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Neuro-Ophthalmology
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What Do I Do Now?
Lawrence C. Newman, MD
Director of the Headache Division
Professor of Neurology
New York University Langone
New York, New York
Morris Levin, MD
Director of the Headache Center
Professor of Neurology
University of California, San Francisco
San Francisco, California
OT H E R VO L U M E S IN T HE SE RIE S
Headache and Facial Pain
Epilepsy
Pain
Emergency Neurology
Neuroinfections
Neurogenetics
Neurotology
Pediatric Neurology
Neurocritical Care
Stroke
Peripheral Nerve and Muscle Disease
Cerebrovascular Disease
Movement Disorders
Women’s Neurology
Neuroimmunology
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Neuro-Ophthalmology
SECOND EDITION
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1
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the University’s objective of excellence in research, scholarship, and education
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Press in the UK and certain other countries.
Published in the United States of America by Oxford University Press
198 Madison Avenue, New York, NY 10016, United States of America.
© Oxford University Press 2019
First Edition published in 2011
Second Edition published in 2019
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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
Names: Thurtell, Matthew J., author. | Tomsak, Robert L., author.
Title: Neuro-ophthalmology / by Matthew J. Thurtell, Robert L. Tomsak.
Description: Second edition. | New York, NY : Oxford University Press, [2019] |
Includes bibliographical references and index.
Identifiers: LCCN 2018054874 | ISBN 9780190603953 (pbk.)
Subjects: | MESH: Eye Diseases—diagnosis | Nervous System Diseases—complications |
Cranial Nerve Diseases | Eye Diseases—therapy | Diagnosis, Differential | Case Reports
Classification: LCC RE75 | NLM WW 460 | DDC 617.7075—dc23
LC record available at https://lccn.loc.gov/2018054874
This material is not intended to be, and should not be considered, a substitute for medical or other
professional advice. Treatment for the conditions described in this material is highly dependent on
the individual circumstances. And, while this material is designed to offer accurate information with
respect to the subject matter covered and to be current as of the time it was written, research and
knowledge about medical and health issues is constantly evolving and dose schedules for medications
are being revised continually, with new side effects recognized and accounted for regularly. Readers
must therefore always check the product information and clinical procedures with the most up-to-date
published product information and data sheets provided by the manufacturers and the most recent
codes of conduct and safety regulation. The publisher and the authors make no representations or
warranties to readers, express or implied, as to the accuracy or completeness of this material. Without
limiting the foregoing, the publisher and the authors make no representations or warranties as to the
accuracy or efficacy of the drug dosages mentioned in the material. The authors and the publisher do
not accept, and expressly disclaim, any responsibility for any liability, loss or risk that may be claimed
or incurred as a consequence of the use and/or application of any of the contents of this material.
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Printed by WebCom, Inc., Canada
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Contents
Preface ix
Acknowledgments xi
1 Optic Neuritis 3
7 Neuroretinitis 35
8 Papilledema 41
10 Pseudopapilledema 55
11 Chiasmal Syndromes 61
12 Homonymous Hemianopia 67
34 Anisocoria 187
Index 231
viii Contents
ix
Preface
Acknowledgments
SECTION I
Afferent Disorders
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3
1 Optic Neuritis
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4
FIGURE 1.1. MRI of the orbits (top row) and brain (bottom row) in a patient with optic neuritis,
demonstrating left optic nerve enhancement and multiple ovoid periventricular white matter
lesions consistent with MS.
1. Optic Neuritis 5
6
FIGURE 1.2. MRI of the orbits in a patient with bilateral optic neuritis in the setting of
neuromyelitis optica, demonstrating longitudinally extensive bilateral optic nerve enhancement.
Lack of pain
Severe vision loss
Poor recovery of vision
Bilateral optic nerve involvement
Systemic symptoms or signs (e.g., fever or rash)
Intraocular inflammation, hemorrhages, or exudates
Immunocompromised patient (e.g., transplant recipient)
Longitudinally extensive optic nerve enhancement on MRI
Optic nerve sheath enhancement on MRI
Rapid improvement following initiation of steroids
Relapsing course following withdrawal of steroids
is not routinely required to evaluate for oligoclonal bands and other CSF
markers of MS, because an increased risk of MS is more reliably predicted
by the presence of white matter lesions on MRI.
The recommended treatment of idiopathic optic neuritis is based on the
findings of the Optic Neuritis Treatment Trial, which was a randomized
controlled trial comparing outcomes in patients who received intravenous
and then oral steroids, oral steroids alone, or placebo. The group receiving
intravenous and then oral steroids showed a more rapid recovery of vision
than the placebo group, although the final visual outcome was similar. This
group also showed a lower risk of developing clinically definite MS in the
first 2 years following treatment. The group receiving oral steroids alone did
not show a faster recovery compared with placebo but did show an increased
rate of recurrent optic neuritis attacks compared with the other two groups.
Thus, the recommended treatment protocol for idiopathic optic neuritis
is intravenous methylprednisone (1 g daily) for 3 days followed by oral
prednisone (1 mg/kg daily) for 11 days. The prognosis for visual recovery is
excellent, with vision recovering to near normal in most patients over weeks
to months, although there can be minor persisting visual deficits (e.g., in
contrast and color vision) and clinical signs of optic nerve dysfunction (e.g.,
a relative afferent pupillary defect and optic disc pallor).
In patients who have one or more white matter lesions on MRI, the risk
of developing MS is greater than 65% in the 15 years following an attack of
idiopathic optic neuritis, compared with 25% in patients who do not have
white matter lesions. Treatment with disease-modifying therapy for MS
(e.g., beta-interferon) can reduce the risk of developing MS in patients with
idiopathic optic neuritis who have white matter lesions on MRI. Although
not all optic neuritis patients with white matter lesions will develop clini-
cally definite MS, initiation of disease-modifying therapy should be care-
fully considered.
1. Optic Neuritis 7
8
Further Reading
Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of
corticosteroids in the treatment of acute optic neuritis. N Engl J Med.
1992;326:581–588.
Beck RW, Cleary PA, Trobe JD, et al. The effect of corticosteroids for acute optic
neuritis on the subsequent development of multiple sclerosis. N Engl J Med.
1993;329:1764–1769.
Chen JJ, Flanagan EP, Jitprapaikulsan J, et al. Myelin oligodendrocyte glycoprotein
antibody (MOG-IgG)-positive optic neuritis: clinical characteristics, radiologic
clues and outcome. Am J Ophthalmol. 2018;195:8–15.
Optic Neuritis Study Group. Multiple sclerosis risk after optic neuritis: final optic
neuritis treatment trial follow-up. Arch Neurol. 2008;65:727–732.
Thompson AJ, Banwell BL, Barkhof F, et al. Diagnosis of multiple sclerosis: 2017
revisions of the McDonald criteria. Lancet Neurol. 2018;17:162–173.
2 Arteritic Ischemic
Optic Neuropathy
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FIGURE 2.1. Fundus photograph demonstrating pallid optic disc edema due to AAION, with
cilioretinal artery occlusion, in a patient with GCA (left). Fluorescein angiography shows patchy
choroidal nonperfusion and cilioretinal artery occlusion (right).
3). Since GCA causes inflammatory narrowing and occlusion of the poste-
rior ciliary arteries (which supply the choroid of the eye) and the cilioretinal
artery (which variably supplies the papillomacular portion of the retina),
concurrent choroidal or cilioretinal ischemia is highly suggestive of GCA
(see Figure 2.1). Prior to the onset of AAION, a significant proportion of
patients will have episodes of transient monocular or binocular vision loss,
which are typically precipitated by postural changes. Some patients expe-
rience transient diplopia, which is thought to be secondary to ischemia
of the extraocular muscles. Many report systemic symptoms, such as tem-
poral headache, jaw claudication, scalp tenderness, malaise, weight loss, and
fever, which should immediately suggest GCA. However, their absence does
not preclude GCA; over 20% of patients with biopsy-proven GCA do not
have systemic symptoms.
For the patient in this scenario, with a dull temporal headache and se-
vere monocular vision loss due to anterior ischemic optic neuropathy, the
clinical suspicion for GCA is high. Therefore, urgent investigations and
treatment are required. An erythrocyte sedimentation rate and C-reactive
protein level should be obtained immediately. Most patients with GCA
have elevation of both inflammatory markers, reflecting systemic inflam-
mation, but occasionally only one might be elevated. In patients who do
not have elevated inflammatory markers, further investigations should still
be obtained and empiric treatment initiated if the clinical suspicion for
Further Reading
González-Gay MA, García-Porrúa C, Llorca J, et al. Visual manifestations of
giant cell arteritis: trends and clinical spectrum in 161 patients. Medicine.
2000;79:283–292.
Hayreh SS, Zimmerman B. Management of giant cell arteritis: our 27-year clinical
study: new light on old controversies. Ophthalmologica. 2003;217:239–259.
Kawasaki A, Purvin V. Giant cell arteritis: an updated review. Acta Ophthalmol.
2009;87:13–32.
Parikh M, Miller NR, Lee AG, et al. Prevalence of a normal C-reactive protein with an
elevated erythrocyte sedimentation rate in biopsy-proven giant cell arteritis.
Ophthalmology. 2006;113:1842–1845.
Stone JH, Tuckwell K, Dimonaco S, et al. Trial of tocilizumab in giant-cell arteritis. N
Engl J Med. 2017;377:317–328.
3 Nonarteritic Ischemic
Optic Neuropathy
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FIGURE 3.1. Fundus photographs demonstrating optic disc edema due to NAION in the right eye
and a small, structurally congested optic disc (“disc at risk”) in the left eye.
* * * * *
“To all whom it may concern: Be it known that
I, Hugh Auld, of the city of Baltimore, in Baltimore
county in the state of Maryland, for divers good
causes and considerations, me thereunto
moving, have released from slavery, liberated,
manumitted, and set free, and by these presents
do hereby release from slavery, liberate,
manumit, and set free, my negro man, named
Frederick Baily, otherwise called Douglass,
being of the age of twenty-eight years, or
thereabouts, and able to work and gain a
sufficient livelihood and maintenance; and him
the said negro man, named Frederick
Douglass, I do declare to be henceforth free,
manumitted, and discharged from all manner of
servitude to me, my executors and
administrators forever.
“In witness whereof, I the said Hugh Auld,
have hereunto set my hand and seal the fifth of
December, in the year one thousand eight
hundred and forty-six.
Hugh Auld.
“Sealed and delivered in presence of T.
Hanson Belt, James N. S. T. Wright.”