- •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
Figure 2-10 Axial MRI scan demonstrates the value of diffusion-weighted imaging in acute infarction. A, Fluid-attenuated inversion recovery image is normal except for nonspecific tiny foci of hyperintense signal in the deep white matter. B, Diffusion-weighted image reveals abnormal restricted diffusion (high-intensity signal) in the territory of the left middle cerebral artery. C, Low-intensity signal on the apparent diffusion coefficient map is consistent with an acute infarction.
(Courtesy of M. Tariq Bhatti, MD.)
Table 2-4
Mukherji SK, Chenevert TL, Castillo M. Diffusion-weighted magnetic resonance imaging. J Neuroophthalmol. 2002;22(2):118– 122.
Symms M, Jäger HR, Schmierer K, Yousry TA. A review of structural magnetic resonance neuroimaging. J Neurol Neurosurg Psychiatry. 2004;75(9):1235–1244.
Vascular Imaging
Several techniques are commonly used to image blood vessels; they are important because of the frequency with which ischemic processes affect the nervous system.
Catheter or Contrast Angiography
The gold standard for intracerebral vascular imaging remains catheter, or contrast, angiography (Fig 2-11). With this technique, a catheter is placed intra-arterially and iodinated radiodense contrast dye is injected. Digital subtraction angiography (DSA) is a technique that reduces artifacts by subtracting densities created by the overlying bony skull. The contrast dye outlines the column of flowing blood within the injected vessel and demonstrates stenosis, aneurysms, vascular malformations, flow dynamics, and vessel wall irregularities such as dissections or vasculitis. The procedure has an overall morbidity of approximately 2.5%, primarily related to ischemia from
emboli or vasospasm, dye-related reactions, or complications at the arterial puncture site (eg, hematoma). The use of digital subtraction technology has enhanced the ability to visualize vascular structures with smaller amounts of contrast dye.
Figure 2-11 Contrast angiogram, lateral view, demonstrates aneurysm of the posterior communicating artery (arrow). ACA = anterior cerebral artery; ICA = internal carotid artery; MCA = middle cerebral artery. (Courtesy of Rod Foroozan, MD.)
Magnetic Resonance Angiography and Magnetic Resonance Venography
Because an MRI signal requires detection of a brief period of excitation and decay, moving tissue often passes out of the plane of assessment before the return signal can be detected. This is the basis of the black “flow void” that characterizes MRI scans of vascular channels with flow. However, protons that are excited in one slice and then move to another may be specifically imaged using the 3- dimensional assessment technique that underlies MRA and MRV. Several techniques, such as 2- and 3-dimensional time-of-flight angiography, phase contrast angiography, and multiple overlapping thinslab acquisition (MOTSA), have been used to obtain images. MRA signals may also be obtained from gadolinium-enhanced images. This technique is particularly useful for imaging the proximal large vessels of the chest and neck. MRA with gadolinium contrast has a very short acquisition time,
making patient movement–related artifacts less of an issue. MRA provides excellent noninvasive information about largeand medium-size vessels (Fig 2-12). Because MRA depends on flow physiology, however, it tends to overestimate vascular stenosis, and the reduced image resolution limits the ability to visualize smaller vessels or vasculitis. MRV may be helpful in excluding thrombosis within the dural venous sinuses (Fig 2-13), a condition that may cause papilledema (see Chapter 14).
Figure 2-12 Magnetic resonance angiography (MRA) provides excellent noninvasive information about largeand medium-size vessels. A, MRA source image showing an abnormality at the junction of the posterior communicating artery and internal carotid artery (arrow). Volume rendered 3-dimensional image (B) and maximal intensity projection image (C) clearly show a posterior communicating artery aneurysm (arrows). (Courtesy of M. Tariq Bhatti, MD.)
Figure 2-13 Venous sinus thrombosis imaging. Magnetic resonance venography image (A) and cerebral angiogram (B) in the venous phase showing absence of flow within the left transverse sinus, sigmoid sinus, and internal jugular vein.
(Reprinted with permission from Foroozan R, Kline LB. Papilledema. In: Kline LB, Foroozan R, eds. Optic Nerve Disorders. 2nd ed. Ophthalmology Monograph 10. New York: Oxford University Press, in cooperation with the American Academy of Ophthalmology; 2007:55.)
Computed Tomography Angiography and Computed Tomography Venography
Computed tomography angiography (CTA) uses a high-speed spiral scanner that provides excellent vessel resolution, with 3-dimensional capability that is complementary to MRA. The technique requires use of iodinated contrast dye and employs ionizing radiation; the procedure takes approximately 15 minutes. Sensitivities in the detection of aneurysms >3 mm in diameter or stenosis >70% are approximately 95%. Some medical centers prefer the use of CTA to MRA in the detection of cerebral aneurysms, including aneurysms causing ocular motor cranial nerve palsies (see Chapter 8, Fig 8-9). New multidetector CTA techniques can accurately detect aneurysms >3–4 mm in diameter in almost all patients with cranial nerve (CN) III palsies.
Computed tomography venography (CTV) is an excellent imaging modality for visualizing the cerebral venous system and is comparable to MRV.
Mathew MR, Teasdale E, McFadzean RM. Multidetector computed tomographic angiography in isolated third nerve palsy. Ophthalmology. 2008;115(8):1411–1415.
Osborn AG, Blaser SI, Salzman KL, et al. Diagnostic Imaging: Brain. 1st ed. Salt Lake City: Amirsys; 2004.
Metabolic and Functional Imaging Modalities
Magnetic resonance spectroscopy (MRS) can provide information about the integrity and metabolism of neural tissue. This technique generally depends on the presence of hydrogen and phosphorus in tissue and produces 5 principal spectra: N-acetylaspartate (NAA), choline, creatinine, lactate, and lipid. These MRS spectra have the following significance:
NAA is associated with neuronal integrity; a decrease in NAA is associated with neuronal loss. Choline is a component of cell membranes.
Creatinine is relatively stable in the brain and can serve as an internal control. Lactate, normally barely visible, is a marker of anaerobic metabolism.
Lipid is not significant.
Changes in the pattern of the spectra peaks are associated with different disease processes; for example, a nonnecrotic brain neoplasm typically produces an elevated choline peak and a reduced NAA peak. Use of MRS can help characterize abnormalities detected initially on MRI.
Functional MRI (fMRI) depends on changes in regional blood flow in the brain in accord with metabolic demand. With very fast MRI analysis, local differences in blood oxygenation (blood oxygenation level–dependent [BOLD] responses) can be evaluated. Based on this technique, areas of higher metabolic activity can be identified during specific tasks, such as reading, speaking, or moving a finger. In neuro-ophthalmology, fMRI is a reliable technique allowing accurate visualization of the cortical areas involved in ocular motility, and of cortical retinotopy within the visual cortex. Besides being less expensive than positron emission tomography (discussed next), fMRI has the additional advantages of faster imaging speed, higher spatial resolution, practical repeatability, and being less
