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Duchenne muscular dystrophy. By Alan E. H. Emery Oxford Oxford University Press 1987 315 pp illustrated $55

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Duchenne Muscular Dystrophy
By Alan E . H . Emery
Oxford, Oxford University Press. 1987
315 pp, illustrated. $53.00
The gene that is defective in Duchenne muscular dystrophy
was isolated and its product, dystrophin, was identified in
1987.The impact of this achievement is only beginning to be
felt, but clearly the knowledge we now have of the primary
biochemical abnormality will change the way we define the
disease and understand its pathogenesis. Deoxyribonucleic
acid probes with binding sites within and around the gene
have markedly improved our ability to provide prenatal diagnosis and carrier detection, and the probes we use today are
better for this purpose than those that were available as recently as one year ago.
Progress in Duchenne muscular dystrophy research has
been so rapid that any attempt to summarize the state of
affairs is at risk of soon becoming dated. Dr Emery’s monograph provides a good historical background and review of
the literature to 1986, but it does not include the new perspective gained as a result of isolation of the defective gene.
We might have seen a similar result in a monograph about
myasthenia gravis written before the identification of antiacetylcholine receptor antibodies 15 years ago. In addition,
the editing of the book is not up to the standards expected
from such an esteemed publisher. For example, the first sentence of the Introduction, “Duchenne muscular dystrophy is
the second most common genetic disorder in Man,” is incorrect. (There are many more common genetic diseases;
Duchenne muscular dystrophy is probably the second most
common lethal genetic disorder after cystic fibrosis.) Perhaps
once the dust has settled from the recent flurry of activity in
Duchenne muscular dystrophy research, Professor Emery or
someone else with his stature and experience will again attempt to summarize our knowledge of this disease.
special interest. Of note, the chapters on the neuropathology
of acquired immunodeficiency syndrome (AIDS) and the
neuroophthalmological problems in AIDS are particularly
elegant in that the authors have chosen excellent photographs and photomicrographs to illustrate their points and
the publishers have provided careful reproductions.
There were probably time pressures in the assembly of
this volume, which may account for some of the editing
problems noted by this reviewer. For example, the chapter
on the biology of human immunodeficiency virus (HIV) is
left to the end of the book, when it more clearly belongs at
the beginning in place of the rather dry chapter on
epidemiology. Many of the chapters are repetitious, presenting similar information from different points of view.
Specifically, the chapter on central nervous system dysfunction in AIDS is essentially a summary of chapters on the
specific abnormalities associated with AIDS (“Primary Infection”; ‘The AIDS Dementia Complex”; “Opportunistic Viral
Infections”; “Non-viral Infections”; “CNS Neoplasms”). All
of these chapters include a great deal of neuropathology,
which is still given its own chapter. The editors should have
included a comprehensive section on neuropsychiatric testing, an area that is likely to become increasingly important
in H I V infections, because many neurologists are complete
neophytes concerning this aspect of diagnosis. Better treatment of the association of human T-lymphotropic virus type
I with neurological disease would have been useful as well.
The book is a good investment for any neurologist or
neurosurgeon practicing in a metropolitan area. It is already
clear that the pathogenesis of the diseases caused by HIV,
and specifically the central nervous system infection, is much
more complex than that of any other viral illness of the
nervous system because it involves many cell types, latency,
and possibly toxic products. This book represents the initial
descriptive analysis of the problem, and it is not likely to be
superseded by rapid advances in the near future.
Francisco Gonzalez-Scarano. M D
Philadelghia, PA
Kenneth H . Fischbeck, M D
Philadebhia. P A
AIDS a n d t h e Nervous System
Edited by Mark L. Rorenblum, Robert M. Levy,
and Dale E . Bredesen
New York, Razm, 1938
424 pp, illustrated, $65.00
Books Received
The editors of AIDS and the Nervous System undertook a
survey of what is a tiineiy topic, and the book nicely complements what is available in the literature in assorted journals
and in the recently published supplement to the Annals of
Neurology. The chapters are a mix of standard “clinical textbook format,” in which personal observations by the authors
are interspersed with previously published information and
formally tabulated data, and of more formal presentations.
This is very useful because it is unlikely that many neurologists or neurosurgeons can keep up with the voluminous
literature in this rapidly expanding area unless they have a
Handbook of Clinical Neuroendocrinology
Edited by Charles B. Nemeroffand Peter T . Loosen
New York, The GuilfordPress, 1987
502 pp, illustrated, $60.00
Neuromuscular Diseases
By Jaap Bethlem and Charlotte E. Knobbout
New York, Oxfird, 1987
158 pp, illustrated, $19.95
Current Problems i n Neurology
4:Advances in
Headache Research
Edited by F . Cllfford Rose
London, John Libbey, 1987
280 pp, illustrated
Copyright 0 1988 by the American Neurological Association
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emery, illustrated, university, duchenne, 315, muscular, 1987, pres, oxford, alan, dystrophy
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