The American Academy of Ophthalmology is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
The American Academy of Ophthalmology designates this enduring material for a maximum of 15 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
CME expiration date: June 1, 2016. AMA PRA Category 1 Credits™ may be claimed only once between June 1, 2013, and the expiration date.
BCSC® volumes are designed to increase the physician’s ophthalmic knowledge through study and review. Users of this activity are encouraged to read the text and then answer the study questions provided at the back of the book.
To claim AMA PRA Category 1 Credits™ upon completion of this activity, learners must demonstrate appropriate knowledge and participation in the activity by taking the posttest for Section 5 and achieving a score of 80% or higher. For further details, please see the instructions for requesting CME credit at the back of the book.
The Academy provides this material for educational purposes only. It is not intended to represent the only or best method or procedure in every case, nor to replace a physician’s own judgment or give specific advice for case management. Including all indications, contraindications, side effects, and alternative agents for each drug or treatment is beyond the scope of this material. All information and recommendations should be verified, prior to use, with current information included in the manufacturers’ package inserts or other independent sources, and considered in light of the patient’s condition and history. Reference to certain drugs, instruments, and other products in this course is made for illustrative purposes only and is not intended to constitute an endorsement of such. Some material may include information on applications that are not considered community standard, that reflect indications not included in approved FDA labeling, or that are approved for use only in restricted research settings. The FDA has stated that it is the responsibility of the physician to determine the FDA status of each drug or device he or she wishes to use, and to use them with appropriate, informed patient consent in compliance with applicable law. The Academy specifically disclaims any and all liability for injury or other damages of any kind, from negligence or otherwise, for any and all claims that may arise from the use of any recommendations or other information contained herein.
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Cover image: From BCSC Section 12, Retina and Vitreous. Ultra-wide-field fundus photograph from a patient with von Hippel–Lindau disease. Courtesy of Colin A. McCannel, MD.
Copyright © 2015
American Academy of Ophthalmology All rights reserved
Basic and Clinical Science Course
Louis B. Cantor, MD, Indianapolis, Indiana, Senior Secretary for Clinical Education Christopher J. Rapuano, MD, Philadelphia, Pennsylvania, Secretary for Ophthalmic
Knowledge
George A. Cioffi, MD, New York, New York, BCSC Course Chair
Section 5
Faculty
Rod Foroozan, MD, Chair, Houston, Texas
M. Tariq Bhatti, MD, Durham, North Carolina
Julie Falardeau, MD, Portland, Oregon
Lynn K. Gordon, MD, PhD, Los Angeles, California
Michael S. Lee, MD, Minneapolis, Minnesota
Prem S. Subramanian, MD, PhD, Baltimore, Maryland
Aki Kawasaki, MD, Consultant, Lausanne, Switzerland
The Academy wishes to acknowledge the North American Neuro-Ophthalmology Society (NANOS) for recommending faculty members to the BCSC Section 5 committee.
The Academy also wishes to acknowledge the following committees for review of this edition:
Committee on Aging: Julie Falardeau, MD, Portland, Oregon
Vision Rehabilitation Committee: Thomas J. O’Donnell, MD, Memphis, Tennessee
Practicing Ophthalmologists Advisory Committee for Education: James Mitchell, MD, Primary Reviewer, Edina, Minnesota; William S. Clifford, MD, Past Chair, Garden City, Kansas; Hardeep S. Dhindsa, MD, Reno, Nevada; Robert Fante, MD, Denver, Colorado; Dasa Gangadhar, MD, Wichita, Kansas; Edward K. Isbey III, MD, Asheville, North Carolina; Sara O’Connell, MD, Overland Park, Kansas; Robert E. Wiggins Jr MD, Asheville, North Carolina
European Board of Ophthalmology: Klara Landau, MD, FEBO, EBO Chair, Zürich, Switzerland; Marko Hawlina, MD, PhD, EBO Liaison, Ljubljana, Slovenia; François-Xavier Borruat, MD, Lausanne, Switzerland; Antonella Boschi, MD, Brussels, Belgium; Michael Anthony Burdon, MBBS,
Birmingham, England, United Kingdom; Wolf A. Lagrèze, MD, Freiburg, Germany; Dan Milea, MD, PhD, Angers, France
Financial Disclosures
Academy staff members who contributed to the development of this product state that within the past 12 months, they have had no financial interest in or other relationship with any entity discussed in this course that produces, markets, resells, or distributes ophthalmic health care goods or services consumed by or used in patients, or with any competing commercial product or service.
The authors and reviewers state the following financial relationships:*
Dr Bhatti: Bayer HealthCare (L), Biogen Idec (C), EMD Serono (L), Novartis Pharmaceuticals (C, L), Pfizer (L)
Dr Clifford: Transcend Medical (S)
Dr Gangadhar: Inspire Pharmaceuticals (C, L)
Dr Gordon: VentiRx Pharmaceuticals (C)
Dr Kawasaki: Bayer HealthCare Pharmaceuticals (C)
Dr Landau: Santhera Pharmaceuticals, Switzerland (C)
Dr Lee: Merz Pharmaceuticals (C), National Eye Institute (S), Pfizer (S), Quark Pharmaceuticals (S), Teva Pharmaceutical Industries (S)
Dr Milea: Alcon Laboratories (L)
Dr Subramanian: Lundbeck (S), Pfizer (S)
Dr Wiggins: Medflow/Allscripts (C), Ophthalmic Mutual Insurance Company (C)
The other authors and reviewers state that they have no significant financial interest or other relationship with the manufacturer of any commercial product discussed in this course or with the manufacturer of any competing commercial product.
*C = consultant fees, paid advisory boards, or fees for attending a meeting; L = lecture fees (honoraria), travel fees, or reimbursements when speaking at the invitation of a commercial sponsor; O = equity ownership/stock options of publicly or privately traded firms (excluding mutual funds) with manufacturers of commercial ophthalmic products or commercial ophthalmic services; P = patents and/or royalties that might be viewed as creating a potential conflict of interest; S = grant support for the past year (all sources) and all sources used for a specific talk or manuscript with no time limitation
Recent Past Faculty
Sophia Mihe Chung, MD
Eric Eggenberger, DO
Karl C. Golnik, MD
Lanning B. Kline, MD
In addition, the Academy gratefully acknowledges the contributions of numerous past faculty and advisory committee members who have played an important role in the development of previous editions of the Basic and Clinical Science Course.
American Academy of Ophthalmology Staff
Dale E. Fajardo, EdD, MBA |
Beth Wilson, CHCP |
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Vice President, Education |
Director, CME, Programs & Acquisitions |
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Daniel Mummert |
Christine A. Arturo |
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Director, Online Education & the |
Acquisitions Manager |
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ONE Network |
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Jasmine Chen |
Stephanie Tanaka |
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Manager, E-Learning |
Publications Manager, BCSC |
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Eric Gerdes |
D. Jean Ray |
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Interactive Designer |
Production Manager |
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Donna Scism |
Ann McGuire |
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E-Editor/Proofreader |
Medical Editor, BCSC |
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Contents
Title Page
Copyright Page
Faculty and Disclosures
General Introduction
Visual Acuity Chart
Objectives
Introduction
1Neuro-Ophthalmic Anatomy
Bony Anatomy
Skull Base
The Orbit
Vascular Anatomy
Arterial System
Venous System
Afferent Visual Pathways
Retina
Optic Nerve
Optic Chiasm
Optic Tract
Cortex
Efferent Visual System (Ocular Motor Pathways)
Cortical Input
Brainstem
Ocular Motor Cranial Nerves
Extraocular Muscles
Sensory and Facial Motor Anatomy
Trigeminal Nerve (CN V)
Facial Nerve (CN VII)
Eyelids
Ocular Autonomic Pathways
Sympathetic Pathways
Parasympathetic Pathways
2Neuroimaging in Neuro-Ophthalmology
Computed Tomography
Magnetic Resonance Imaging
Vascular Imaging
Catheter or Contrast Angiography
Magnetic Resonance Angiography and Magnetic Resonance Venography
Computed Tomography Angiography and Computed Tomography Venography
Metabolic and Functional Imaging Modalities
Sonography
Retinal and Nerve Fiber Layer Imaging
Fundamental Concepts in Localization
Crucial Questions in Imaging
When to Order
What to Order
How to Order
Negative Study Results
Glossary
3The Patient With Decreased Vision: Evaluation
History
Unilateral Versus Bilateral Involvement
Time Course of Vision Loss
Associated Symptoms
Examination
Best-Corrected Visual Acuity
Color Vision Testing
Pupillary Testing
Fundus Examination
Visual Field Evaluation
Adjunctive Testing
4The Patient With Decreased Vision: Classification and
Management
Ocular Media Abnormality
Retinopathy
Acute Idiopathic Blind-Spot Enlargement and Multiple Evanescent White Dot Syndrome
Vitamin A Deficiency
Hydroxychloroquine and Chloroquine Retinopathy
Cone Dystrophy
Paraneoplastic Syndromes
Optic Neuropathy
Visual Field Patterns in Optic Neuropathy
Anterior Optic Neuropathies With Optic Disc Edema
Anterior Optic Neuropathies Without Optic Disc Edema
Posterior Optic Neuropathies
Optic Atrophy
Chiasmal Lesions
Visual Field Loss Patterns
Etiology of Chiasmal Disorders
Retrochiasmal Lesions
Optic Tract
Lateral Geniculate Body
Temporal Lobe
Parietal Lobe
Occipital Lobe
Visual Rehabilitation
5The Patient With Transient Visual Loss
Examination
Transient Monocular Visual Loss
Ocular Causes
Orbital Causes
Systemic Causes
Vasospasm, Hyperviscosity, and Hypercoagulability
Transient Binocular Visual Loss
Migraine
Occipital Mass Lesions
Occipital Ischemia
Occipital Seizures
6The Patient With Illusions, Hallucinations, and Disorders of
Higher Cortical Function
The Patient With Visual Illusions and Distortions
Ocular Origin
Optic Nerve Origin
Cortical Origin
The Patient With Hallucinations
Ocular Origin
Optic Nerve Origin
Cortical Origin
The Patient With Disorders of Higher Cortical Function
Disorders of Recognition
Disorders of Visual–Spatial Relationships
Disorders of Awareness of Vision or Visual Deficit
7The Patient With Supranuclear Disorders of Ocular Motility
Fundamental Principles of Ocular Motor Control
Anatomy and Clinical Testing of the Functional Classes of Eye Movements
Ocular Stability
Vestibular Ocular Reflex
Optokinetic Nystagmus
Saccadic System
Pursuit System
Vergence
Clinical Disorders of the Ocular Motor Systems
Ocular Stability Dysfunction
Vestibular Ocular Dysfunction
Optokinetic Nystagmus Dysfunction
Saccadic Dysfunction
Pursuit Dysfunction
Vergence Disorders
8The Patient With Diplopia
History
Physical Examination
Monocular Diplopia
Differentiating Paretic From Restrictive Etiologies of Diplopia
Comitant and Incomitant Deviations
Localization
Supranuclear Causes of Diplopia
Skew Deviation
Thalamic Esodeviation
Vergence Dysfunction
Nuclear Causes of Diplopia
Internuclear Causes of Diplopia
One-and-a-Half Syndrome
Infranuclear Causes of Diplopia
Third Nerve Palsy
Fourth Nerve Palsy
Sixth Nerve Palsy
Neuromyotonia
Paresis of More Than One Cranial Nerve
Cavernous Sinus and Superior Orbital Fissure Involvement
Neuromuscular Junction Causes of Diplopia
Myopathic, Restrictive, and Orbital Causes of Diplopia
Thyroid Eye Disease
Posttraumatic Restriction
Post–Cataract Extraction Restriction
Orbital Myositis
Neoplastic Involvement
Brown Syndrome
9The Patient With Nystagmus or Spontaneous Eye Movement
Disorders
Introduction
Early-Onset (Childhood) Nystagmus
Infantile Nystagmus Syndrome (Congenital Nystagmus)
Fusional Maldevelopment Nystagmus Syndrome (Latent Nystagmus)
Monocular Nystagmus of Childhood
Spasmus Nutans
Gaze-Evoked Nystagmus
Rebound Nystagmus
Vestibular Nystagmus
Peripheral Vestibular Nystagmus
Central Forms of Vestibular Nystagmus
Acquired Pendular Nystagmus
Oculopalatal Myoclonus or Tremor
See-Saw Nystagmus
Dissociated Nystagmus
Saccadic Intrusions
Saccadic Intrusions With Normal Intersaccadic Intervals
Saccadic Intrusions Without Normal Intersaccadic Intervals
Voluntary Flutter (“Nystagmus”)
Additional Eye Movement Disorders
Convergence-Retraction Nystagmus
Superior Oblique Myokymia
Oculomasticatory Myorhythmia
Treatment of Nystagmus and Other Eye Movement Disorders
Eye Movements in Comatose Patients
Ocular Bobbing
10 The Patient With Pupillary Abnormalities
History
Pupillary Examination
Baseline Pupil Size
Pupil Irregularity
Anisocoria
Anisocoria Equal in Dim and Bright Light
Anisocoria Greater in Dim Light
Anisocoria Greater in Bright Light
Disorders of Pupillary Reactivity: Light–Near Dissociation
Afferent Visual Pathway
Midbrain
Aberrant Regeneration
Other Pupillary Disorders
Benign Episodic Pupillary Mydriasis
11 The Patient With Eyelid or Facial Abnormalities
Examination Techniques Ptosis
Congenital Ptosis
Acquired Ptosis Pseudoptosis
Apraxia of Eyelid Opening Eyelid Retraction
Abnormalities of Facial Movement Seventh Nerve Disorders
Disorders of Underactivity of the Seventh Nerve
Disorders of Overactivity of the Seventh Nerve
12 The Patient With Head, Ocular, or Facial Pain
Evaluation of Headache
Migraine and Tension-type Headache
Trigeminal Autonomic Cephalgias and Hemicrania Continua Idiopathic Stabbing Headache
Inherited Encephalopathies Resembling Migraine Ocular and Orbital Causes of Pain
Trochlear Headache and Trochleitis Photophobia
Facial Pain
Trigeminal Neuralgia Glossopharyngeal Neuralgia Occipital Neuralgia Temporomandibular Disease Carotid Dissection
Herpes Zoster Ophthalmicus Neoplastic Processes Mental Nerve Neuropathy
13 The Patient With Nonorganic Ophthalmic Disorders
Examination Techniques
Afferent Visual Pathway
Ocular Motility and Alignment
Pupils and Accommodation
Eyelid Position and Function
Management of the Patient With Nonorganic Complaints
14 Selected Systemic Conditions With Neuro-Ophthalmic Signs
Immunologic Disorders
Giant Cell Arteritis
Multiple Sclerosis
Myasthenia Gravis
Thyroid Eye Disease
Sarcoidosis
Inherited Disorders With Neuro-Ophthalmic Signs
Myopathies
Neurocutaneous Syndromes
Selected Neuro-Ophthalmic Disorders Associated With Pregnancy
Posterior Reversible Encephalopathy Syndrome
Lymphocytic Hypophysitis
Cerebrovascular Disorders
Transient Visual Loss
Vertebrobasilar System Disease
Cerebral Aneurysms
Arterial Dissection
Arteriovenous Malformations
Cerebral Venous Thrombosis
Neuro-Ophthalmic Manifestations of Infectious Diseases
Human Immunodeficiency Virus Infection
Herpesvirus
Mycobacterium
Syphilis
Progressive Multifocal Leukoencephalopathy
Toxoplasmosis
Lyme Disease
Fungal Infections
Prion Diseases
Radiation Therapy
Basic Texts
Related Academy Materials
Requesting Continuing Medical Education Credit