- •Contents
- •General Introduction
- •Objectives
- •Introduction
- •1 Neuro-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
- •2 Neuroimaging 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
- •3 The 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
- •Ocular Media Abnormality
- •Retinopathy
- •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
- •5 The 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
- •6 The 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
- •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
- •8 The Patient With Diplopia
- •History
- •Physical Examination
- •Monocular 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
- •9 The 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
- •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
- •Examination Techniques
- •Afferent Visual Pathway
- •Ocular Motility and Alignment
- •Pupils and Accommodation
- •Eyelid Position and Function
- •Management of the Patient With Nonorganic Complaints
- •Immunologic Disorders
- •Giant Cell Arteritis
- •Multiple Sclerosis
- •Myasthenia Gravis
- •Thyroid Eye Disease
- •Sarcoidosis
- •Inherited Disorders With Neuro-Ophthalmic Signs
- •Myopathies
- •Neurocutaneous Syndromes
- •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
CHAPTER 2
Neuroimaging in Neuro-Ophthalmology
Ophthalmologists have a variety of neuroimaging modalities at their disposal in the diagnosis and management of ocular, orbital, and intracranial diseases. Three broad clinical questions should be addressed before selecting the appropriate neuroimaging technique:
1.What is the clinical presentation?
2.Where is the lesion resulting in the clinical presentation?
3.What are the potential etiologies of the lesion?
The choice of imaging modality and the timing of the study should be based on the
differential diagnosis
type of lesion suspected (eg, vascular) patient characteristics
age
weight
comorbidities
issues with claustrophobia
possible allergies

presence of metallic foreign bodies or medical devices (eg, cardiac pacemaker) availability of imaging modalities to match the clinical urgency of the condition
In general, most neuro-ophthalmic diseases are best assessed with magnetic resonance imaging (MRI) of the brain and orbit using contrast and fat-saturation techniques. If a vascular abnormality is suspected, magnetic resonance angiography (MRA) or magnetic resonance venography (MRV) can be added.
The importance of open communication between the ophthalmologist and radiologist cannot be stressed enough, not only in selecting the best imaging modality for the patient but also when interpreting the study results. Several types of prescriptive or interpretive errors may occur when ordering and interpreting an MRI:
Prescriptive errors include
failure to apply a dedicated study
inappropriate use of a dedicated study
omission of intravenous contrast material

omission of specialized sequences Interpretive errors include
failure to detect a lesion due to misleading clinical information
rejection of a clinical diagnosis because an expected imaging abnormality is absent the assumption that a striking imaging abnormality accounts for the clinical abnormality failure to consider the lack of clinical specificity of an imaging abnormality
This chapter discusses the 2 most common neuroimaging techniques used in neuro-ophthalmology clinical practice: computed tomography (CT; Fig 2-1) and MRI (Fig 2-2). The use of plain (x-ray) films in the evaluation of neuro-ophthalmic diseases has largely become obsolete, except to evaluate for a metallic foreign body, and is not discussed. A glossary of neuroimaging terminology appears at the end of the chapter.
Figure 2-1 Computed tomography (CT) scans. Axial (A) and coronal (B) orbital views of a healthy subject. (Courtesy of Rod
Foroozan, MD.)
Figure 2-2 Brain and orbital MRI scans showing anatomical visual and orbital structures from the chiasm to the anterior orbit. Note the left eyeball is abnormal but not pertinent to the objective of this figure. Axial T1-weighted (A), coronal T1weighted (B–D), coronal T2-weighted with fat saturation (E), and coronal T1-weighted (F) images. ACF = anterior cranial fossa; Ant segment = anterior segment; ICA = internal carotid artery; IO = inferior oblique muscle; IR = inferior rectus muscle; LR = lateral rectus muscle; Lev P = levator palpebrae superioris muscle; MCF = middle cranial fossa; MR = medial rectus muscle; olf fossa = olfactory fossa; SO = superior oblique muscle; sph sinus = sphenoid sinus; sph wing = sphenoid wing; SR = superior rectus muscle; temp lobe = temporal lobe; vit = vitreous. (Courtesy of M. Tariq Bhatti, MD.)
Jäger HR. Loss of vision: imaging the visual pathways. Eur Radiol. 2005;15(3):501–510. Epub 2005 Jan 26.
Mafee MF, Karimi A, Shah JD, Rapoport M, Ansari SA. Anatomy and pathology of the eye: role of MR imaging and CT. Magn Reson Imaging Clin N Am. 2006;14(2):249–270.
Vaphiades MS. Imaging the neurovisual system. Ophthalmol Clin North Am. 2004;17(3):465–480, viii.
Wolintz RJ, Trobe JD, Cornblath WT, Gebarski SS, Mark AS, Kolsky MP. Common errors in the use of magnetic resonance imaging for neuro-ophthalmic diagnosis. Surv Ophthalmol. 2000;45(2):107–114.
Computed Tomography
Computed tomography (CT) uses computer (digital) processing to generate a 2-dimensional or 3- dimensional image from a series of 2-dimensional x-ray (sectional) images. By manipulating (“windowing”) the data, various structures can be visualized. The orbit is particularly suited to x- ray–based imaging because fat provides excellent contrast to the globe, lacrimal gland, optic nerve, and extraocular muscles. Bone and other calcium-containing processes can be visualized easily because of their marked x-ray attenuation. Soft-tissue details can be further enhanced by the injection of iodinated contrast material, which crosses a disturbed blood–brain barrier to accumulate within a local lesion and reveals inflammatory and neoplastic processes.
Tremendous advancements in CT technology and techniques have occurred since the introduction of CT into clinical practice in the early 1980s. Compared with the single-step imaging technique of first-generation CT scanners, most modern CT scanners image the patient with simultaneous sectioning (ie, spiral or helical) and have multiplanar reconstruction capabilities using a volumerendering method known as maximum intensity projection. The advantages of CT include relatively low cost, rapid image acquisition, wide availability, and excellent spatial resolution. The speed of CT and its ability to identify acute blood or bone abnormalities accurately make this technique especially useful in trauma, which may involve a confused or combative patient, as well as when identification of hemorrhage or bony abnormalities is crucial. The advantages and disadvantages of CT are summarized in Table 2-1.
Table 2-1
