Escourolle & Poirier Manual of Basic Neuropathology

405 Pages·2004·44.172 MB·English

by  Françoise Gray, MD, PhD, Umberto De Girolami, MD, and Jacques Poirier, MD, PhD (Eds.)

Butterworth–Heinemann
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ESCOUROLLE & POIRIER MANUALOF BASIC NEUROPATHOLOGY
ISBN: 0-7506-7405-9
Copyright 2004, Elsevier Inc. All rights reserved.
Previous editions copyright 1990, 1978, 1973
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Editor: Susan F. Pioli
Editorial Assistant: Joan Ryan
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Contributors
Homa Adle-Biassette, MD, PhD
Associate Professor of Pathology, Faculté de Médecine Paris VII (Bichat–Claude
Bernard), Université Denis Diderot; Praticien Hospitalier, Service d’Anatomie
Pathologique, Hôpital Bichat–Claude Bernard, Paris, France
Douglas C. Anthony, MD, PhD
Professor and Chair, Department of Pathology and Anatomical Sciences,
University of Missouri, Columbia, Missouri
Umberto De Girolami, MD
Professor of Pathology, Harvard Medical School; Director, Neuropathology
Divisions, Departments of Pathology, Brigham and Women’s Hospital; Children’s
Hospital Boston, Boston, Massachusetts
Charles Duyckaerts, MD, PhD
Professor of Pathology, Faculté de Médecine Paris VI (Pitié-Salpêtrière),
Université Pierre et Marie Curie; Praticien Hospitalier, Laboratoire de
Neuropathologie Raymond Escourolle, Hôpital de la Salpêtrière, Paris, France
Férechté Encha-Razavi, MD
Associate Professor of Histology, Embryology, and Cytogenetics, Faculté de
Médecine Paris V(Necker-Enfants Malades); Université René Descartes; Head,
Unit of Fetal Genetic Development, Department of Genetics, Hôpital Necker-
Enfants Malades, Paris, France
Margaret Esiri, DM, FRCPath
Professor of Neuropathology, Department of Clinical Neurology, Oxford
University; Honorary Consultant Neuropathologist, Oxford Radcliffe NHS Trust,
Oxford, United Kingdom
Rebecca D. Folkerth, MD
Assistant Professor of Pathology, Harvard Medical School; Associate in
Pathology, Brigham and Women’s Hospital; Consultant Neuropathologist,
Children’s Hospital Boston, Boston, Massachusetts
v
vi CONTRIBUTORS
Matthew P. Frosch, MD, PhD
Assistant Professor of Pathology, Harvard Medical School; Assistant Pathologist,
C.S. Kubik Laboratory for Neuropathology, Massachusetts General Hospital,
Boston, Massachusetts
Jennian F. Geddes, MB, BS, FRCPath
Reader in Clinical Neuropathology, Department of Histopathology and Morbid
Anatomy, Queen Mary—University of London; Honorary Consultant
Neuropathologist, Royal London Hospital, London, United Kingdom
Romain K. Gherardi, MD
Professor of Histology, Faculté de Médecine Paris XII (Créteil); Université
Paris–Val de Marne; Director, INSERM, Institute of Molecular Medicine; Head,
Service d’Histologie and Groupe Nerf-Muscle, Department of Pathology, Hôpital
Henri Mondor, Creteil, France
Bernardino Ghetti, MD
Distinguished Professor and Director of Neuropathology, Department of
Pathology and Laboratory Medicine, Indiana University School of Medicine,
Indianapolis, Indiana
Hans H. Goebel, MD
Professor of Neuropathology, Johannes Gutenberg University Medical Center,
Mainz, Germany
David I. Graham, MB BCh, PhD, FRCPath
Professor of Neuropathology, University of Glasgow; Honorary Consultant
Neuropathologist, South Glasgow University Hospitals NHS Trust, Glasgow,
Scotland, United Kingdom
Françoise Gray, MD, PhD
Professor of Pathology, Faculté de Médecine Paris VII (Lariboisière–St. Louis),
Université René Descartes; Director, Service d’Anatomie Pathologique, Hôpital
Lariboisière, Paris, France
Brian Harding, D.Phil, BM BCh, FRCPath
Senior Lecturer, Neurosciences Unit, Institute of Child Health; Consultant
Neuropathologist, Department of Histopathology, Great Ormond Street Hospital
for Children, London, United Kingdom
Jean-Jacques Hauw, MD
Professor of Pathology, Faculté de Médecine Paris VI (Pitié-Salpêtrière),
Université Pierre et Marie Curie; Director, Laboratoire de Neuropathologie
Raymond Escourolle, Hôpital de la Salpêtrière, Paris, France
E. Tessa Hedley-Whyte, MD
Professor of Pathology, Harvard Medical School; Neuropathologist, Massachusetts
General Hospital, Boston, Massachusetts
CONTRIBUTORS vii
William F. Hickey, MD
The Hampers Professor and Senior Associate Dean of Academic Affairs,
Department of Pathology, Dartmouth Medical School, Hanover; Attending
Pathologist, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
James W. Ironside, MBChB, FRCPath, FRCPE
Professor of Clinical Neuropathology, University of Edinburgh; Honorary
Consultant Neuropathologist, Lothian University Hospitals Trust, Edinburgh,
Scotland, United Kingdom
Hart G. W. Lidov, MD, PhD
Associate Professor of Pathology and Neurology, Harvard Medical School; Staff
Neuropathologist, Children’s Hospital Boston and Brigham and Women’s
Hospital, Boston, Massachusetts
David N. Louis, MD
Professor of Pathology, Harvard Medical School; Associate Chief of Pathology,
Massachusetts General Hospital, Boston, Massachusetts
James Lowe, B.Med.Sci., BM BS, DM, FRCPath
Professor of Neuropathology, School of Clinical Laboratory Sciences, University
of Nottingham Medical School; Clinical Head of Service, Department of
Pathology, Queen’s Medical Centre, Nottingham, United Kingdom
Jacqueline Mikol, MD
Professor of Pathology, Faculté de Médecine Paris VII (Lariboisière-St. Louis),
Université René Descartes; Director, Service d’Anatomie Pathologique, Hôpital
Lariboisière, Paris, France
Vânia Nosé, MD, PhD
Associate Professor of Pathology, Harvard Medical School; Associate Director of
Surgical Pathology, Brigham and Women’s Hospital; Consultant Pathologist,
Children’s Hospital Boston, Boston, Massachusetts
Jacques Poirier, MD, PhD
Professor of Histology, Faculté de Médecine Paris VI (Pitié-Salpêtrière),
Université Pierre et Marie Curie, Praticien Hospitalier, Laboratorire de
Neuropathologie Raymond Escourolle, Hôpital de la Salpêtrière, Paris, France
Francesco Scaravilli, MD, PhD, DSc, FRCPath
Professor of Neuropathology, Department of Molecular Neuroscience, Institute of
Neurology, University College of London; Honorary Consultant Neuropathologist,
University College Hospital NHS Trust, London, United Kingdom
Sydney S. Schochet, Jr., MD
Professor of Pathology, Neurology and Neurosurgery, Robert C. Byrd Health
Sciences Center of West Virginia University; Neuropathologist, Ruby Memorial
Hospital, Morgantown, West Virginia
viii CONTRIBUTORS
Danielle Seilhean, MD, PhD
Associate Professor of Pathology, Faculté de Médecine Paris VI (Pitié-
Salpêtrière), Université Pierre et Marie Curie; Praticien Hospitalier, Laboratoire de
Neuropathologie Raymond Escourolle, Hôpital de la Salpêtrière, Paris, France
Leroy R. Sharer, MD
Professor of Pathology (Neuropathology), Department of Pathology and
Laboratory Medicine, New Jersey Medical School; Attending Pathologist,
Department of Pathology and Laboratory Medicine, University Hospital, Newark,
New Jersey
Thomas W. Smith, MD
Professor of Pathology, University of Massachusetts Medical School; Director of
Neuropathology and Diagnostic Electron Microscopy, Department of Pathology,
University of Massachusetts Memorial Medical Center, Worcester, Massachusetts
Raymond A. Sobel, MD
Associate Professor of Pathology, Stanford University School of Medicine; Staff
Neuropathologist, Laboratory Service, Veterans Affairs Health Care System, Palo
Alto, California
Maria Thom, MB BS, BSc, FRCPath
Senior Lecturer in Neuropathology, Department of Clinical and Experimental
Epilepsy, Institute of Neurology, University College London; Honorary Consultant
in Neuropathology, National Hospital for Neurology and Neurosurgery, London,
United Kingdom
Jean-Michel Vallat, MD
Professor of Neurology, Faculté de Médecine de Limoges; Chairman, Department
of Neurology, Hôpital Universitaire Dupuytren, Limoges, France
Harry V. Vinters, MD, FRCP(C), FCAP, FFPath (RCPI)
Professor of Pathology and Laboratory Medicine and Neurology, David Geffen
School of Medicine at University of California, Los Angeles; Chief, Laboratory of
Neuropathology, Department of Pathology and Laboratory Medicine, University
of California, Los Angeles Medical Center, Los Angeles, California
Preface
The first French editions of the Manuel de Neuropathologie, published in 1971 and
1977, were conceived, written, and edited by Raymond Escourolle and Jacques
Poirier. After the death of Raymond Escourolle in 1984, Françoise Gray joined
Jacques Poirier for the third edition; Jean-Jacques Hauw and Romain Gherardi con-
tributed to selected chapters as well. The first three editions reached the English-
speaking public thanks to the friendship and translating ability of Lucien Rubinstein.
Neuropathology has evolved to such an extraordinary extent since 1993, the date
of publication of the third English edition, that it is no longer possible for just two
or three individuals to produce a high-quality, updated new edition. For this fourth
edition, therefore, Umberto De Girolami has joined us as a co-editor, and we have
sought the collaborative efforts of multiple experts throughout the world to write the
English language text.
The compilation of a basic work designed to familiarize physicians-in-training
with such a highly specialized discipline as neuropathology entails two opposing
risks: in attempting to compress the maximum amount of information within the
minimum space, the text is liable to become unintelligible to beginners; if, on
the contrary, one tries to maintain too elementary a level, the danger is that only the
obvious will be stated. In presenting to the noninitiated reader neuropathological
information that some may find too simple, we have opted for the hazard of the
second pitfall. Names have purposefully been avoided, with the exception of those
traditionally associated with a particular disease. Discussions on the interpretation
of lesions have been limited to relatively simple observations. Clinico-pathologic
correlations have been reduced to essentials. Rare diseases have been glossed over
or mentioned only for completeness.Despite their interest, historical data have been
omitted. We have taken for granted that the reader has some familiarity with basic
concepts of neuroanatomy, neurohistology, and general principles of anatomic
pathology.
This manual is indeed designed not for neuropathologists, or even trained neu-
rologists, but for students and physicians-in-training, particularly those interested in
anatomic pathology, neurology, neuroradiology and neurosurgery, who have recently
joined a neuropathology laboratory hoping to become familiar with the essential
topics of the pathology of the nervous system. Those who do not plan a career in
ix
x PREFACE
neurology or neuropathology need not extend their studies much beyond the mate-
rial covered herein; the others should regard this manual simply as an introduction
to the textbooks and major references listed in the bibliography at the end of this
text.
We hope that the readers of the fourth edition of this book will grant it as favor-
able a reception as that happily enjoyed by earlier editions.
Acknowledgments
The editors and authors acknowledge with pleasure Ms. Nancy Lombardi for supe-
rior editorial assistance; Ms. Susan Pioli, editor representing the Butterworth-
Heinemann Company, for making this work possible; and Ms. Diane Brown and
Ms. Linda Palcsey for excellent support in helping us prepare a final draft of this
manuscript.
Jacques Poirier, MD, PhD
Francoise Gray, MD, PhD
Umberto De Girolami, MD
x PREFACE
neurology or neuropathology need not extend their studies much beyond the mate-
rial covered herein; the others should regard this manual simply as an introduction
to the textbooks and major references listed in the bibliography at the end of this
text.
We hope that the readers of the fourth edition of this book will grant it as favor-
able a reception as that happily enjoyed by earlier editions.
Acknowledgments
The editors and authors acknowledge with pleasure Ms. Nancy Lombardi for supe-
rior editorial assistance; Ms. Susan Pioli, editor representing the Butterworth-
Heinemann Company, for making this work possible; and Ms. Diane Brown and
Ms. Linda Palcsey for excellent support in helping us prepare a final draft of this
manuscript.
Jacques Poirier, MD, PhD
Francoise Gray, MD, PhD
Umberto De Girolami, MD
Foreword
When I was learning neurology in the mid-1970s, neuroanatomy and neuropathol-
ogy were the basic sciences of the field. Nearly a third of my formal neurological
training was devoted to a serious study of neuropathology. I went to the autopsy
room, removed the brain and spinal cord, fixed the tissue, and later examined it
grossly and microscopically, ultimately generating a clinical-pathologic correlation.
Aformal report of the findings was written, supervised by a senior neuropatholo-
gist. These 50 personally analyzed cases formed the foundation of my entire career
in clinical neurology. Through these cases I learned the anatomy of the nervous
system, its reaction to disease, and the rigorous methods necessary to hone my
clinical approach using the gold standard of neuropathology.
At that point in my nascent career I had no knowledge of neuropathology except
for the very simple principles learned in my medical school general pathology
course. Thank goodness for the then newly available Manual of Basic Neu-
ropathologyby Raymond Escourolle and Jacques Poirier. The original French mono-
graph entitled Manuel Élémentaire de Neuropathologiehad just been translated into
English by Lucien J. Rubinstein and published in 1973. It was my bible and savior.
With it I was able to learn approaches and techniques to analyze my cases and unlock
the enormous trove of invaluable knowledge that the cases held. To this day I fre-
quently refer back to the little blue paperback book that had such an enormous
impact on the way in which I approach neurological problems.
In many ways neurology and neurological training have fundamentally changed.
The basic sciences of neurology are now molecular genetics and cell biology.
Current trainees must learn a great deal about structural and functional imaging of
the nervous system as well as the details of a whole array of subspecialty subjects
such as movement disorders, cancer neurology, stroke, demyelinating diseases,
epilepsy, neuromuscular diseases, neuro-ophthalmology, cognitive and behavioral
neurology, and pain. Thankfully, therapeutics is now a real part of neurology, so
modern neurologists in training are faced with a dizzying array of complex treat-
ments for nearly every category of neurologic diseases.
As the complexity of clinical neurology has increased, the subject of neu-
ropathology has become progressively neglected. The opportunity to spend nearly
a year on the study of neuroanatomy and neuropathology is unavailable; yet the
lessons that can be learned are even more important than they were in the past. A
xi
xii FOREWORD
rigorous approach to neurologic disease, with the gold standard of modern neu-
ropathology, is absolutely required for the neurologist of the future.
In this context, Jacques Poirier, one of the original authors, is joined on the edi-
torial team by Françoise Gray and Umberto De Girolami, together with 30 distin-
guished neuropathologists from around the world to remodel, update, and expand
Manual of Basic Neuropathologyfor the modern era. The reader will find the fourth
edition to be just as clearly written and accessible as the earlier ones, but it now con-
tains the relevant principles and advances in the fields of molecular genetics and cell
biology. The old and new neuropathology are skillfully blended to enlighten the
student of nervous system diseases with the basic principles and processes that
underlie all of the major categories of illness.
The basic principles of pathology of the central nervous system lead the book,
with a survey of modern neuropathological methods at the end. New chapters on
tumors, trauma, vascular diseases, and infections follow. A new, beautifully clear
chapter on the important prion diseases is followed by thoroughly updated chapters
on demyelinating diseases and degenerative disorders. Three new chapters on
acquired metabolic disorders, hereditary metabolic disorders, and congenital mal-
formations and perinatal diseases replace the single small chapter in the original
work on toxic, deficiency, and metabolic diseases.
Two new separate chapters on nerve and muscle neuropathology replace the
single old chapter, reflecting the major changes that have occurred in this area of
peripheral nervous system neuropathology. Finally, a wholly new chapter on the
pituitary gland completes the book. The authors of the individual chapters are experts
in their fields and write clearly and concisely.
I found the new Manual of Basic Neuropathologya marvelous and welcome addi-
tion to neurologic literature. I look forward to referring to this new version for many
years to come. It will have an honored place on my library shelf, right next to the
original little blue book that gave me such a great start so many years ago.
Martin A. Samuels, MD