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Duchenne muscular dystrophy ed 2. Edited Alan E. H. Emery Oxford University 1993 392 pp illustrated $75

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rosurgeons. The pathology, natural history, and management
of each type of primary brain tumor is reviewed. The coverage of pediatric brain tumors is especially strong, and the
literature review is up to date. Chapters addressing the basic
biology of brain tumors, including genetics and immunology,
are too superficial to be useful. Nevertheless, this text comes
closer to being a “comprehensive text” of clinical aspects of
primary brain tumors than any other book currently available. Its strengths nicely complement those of another recent
text of neurooncology (Twijnstra A, Keyser A, Ongerboer
de Vissar BW, eds. Amsterdam: Elsevier, 1993). Neurooncology. Primary tumors and neurological complications of
cancer. Together, these two volumes provide a comprehensive survey of current neurooncology.
Stephen M . Sagar, M D
San Francisco. C A
HIV, AIDS, and the Brain. Research Publications:
Association for Research i n Nervous and Mental
Disease, Vol 72
Edited by Richard W . Pritr and Samuel W . Perry III
New York, Raven. 1994
3 34 pp. illustrated, $99.00
HIV, Aids and the Brain, volume 7 2 in the Association for
Research in Nervous and Mental Disease series, focuses on
primary human immunodeficiency virus (HIV)-associated
brain disease, or AIDS dementia complex (ADC). In an area
where hypotheses outnumber hard facts, Richard Price and
Samuel Perry have done an extraordinary job selecting essays
that balance rigorous scientific observation with intelligent
The introductory chapter by Price is a masterful review of
what is and is not known regarding the pathogenesis of AIDS
dementia. The currently favored hypothesis of cytokinedirected cytotoxicity is discussed with both clarity and appropriate caution. The clinicopathologic correlations claimed for
ADC are oversimplified; nonetheless, a model of ADC as a
disease of cytokine dysregulation is largely successful in offering testable hypotheses and therapeutic approaches.
Chapters 2 through 4 review the genetics of macrophage
tropism of HIV type 1, the neurotoxic effects of cytokines
implicated in ADC, and a general review (of limited usefulness) of cytotoxic T lymphocytes. Chapters 5 through 11
summarize much of the past decade of neuroAIDS research
and are the heart of the volume. These chapters, authored
by neuroAIDS pioneers such as Masliah, Wiley, and Sharer,
cover microglial infection, selectively vulnerable neuronal
populations, dendritic changes, and the nonhuman primate
model of ADC. With regard to new data, the strongest chapter, ironically, is not on “AIDS and the brain,” but on the
peripheral nerve in AIDS. Written by John Griffin and colleagues at Johns Hopkins, it provides new insight into the
commonly disabling distal neuropathy of AIDS, and is an
example of pathologic formulations that share both scrupulous clinical observation and rigorous basic science. Despite
reference to cytokine dysregulation in nearly every chapter,
it is here we find the most specific and clearly stated hypothe-
120 Annals of Neurology
Vol 36 N o 1 July 1994
ses. Poor copyediting mars this otherwise exciting contribution.
Stuart Lipton’s chapter on calcium dysregulation and
NMDA receptor-mediated neurotoxicity in HJV-associated
brain injury suggests potential pharmacologic interventions;
however, the chapter is weakened by lack of justification
for human clinial trials based on animal cell culture models
(rodedchick) for a uniquely human brain disease.
Neurobehavioral aspects of AIDS are often overlooked in
the neurologic literature; here, the epidemiologic and clinical
aspects of depression and other disorders of mood are well
represented in contributions by Perry and Treisman. Two
important new studies from Johns Hopkins are summarized
that demonstrate excellent treatment efficacy in HIVassociated psychiatric disorders.
With 40,000 new cases of ADC projected this year, clinicians will find the chapter on AIDS dementia (McArthur and
colleagues) invaluable. Epidemiologic, clinical, laboratory,
and pathologic features of ADC are reviewed. The section on
antiretroviral therapy balances the slim evidence for (limited)
efficacy in ADC with a cautious optimism. Practical aspects
of neuropsychiatric evaluation of ADC, reviewed by Sidtis,
complete this important section. Finally, R. T. Johnson summarizes the proceedings in an elegantly written essay that
places AIDS in its historical perspective and outlines the
questions we must address if we are to alter the course of
the pandemic.
Notably missing is a discussion of the influence of possible
cofactors, such as cytomegalovirus, in the development of
acquired immunodeficiency syndrome (AIDS) neurologic
disease. Overall, however, this volume is a state-of-art synthesis of neuroAIDS research, with well-chosen references and
a thorough index. It belongs on the shelves of neurologists,
neuroimmunologists, and AIDS basic scientists, as well as
primary care clinicians treating AIDS patients. As the field
of neuroAIDS evolves, I suspect these essays will remain
unsurpassed as a basis for organizing new data and insight.
Dawn MGuire, MD
San Francisco. C A
Duchenne Muscular Dystrophy, Ed 2
By Alan E. H . Emevy
Oxford, Oxford Unibersity. 1993
392 pp. illustrated, $75.00
Research in the past several years has given us a whole new
way of looking at Duchenne muscular dystrophy. In essence,
the disease has been redefined in terms of its primary biochemical defect, deficiency of the muscle protein dystrophin.
Thus, a new edition of Dr Emery’s monograph comes at an
opportune time.
There are problems with this book, however. For example,
the first sentence has an error left uncorrected from the first
edition. Duchenne dystrophy is not “the second most common genetic disorder in humans” (what about color blindness, colon cancer, Alzheimer’s disease?); it may be the second most common lethal genetic disorder of childhood after
cystic fibrosis. An error at the outset makes one wonder what
other inaccuracies might be hidden in the text. Although
much new information has been added to this edition, the
book has not quite incorporated the shift in paradigm that
came with discovery of the disease gene. Thus, a section on
D N A analysis has been added at the end of a chapter on
diagnosis, after a relatively detailed discussion of electromyography and muscle biopsy; D N A testing is now accurate,
efficient, and available enough to be featured more prominently. Also, the book still treats Duchenne and Becker’s
dystrophies as separate entities, rather than as parts of a more
or less continuous spectrum of “dystrophinopathy.” Furthermore, one wonders how much of the description of the range
of clinical manifestations of Duchenne dystrophy is based on
patients without confirmed dystrophin deficiency. Finally, the
recent evidence for efficacy of steroid treatment is given
short shrift (a paragraph and a table); it would help to have
more guidance through the literature on this aspect of management.
These shortcomings aside, the compendium of literature
references and discussion of the author’s extensive personal
experience make this book a useful resource; it is the most
extensive review now available on Duchenne muscular dystrophy.
Kenneth H . Fischbeck, M D
Philadelphia, PA
Basic Neurochemistry: Molecular, Cellular and
Medical Aspects, Ed 5
Edited by George J . Siegel, Bernard W. Agranoff;
R. Wayne Albers, and Perry B. Molinoff
New York, Raven Press, 1994
I080 pp, illustrated, $84.50
The first article in the inaugural issue of the Annals (published in 1977) was a review by Donald Tower, then director
of the NINCDS, entitled “Neurochemistry-One Hundred
Years, 1875-1775.” The state of knowledge in cellular neurophysiology was illustrated by a diagram of the action potential and its decomposition into sodium and potassium currents. Discussion of synaptic transmission was restricted
largely to acetylcholine and catecholamines in the peripheral
nervous system. The forerunner of present-day molecular
biology was a description of the biochemical defects in phenylketonuria and storage diseases. The author quoted Thudichum (1829-1701), father of modern neurochemistry, who
expressed the hope that “by the aid of chemistry many derangements of the brain and mind, which are at present obscure, will become accurately definable and amenable to precise treatment.”
The fifth edition of Basic Neurochemisty, the standard in
its field for many years, offers striking evidence of the extent
to which neurologic disease, “the object of anxious empiricism’’ to Thudichum, has become in his words an arena “for
the proud exercise of exact sciences.” That those sciences
have lost their distinct boundaries is also apparenr from the
wide array of areas-anatomy, physiology, molecular biology, pharmacology-to which neurochemistry here stakes a
claim. Basic Neurochemisty, directed at medical and graduate
students and postdoctoral fellows, is comprehensive, up to
date, and profusely and attractively illustrated. Eighty authors
have contributed 50 chapters, which are organized into sections on neural membranes, synaptic function, molecular
neurobiology ,cellular neurochemistry, medical neurochemistry, and behavioral neurochemistry. A table of contents is
given at the beginning of each chapter, and chapter subsections are introduced by declarative headings of the sort used
in Kandel, Schwartz, and Jessell’s Principles of Neural Science.
The chapter on “Electrical Excitability and Ion Channels”
contains an exceptionally lucid discussion of the membrane
potential and outstanding diagrams showing structural and
pharmacological features of the sodium channel but should
have been allotted additional space for more detailed coverage of calcium channels. The chapter on “Excitatory Amino
Acid Transmitters” is also outstanding. The diagrams chosen
to illustrate ligand binding sites and effector mechanisms associated with each of the major glut&ate receptor subtypes,
as well as structural interrelationships among receptor subunirs, are far superior in their clarity to most figures of this
sort. Glutamate receptor heterogeneity, including the role of
splice variants and regional differences in the expression of
receptor subunits, is considered in a thorough and timely
fashion. Three chapters devoted to molecular neurobiology
are written in a hlghly accessible style that manages to avoid
overwhelming the reader with jargon, with technical terms
defined clearly and gradually, as the discussion warrants.
These chapters are superb but might benefit from a few more
illustrations. The chapters dealing with neurochemistry of
clinical disorders, on the other hand, are uneven, and are
informed to differing extents by clinical experience. They are
most successful-as in the chapters on disorders of muscle
and on Alzheimer’s disease-where the subject lends itself
to coherent exposition of the kind used for most of the preclinical topics, and less so when the subject of the chapter
does not permit the development of a unifying theme.
This is an excellent book to use in neuroscience survey
courses for pre- or postdoctoral students, including neurology residents. Compared with Principles of Neuraj Science (referred to above), which is directed at a similar audience, Basic
Neurochemisty places more emphasis on biochemistry and
pharmacology and less on anatomy and physiology.
David A. Greenberg, M D , PhD
San Francisco. CA
Sleep Disorders Medicine: Basic Science, Technical
Considerations, and Clinical Aspects
Edited by Sudhanszr Chokrowerty, MD
Stoneham, M A . Buttemoorth-Heinemann, 1794
504 pp, illustrated, $125.00
Until this century, sleep had been considered a twilight state
on a continuum between wakefulness and death (in Greek
mythology, among the children of the goddess Night were
the brothers Sleep and Death). We now know that sleep is
not the product of an inert brain, that the operating characteristics of physiological systems change dramatically from
wakefulness to sleep, and that many systems that function
normally during the day can decompensate during the night.
The multidisciplinary specialty of sleep disorders medicine
has emerged to care for the estimated one-third of all adult
Annals of Neurology
Vol 36 No 1 July 1794
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1993, emery, illustrated, university, duchenne, 392, edited, muscular, oxford, alan, dystrophy
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