Neurology of the Newborn, 5e

1106 Pages·2008·42.075 MB·English

by  Joseph J. Volpe MD

To my wife,
Sara,
for her love and understanding,
without which this book would not be possible
http://avaxho.me/blogs/ChrisRedfield

Preface to the Fifth Edition
Thenearly30yearssincepublicationofthefirstedition points throughout the book. This edition contains
of thisbook havebeen aperiod ofextraordinary devel- approximately 550 tables. Many of these are new,
opment in the discipline of the neurology of the many replace earlier tables, and many of the original
newborn. In 1981, at the time of publication of the tables contain new information. As with tables,
first edition, there was a sense of a new frontier to be I value greatly the illustrative power of figures, in the
pioneered.Currentlyarticlesonneonatalneurologyare form of flow diagrams, experimental findings, clinical
abundantinthemajorjournalsinpediatrics,childneu- and pathological specimens, and all types of brain im-
rology, and related disciplines. Current-day annual aging.Thiseditioncontainsapproximately665figures,
meetings of scientific societies of pediatrics and child manyofwhicharenew.Moreover,manyoftheoriginal
neurology are dominated by research and clinical pre- figures have been replaced with better examples of the
sentationsontheneurologyofthenewborn.Thus,the relevant findings.
field now has matured fully into a discipline in its own The extraordinary progress in the study of the neu-
right. rology ofthe newborn isreflected inpart by the explo-
The fifth edition of this book has been completely sion of new literature in the relevant disciplines. This
updated and extensively revised. All of the changes edition contains approximately 12,500 references,
have been incorporated into an organization that is nearly 3000 more than in the previous edition. The
identical to that of the previous editions. Thus, the enormous increase in the relevant literature between
four initial chapters establish the foundation of the the last and current edition is a tangible reflection of
remainder of the book. These four chapters deal with the intense interest in this extraordinarily important
the development of the nervous system, the disorders field. Every chapter contains many new citations as
caused by anomalous development, the clinical neuro- part of the updating of the entire book.
logical examination, and the specialized techniques in I have been extremely fortunate to have the help of
the neurological evaluation. The fifth chapter, con- many very talented and dedicated people in the prep-
cerning neonatal seizures, serves as an effective bridge aration of this book. As she did for the previous two
between the initial chapters and the later, disease- editions, Irene Miller performed the simply incredible
focused chapters, because neonatal seizure is a key taskoftypingandretypingtheentirebook: text,tables,
manifestation of many of the neurological disorders legends, and references. She manipulated and renum-
dealt with later in the book. The next 19 chapters bered the 12,500 references with aplomb; to this day,
focusontheneurologicaldisorders,withastrongclin- I don’t understand how she did it so efficiently and
ical emphasis. However, as in the past, the lessons without losing her mind. Janine Zieg prepared many
learned from basic and clinical research are brought new flow diagrams and schematics and updated many
to the bedside in the discussions of the diseases. others with great skill and patience, particularly
This book is intended for a broad audience, that is, because I revised them incessantly. Sarah Andiman
fromthemosthighlyspecializedneonatalphysiciansto ably assisted in this endeavor. My young colleague,
thosewithamoregeneralperspective.Ihaveattempted Dr. Omar Khwaja, spent many hours at the computer
to generate a systematic, readable, and comprehensive helping a computer-naı¨ve author with illustrations; he
synthesis of the neurology of the newborn that will be contributed important images and helped restore the
of value to all individuals who care for the infant, both valueofsomeoriginals.Finally,asinpreviouseditions,
in the critical neonatal period and later. The clinical I acknowledge the support and patience at Elsevier of
discussions are buttressed by information generated Judy Fletcher, with whom I have worked for almost
from the most recent diagnostic methodologies, by 20 years since the third edition and who supervised
the results of promising new therapies, andby insights theoverallproject.EllenZanolledesignedthefetching
gained from basic research in such relevant disciplines cover and successfully convinced a stodgy author that
as neuroscience, genetics, and developmental biology. covers should be eye-catching. Lee Ann Draud
Attemptingtodoallthishasbeenstimulatingandchal- superbly led the production efforts. Not only was her
lenging,andIapologizeifIhaveoversimplifiedinsome Elsevier group so tolerant of my obsessive pursuit of
areas and displayed my ignorance in others. After perfection, but they allowed me to add new references
five editions I hope that these two problems are few. until the very end of 2007.
Previous readers will recognize that I place great
value on the liberal use of tables to synthesize major Joseph J. Volpe, MD
vii

Preface to the First Edition
The neurology of the newborn is a topic of major important clinical clues when we elicit a complete his-
importancebecauseofthepreeminenceofneurological tory and perform a careful physical examination. It is
disorders in neonatology today. The advent of modern this quality of discovery with simple techniques that
perinatal medicine, accompanied by striking improve- hasmadetheneurologyofthenewbornsostimulating
mentsinobstetricalandneonatalcare,haschangedthe forme,andIhopethatthisbookcanleadthereaderto
spectrum of neonatal disease drastically. Many pre- similar discoveries.
viously dreaded disorders such as respiratory disease With accomplishment of the essential first step of
have been controlled to a major degree. At the same definition of the clinical problem, we can turn in a
time, certain beneficial results of improved care, for rational way to the increasingly sophisticated means
example,markedlydecreasedmortalityratesforprema- of studying the infant’s deranged neural structure and
ture infants, have been accompanied by neurological function. Although my emphasis is, first, on the sim-
disorders that would not have had time to evolve in plest and least invasive techniques for providing us
past years. with the necessary information, we are in an era
This major importance of neonatal neurological when sophisticated and informative procedures such
disease has stimulated efforts by workers in many dis- as imaging the brain itself can be done in a safe and
ciplines to recognize, understand, treat, and ultimately effective way.
prevent such disease. This book is an attempt to bring The final process in our understanding the infant
together the knowledge gained from these efforts and with a neurological disorder requires an awareness of
to present my current understanding of the neurology a burgeoning corpus of information derived from stu-
of the newborn. Because of the diversity of knowledge diesinhumanandexperimentalpathology,physiology,
that I have attempted to bring to bear upon the prob- biochemistry, and related fields. Of necessity, often we
lems discussed in this book, I may have oversimplified must extrapolate toour newborn patient data obtained
in certain areas and displayed my own ignorance in fromanimals.Suchextrapolationmustalwaysbemade
others. Nevertheless, I have written the material in cautiously, and yet we cannot ignore the many lessons
the hope that it will be of value to all health profes- learnedfromthelaboratorythathaveprovedinvaluable
sionals involved in the care and follow-up of the new- in our understanding of neonatal neurological disease.
born infant with neurological disease. In this book, on the one hand, I attempt to synthesize
Theprimefocusofthediscussionsofneonatalneu- in a comprehensible manner relevant material from a
rological disease throughout this book is the clinical diversity of disciplines and, on the other hand, try very
evaluation of the infant, that is, what we can learn hard not to oversimplify what are clearly very complex
from observation of the setting and mode of presenta- issues.
tionofthediseaseandthedisturbancesofneurological I believe that the neurology of the newborn has
function apparent on careful examination. The theme come of age and, indeed, should be viewed as a disci-
that recurs most often is that careful clinical assess- plineinitsownright.Ihopethatinsomewaythisbook
ment, in the traditional sense, is the prerequisite and willcontributetoestablishingthatstatus.Mymostfer-
the essential foundation for understanding the neuro- venthopeisthatthisdisciplineexcitestheinterestsand
logical disorders of the newborn. The infant does not efforts of others concerned with the neonatal patient
advertise his or her neurological disorder with the and that, through concerted actions, the greatest pos-
drama that older children and adults exhibit, but with sible benefits accrue to the infant with neurological
patience and diligence we can discover a treasure of disease.
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