who are concerned with this disorder,”
as t h e book cover touts? For me, the answer was yes. But as
you look at your own library you may well ask yourself if
you actually need another review on ALS, especially since
several already o n your shelf may be edited by Rowland as
it is. Yet the motor neuron and its diseases is a burgeoning
area of research. and it is impossible to keep up one’s research in it or to be helpful and informative to one’s patients
by scanning the abstracts in the green journal or the blue
journal. Most of the advances in this field in the last decade
have come from molecular genetics, experimental immunology. and neurochemistry.
This book comprises 46 papers from an international,
MDA-sponsored meeting of about 200 invited investigators,
held in December 1989. A similar meeting occurred in 1981,
resulting in volume 36 of the same Adeances in Neurology
series by Raven Press. Only 13 of the authors are the same
as in the previous volume, and none of the chapters appear
to be tired rewrites. It is interesting that many of the topics
haven’t changed much from the ones in David Goldblatt’s
chapter on ALS in volume 1 7 of Advances in Neurology 14
years ago-aging, environmental factors, circulating neurotoxins, virus infection, immune disorder, and empirical therapies. However, this is a surprisingly good book with a wealth
of new material that will be useful for years to come. A
fourth to a half of the references date from 1988 to early
1030. Many truly important discoveries-the genetic loci for
familial ALS and X-linked spinobulbar amyotrophy, an autoimmune model of ALS, and the allelism of infantile and juvenile spinal muscular atrophy, all of which were recently published in the weekly scientific periodicals-are presaged in
this volume.
As you approach the decision to buy this book, you may
be drawn in by reading the great introductory essay by Rowland, written in his usual provocative and entertaining style.
Entitled, “Ten Central Themes in a Decade of ALS Research,” it was a bargain that contained 14. He begins with
helpful definitions to overcome the confused usage of terminology that pervades research on motor neuron diseases.
Nevertheless, some of the distinctions he makes will mystify
the average reader. Although he defines ALS as the combination of lower motor neuron signs and unequivocal upper
motor neuron signs, he views ALS as a large syndromic classi-
578 Annals of Neurology
Vol 31
No 5
May 1992
fication rather than a narrow, semiologically and electrophysiologically defined disease. This usage allows him to view
monomelic amyotrophy, ocular syndromes with motor neuron involvement, and lower motor neuron syndromes as
“ALS syndromes.” It permits him to construct an odd table
called “Heterogeneity of ALS-dementia; histopathology,”
which lists 17 diseases as diagnostically remote as Hallcvorden-Spatz disease as being “syndromes of ALS and dt:mentia.” Rowland is at his best when providing analysis of
the links between lymphoma or paraproteinemia and motor
neuron diseases and when arguing strongly why the “persistent viral” hypothesis should persist.
If, however, you begin to look at this book as I did, by
thumbing through the pages, and happen onto page 129, an
idiosyncratic, full-page graphic composed of 5 0 arrows leading only to ellipses, you may be seized by an uncontrollable
urge to put the book down. If you scan the pages and find a
grammatically correct but empty sentence like this one,
“Thus an . . . abnormality could adversely affect MNs by a
deficiency or toxic mechanism, involving a substance presently known . . . or unknown that might ( o r not) involve
R N A metabolism,” you may be overwhelmed with the neeA
to resist this book and look for real content elsewhere. Fortunately, there were many standout authors, and these most
often presented new material. Among them were Leber and
Sanes (lineage of motor neurons), Hausmanowa-Petrusewicz
(clinical genetics of SMAs), Brzustowicz et a1 (genetic locus
of childhood SMA), Neote et a1 (P-hexosaminidase deficiencies), Fischbeck et a1 (gene for X-linked spinobulbar amyotrophy), Lavine et a1 (Guamanian ALS is not disappearing).
Engelhardt et al (experimental autoimmune motor neuron
disease), and Appel et a1 (autoimmunity in human ALS).
Some of the chapters bring together material hard to find
elsewhere (Bradley et a1 postradiation motor neuron syndromes) or provide a useful short course (Antel and Minuk,
immunogenetics and motor neuron disease). As expected for
a symposium, the chapters are spotty. Overall, the reader has
an easy guide to what’s good to read, since there is an apparent inverse correlation between excellence and chaptcr
length in this book (r = 0.36, p < 0.02).
In short, don’t avoid the book because it was occasioned
by a symposium; buy it as a complete reference work or buy
it to be informed for your patients, but buy this book. I t has
more to offer than any of its recent competitors such as
Hudson’s ALS: Concepts in Pathogenesi, and Etiology, o r Rose’s
Progress in Clinical Neurologic Trials. Voltimr I :Amyotrophic
LteralSclerosis. Every medical library in the world and many
neurologists should have this one.
Ralph W. Kuncl, M U , PhD
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motor, neurology, disease, amyotrophic, new, 1991, volume, york, rowland, advanced, lateral, edited, rave, neurons, sclerosis, pres, lewis, 560
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