Table Of ContentEye Movement Disorders in
Clinical Practice
Shirley H. Wray, MD, PhD, FRCP
PROFESSOR OF NEUROLOGY
HARVARD MEDICAL SCHOOL
MASTER CLINICIAN
DEPARTMENT OF NEUROLOGY
MASSACHUSETTS GENERAL HOSPITAL
BOSTON, MASSACHUSETTS
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© Shirley H. Wray 2014
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Library of Congress Cataloging-in-Publication Data
Wray, Shirley H.
Eye movement disorders in clinical practice / Shirley H. Wray.
p. ; cm.
ISBN 978–0–19–992180–5 (alk. paper)—ISBN 978–0–19–992181–2
(alk. paper)—
ISBN 978–0–19–998149–6 (alk. paper)
I. Title.
[DNLM: 1. Ocular Motility Disorders—diagnosis—Case Reports. 2.
Ocular Motility Disorders—etiology—Case
Reports. 3. Cranial Nerve Diseases—complications—Case Reports.
4. Cranial Nerve Diseases—diagnosis—Case
Reports. 5. Diagnosis, Differential—Case Reports. 6.
Neuropsychological Tests—Case Reports. WW 410]
RE731
617.7′62—dc23
2013010302
The science of medicine is a rapidly changing field. As new research
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work have checked with sources believed to be reliable in their
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However, in light of the possibility of human error or changes in the
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1 3 5 7 9 8 6 4 2
Printed in the United States of America
on acid-free paper
For Anne
With All My Thanks
CONTENTS
Preface
1. How the Brain Moves the Eyes
2. The Eyelid and Its Signs
3. Ptosis and Neuromuscular Syndromes
4. The Extraocular Muscles and Diplopia
5. Cranial Nerves Three, Four, Six, and Their Syndromes
6. Horizontal Gaze and Syndromes of the Pons
7. Vertical Gaze and Syndromes of the Midbrain
8. Dizziness, Vertigo, and Syndromes of the Medulla
9. The Cerebellum and Its Syndromes
10. Oscillopsia, Nystagmus, Saccadic Oscillations, and
Intrusions
Index of Case Studies with Video Displays
Index
PREFACE
T
his is a very personal book. It reflects my experience as a
neurologist for more than 50 years. In the course of that time I knew
and learned from the great neurologists at Queen Square, London,
during my residency and fellowship in neurophysiology, and at the
Massachusetts General Hospital when I arrived there in 1967. Their
influence is part of this book and my specific indebtedness is to the
late Raymond D. Adams and Charles Miller Fisher.
Eye movement disorders are common in clinical practice and yet
they raise many difficult questions with respect to diagnosis,
prognosis and management. The approach I have taken in this book
is clinically oriented but the contents covers much of the relevant
literature, and include many aspects of eye movement disorders. I
realize that some of the views expressed, for example, my guides to
clinical points to remember, are personal preferences but they have
worked for me and I hope they may work for others. I have
attempted as well to clarify the complex terminology and methods of
ophthalmologists for neurologists and neurosurgeons, and to provide
a comprehensive physiologic framework to aid in diagnosis.
While written with neurologists and neurosurgeons specifically in
mind, I hope the students of other disciplines, among them
ophthalmology, neuro-otology, neuro-pediatrics, neurooncology and
internal medicine, will find my approach useful. Readers who would
like to view additional videos of a variety of other eye movement
disorders can access my website by accessing the NOVEL website
of the North American Neuro-Ophthalmology Society:
http://NOVEL.utah.edu or by accessing my collection at Harvard
Medical School Countway Library:
http://Repository.Countway.Harvard.edu/Wray.
I am very grateful to many colleagues who have made the writing
of this book possible. This especially applies to the late David Cogan
at the Massachusetts Eye and Ear Infirmary who strongly
encouraged me to film and video many unique cases for teaching
purposes and I am especially grateful to my patients who contributed
so much to my clinical experience and whose permission made
possible the case studies published in this book. The expert help of
Nancy Lombardo, Associate Director of Information Technology,
Spencer S. Eccles Health Sciences Library, University of Utah and of
Stephen Smith, Department of Surgery, Massachusetts General
Hospital made the DVD collection possible.
A number of people provided encouragement throughout this
project and I would like to express my gratitude to John Leigh who
critically reviewed the manuscript and David Zee who reviewed the
case videos with me and to Agnes Wong and Hal Blumenfeld for
their unfailing authorial generosity. Any errors that may remain are
my own. I am also particularly indebted to Anne Jardim for her
editing skill, helpful criticism and encouragement over the last
several years, and to my secretary, Fran Christie, for her unstinting
help in getting the manuscript ready for publication and who with
Karen Hoenig and Marylou Moar typed draft after draft after draft
until we were satisfied. My grateful thanks also go to members of the
Massachusetts General Hospital Photographic Department, Michelle
Rose and Paul Batista, for their remarkable professional competence
and to the wonderful creative team at the Cambridge Side Galleria
Apple Store for their support in creating the DVD. Thank you Tommy,
Dan, Tony, Brook, and Akira.
Shirley H. Wray
| 1 |
HOW THE BRAIN MOVES THE EYES
THE CEREBRAL CORTEX
The brain sees what the eyes look at. Multiple, well-delineated visual
areas analyze the visual scene, with each area having its own
retinotopic map of the visual field. The visual areas act
simultaneously to analyze the image on the retina, sending this
information to the cortical areas controlling eye movements, where
the image seen combines with internally stored neural information to
produce a more comprehensive blueprint of the visual environment.
The speed with which this is accomplished is remarkable. The
specialized extrastriate areas send output to satellite areas and to
other regions of the cortex. There, potential targets for gaze are
analyzed and selected and quick decisions made: whether or not to
execute a saccadic eye movement from one target to another, for
example, or whether to pursue a moving target in a field of moving
and stationary potential targets or to stay fixed on a target waiting for
it to move.
Once the decision is made, two major types of eye movements
are generated by the cerebral cortex. Volitional intentional saccades,
which are internally triggered to move the eyes toward a target, and
reflexive saccades, which respond to the sudden appearance of a
target on the retina. Both modes of saccadic generation act in
concert and, once initiated, they cannot be stopped. They originate
