Eye Movement Disorders in Clinical Practice

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 You must not circulate this work in any other form and you must
impose this same condition on any acquirer.
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
and clinical experience broaden our knowledge, changes in
treatment and drug therapy occur. The author and publisher of this
work have checked with sources believed to be reliable in their
efforts to provide information that is accurate and complete, and in
accordance with the standards accepted at the time of publication.
However, in light of the possibility of human error or changes in the
practice of medicine, neither the author, nor the publisher, nor any
other party who has been involved in the preparation or publication
of this work warrants that the information contained herein is in every
respect accurate or complete. Readers are encouraged to confirm
the information contained herein with other reliable sources, and are
strongly advised to check the product information sheet provided by
the pharmaceutical company for each drug they plan to administer.
 135798642
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

This 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
either in anterior cortical areas in the frontal cortex or in posterior
cortical areas in the parietal lobe and superior temporal sulcus.

Stabilizing the Image Seen

For the clearest view of an image of interest, the image must be held
within 0.5 degrees of the fovea, where photoreceptor density is
greatest and visual acuity is at its best, and it must be stable and
steady.1 Unless movements of the eyes compensate for motion,
images of interest cannot be held stable and steady and would slip
off the fovea with every head movement. Retinal slip causes visual
blur, nystagmus, and oscillopsia, an illusion that the visual world
itself is moving.
Two types of reflex eye movements are needed to stabilize the
image on the retina, the vestibulo-ocular reflex (VOR) and visually
mediated reflexes. During brief head movements, the VOR stabilizes
retinal images by counter-rotating the eyes at the same speed as the
head but in the opposite direction. Head acceleration signals pass
from the vestibular sensors in the labyrinth of the inner ear to VOR
circuits in the brainstem that compute an appropriate eye velocity
command to compensate for changes in position and orientation of
the head; because the VOR is a vestibular-mediated reflex, it
operates even in the dark.
Acting together with the VOR, three visually mediated classes of
eye movements keep the image on the fovea: visual fixation,
optokinetic eye movements, and smooth pursuit. Fixation opposes
saccadic movement of the eyes away from a stationary target. When
the eyes adjust to a visual scene during sustained self-rotation (e.g.,
being in a stationary vehicle next to one that is moving but feeling as
if the stationary vehicle is moving and the moving vehicle is still), the
eye movements needed to correct this are described as optokinetic.
When the eye follows a moving object or maintains fixation on a near
stationary target while one is oneself in motion, the movements are
called smooth pursuit.
In each case, areas of cerebral cortex (the striate and extrastriate
visual cortex) extract information about the direction and speed of
retinal image-slip from each eye and send signals to the brainstem
and cerebellar circuits to program an eye movement to correct
image-slip as the image moves off the fovea. These visual areas all
act together to stabilize the angle of gaze (eye position in space) so
as to hold the image of interest fairly stationary on the fovea of each
eye while the subject is in motion.
Still other eye movements are needed to look at a new object of
interest because this requires shifting gaze and redirecting the line of
sight to a new target. Saccades jump the fixation point from one
feature to another during visual search, and most saccades are
completed in less than 100 ms. During these brief eye movements
the eye is apparently sightless.2

Parallel Visual Pathways

Cortical areas concerned with vision are organized into two parallel
pathways: one, the ventral pathway, processes form and color; the
other, the dorsal pathway, processes motion (Figure 1-1). This is a
useful division, allowing disorders of color processing and object
recognition to be grouped as ventral pathway disorders involving
occipitotemporal structures and disorders of motor processing and
spatial processing as dorsal pathway disorders involving lateral
occipitoparietal structures. Colloquially, these have been dubbed the
“what and where” pathways, although this easy mnemonic has been
challenged by the suggestion that the dorsal pathway is configured
for preparing responses to the environment and is better identified as
an “action” pathway.

THE STRIATE CORTEX

The striate cortex (visual area V1) is of fundamental importance in

controlling visually guided eye movements.3
FIGURE 1-1 Schematic of the dorsal and ventral processing
streams. MST, medial superior temporal area; LO, lateral occipital;
FFA, fusiform face area; PPA, parahippocampal place area. The
ventral stream begins in layer 4Cβ of area V1. The dorsal stream
begins in motion-sensitive components of layer 4Cα in area V1.
Reproduced with permission.3

The primary visual cortex (the striate and extrastriate cortex V1 to

V2) receives visual inputs from the retina. The attributes of the visual
images received (color, motion, form, depth, and distance) are then
distributed along the ventral and dorsal parallel pathways for further
analysis by other visual association areas (V3–V4, color sensitive;
V5, the middle temporal (MT) area sensitive to motion; and V6).
The dorsal motion detection pathway includes MT and the medial
superior temporal (MST) area. MT contains neurons that encode the
speed and direction of a target4 and MST’s neurons carry, in
addition, eye movement signals that help to maintain smooth
pursuit.5,6
 THE FRONTAL CORTEX

There are four saccadic areas or eye fields in the frontal cortex: the
frontal eye fields (FEFs), the supplementary eye field (SEF), the
dorsolateral prefrontal cortex (DLPC), and the cingulate eye field
(CEF) (Figure 1-2).
The FEFs generate all voluntary intentional saccades (memory-
guided saccades, predictive saccades, antisaccades, and visually
guided saccades) and help maintain smooth pursuit and vergence.
They signal the brainstem circuits for immediate premotor saccade
commands.
The SEF is important in generating saccades to respond to both
visual and nonvisual cues.
The DLPC, also called the prefrontal eye field (PFEF), lies on the
dorsal convexity of the frontal lobe and receives inputs from the FEF,
SEF, and the posterior parietal cortex (PPC). This cortical area
projects to the FEF, SEF, superior colliculus (SC), and the
paramedian pontine reticular formation (PPRF). It is important for
programming memory-guided saccades and antisaccades.
FIGURE 1-2 Location of cortical visual areas in the frontal lobe.
Reproduced with permission.7

The CEF in the anterior cingulate cortex is active during memory-

guided saccades, antisaccades, and intentional saccades.7,8

Clinical Points to Remember About Lesions of the Frontal

Cortex
• Acute FEF lesions produce a contralateral horizontal gaze palsy
manifested by gaze deviation or gaze preference (the patient
looks away from the hemiparesis to the opposite side). This
resolves in time.
• Patients with chronic bilateral frontal lobe lesions have impaired
initiation of voluntary saccades on command, a condition often
called acquired ocular motor apraxia. A better term is saccadic
initiation defect.
 • Patients with frontocortical dementia have impaired ability to
suppress a reflexive saccade in the antisaccade task (i.e.,
impaired reflexive saccadic inhibition9,10) and are unable to
suppress inappropriate saccades to a novel visual stimulus.
• Lesions of the SEF result in a loss of ability to make a
remembered sequence of saccades to an array of visible targets
in the order that the targets appear (especially the case with left-
sided lesions).
• Focal lesions of the DLPC cause errors in the antisaccade test.
• Focal unilateral lesions of the CEF cause hypometric memory-
guided saccades.11 Larger lesions, for example those due to
resection of a tumor, cause deficits in the generation of
antisaccades.12

In patients with frontal lobe neurodegeneration, deficits in the

ability to suppress automatic behavior can lead to impaired decision
making, aberrant motor behavior, and impaired social function, as
seen in Case 1-1.

CASE 1-1 Pick’s Disease: Frontotemporal

Dementia
FIGURE 1-3 Sixty-eight-year-old man with echolalia.

The patient is a 68-year-old, retired, right-handed man who

presented with a 1-year history of progressive behavioral change
(Figure 1-3). His writing had become less legible, his speech slow
and hesitant, and he was unable to balance his checkbook. When
answering a question, he often had to repeat the question before
giving the answer.
He denied any visual symptoms except trouble judging space and
distance. He reported having had a minor car accident when he
drove 100 feet in reverse, stopping only when he hit the garage door.
He said he had no trouble with memory, no confusion, no difficulty
finding words or understanding speech. He also denied weakness,
stiffness, tremors, numbness, headache, or syncope.
He had experienced a progressive loss of balance, slowness
walking, and difficulty climbing stairs, dressing, using a knife and
fork, and getting in and out of a chair. Because of impaired balance,
he needed to sit to put his trousers on.
Concerned that his “slowing up” might be due to Parkinson’s
disease (PD), his family consulted his primary care doctor who
referred him to Dr. Raymond Adams for an opinion.
Family history was negative for degenerative central nervous
system (CNS) disease. There was no past history of alcohol abuse
or previous stroke or head trauma.

Analysis of the History

• What are the major presenting symptoms?
• Where are the CNS lesion(s) likely to be?

CASE 1-1 SYMPTOMS

Symptoms Location
Correlation
Progressive change in behavior Frontotemporal
lobe
Impaired executive function (unable to Frontal lobe
balance checkbook)
Loss of balance Vestibular
system

The analysis guides the clinical examination.