- •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 2-15 A 19-year-old man was referred for evaluation of double vision following a motor vehicle accident. A, There is difficulty with adduction of the left eye (left panel), and on attempted abduction of the left eye, there is palpebral fissure narrowing of the same eye (right panel). B, A subsequent coronal CT scan demonstrates an entrapped left medial rectus
muscle (arrow) causing restriction. (Courtesy of Steven A. Newman, MD.)
Mafee MF, Rapoport M, Karimi A, Ansari SA, Shah J. Orbital and ocular imaging using 3- and 1.5-T MR imaging systems.
Neuroimaging Clin N Am. 2005;15(1):1–21.
How to Order
The ophthalmologist may have a role in the selection of the specific type of imaging procedure. The more pertinent the clinical information supplied to the radiologist, the more appropriately the imaging can be tailored to a particular patient. Such information should at least include the expected location of the pathology and the suspected differential diagnosis (eg, region and lesion of interest). Failure to supply such information often results in images that do not show the area of interest or do so with insufficient detail. Inappropriate images (eg, wrong location or orientation, lack of contrast administration, or overly thick slices) are often worse than no images at all because they may provide a false sense of security and may create third-party–payer barriers to the required reimaging. By conveying as much specific clinical information to the radiologist as possible, the ophthalmologist will increase the usefulness of any subsequent studies.
Negative Study Results
The discipline of neuro-ophthalmology has on occasion been called “the reinterpretation of previously negative imaging studies.” When an imaging study fails to demonstrate expected pathology or answer the clinical question, the first step is to reexamine the study parameters, ideally with a neuroradiologist. Several questions to keep in mind are as follows:
Were the appropriate studies performed, including required sequences and orientations? Was the area of interest adequately imaged (Fig 2-16)?
Are the study results really negative (Fig 2-17)?
Figure 2-16 Delayed diagnosis of a right third nerve schwannoma. A, An 11-year-old patient was noted to have a right third nerve palsy that began at age 5 years and became complete by age 7 years. B, Initial study results were negative, but fine cuts through the cavernous sinus demonstrated asymmetry, with a slight nodule in the superior portion of the cavernous sinus on the right. C, This area became bright with administration of gadolinium contrast, which indicated the presence of a right third nerve schwannoma (arrow). (Courtesy of Steven A. Newman, MD.)
Figure 2-17 A 41-year-old woman was referred for progressive vision loss in the right eye. She had been told she had a swollen optic nerve on the right; her condition was diagnosed as a “mild form of multiple sclerosis.” Visual acuity was 2/200 OD and 20/20 OS, with a right afferent pupillary defect. She had reportedly had 2 previous MRI scans, with negative results. A, Fundus photograph of the right optic disc demonstrated temporal pallor with optociliary shunt vessels. The patient was referred for a third MRI scan, but this study was misdirected for workup of “microvascular brainstem disease” and revealed no abnormalities. B, Sagittal MRI scan taken through the orbit shows abnormal optic nerve sheath appearance consistent with optic nerve sheath meningioma (arrow). (Part A courtesy of Steven A. Newman, MD; part B courtesy of
Eric Eggenb erger, DO.)
Even if the ophthalmologist cannot personally review the studies, speaking directly with the radiologist may prevent certain lesions from being overlooked and can provide the clinical information required to enhance the radiographic report’s accuracy and usefulness.
Glossary
ADC (apparent diffusion coefficient) See DWI (diffusion-weighted imaging).
BOLD (blood oxygenation level–dependent) MRI functional imaging technique that can demarcate areas of high activity during a specific task.
CTA (computed tomography angiography) CT technique used to image blood vessels.
DWI (diffusion-weighted imaging) and ADC (apparent diffusion coefficient) MRI techniques especially useful for detecting acute and subacute stroke and for differentiating vasogenic from cytotoxic edema.
FLAIR (fluid-attenuated inversion recovery) MRI technique that highlights T2-hyperintense abnormalities adjacent to CSF (cerebrospinal fluid)-containing spaces, such as the ventricles, by
suppressing CSF signal intensity.
fMRI (functional magnetic resonance imaging) MRI technique that allows visualization of more active brain areas during a specific task, such as reading.
Gadolinium Paramagnetic contrast agent administered intravenously to enhance lesions.
IR (inversion recovery) MRI pulse sequence that nulls the bright signal of fat or water to create a FLAIR or STIR (short tau [or TI] inversion recovery) image. During MRI, initial 180° radiofrequency pulses are followed by a 90° pulse and immediate acquisition of the signal; in IR sequences, the interpulse time is given by TI.
MRA (magnetic resonance angiography) MRI technique for imaging blood vessels.
MRS (magnetic resonance spectroscopy) MRI technique that can further characterize the tissue composition of part of the brain, which helps differentiate tumor, demyelination, and necrosis.
Pixel Picture unit; any of the small, discrete units that together constitute an image (as on a computer screen); increasing the number of pixels that comprise an image increases image resolution.
Relaxation Process by which an element releases (re-emits) energy that has been absorbed from the radiofrequency pulses during an MRI sequence.
SE (spin echo) In the most commonly employed spin-echo sequence, a 180° pulse follows a 90° pulse. For T2-weighted images, the 90° pulse is followed by 2 180° pulses. The first 180° pulse is administered at one-half the TE (time to echo), and the second 180° pulse is administered one full TE later. The “first echo” image is referred to as proton density, and the “second echo” is T2-weighted.
SR (saturation recovery) With SR, the radiofrequency signal is recorded after a series of 90° pulses, with an interpulse interval less than or equal to an average tissue T1 (0.1–1.5 sec).
T1 Time required for 63% of protons to return to the longitudinal plane after cessation of a 90° radiofrequency pulse. This is also referred to as the longitudinal, or spin-lattice, relaxation time.
T2 Time required for 63% of the magnetic field in the transverse plane created by the radiofrequency pulse to dissipate. This dispersion of the magnetic vector corresponds to the exchange of spin among protons and is referred to as spin-spin relaxation; it is completed much more rapidly than is T1 relaxation.
TE (time to echo) Time following the radiofrequency pulse in which the signal is assessed.
Tesla The unit of measure of magnetic field strength.
TI (interpulse time) See IR (inversion recovery).
TR Time to repetition of radiofrequency pulse.
Voxel Three-dimensional cube determined by the product of the pixel size and the slice thickness.
