- •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
Crucial Questions in Imaging
State-of-the-art medical imaging facilities are now widely available. Imaging, however, remains expensive, is a potential drain on medical resources, and is not without risks. Accordingly, it is crucial that the clinician address the following issues while planning the use of an imaging study as part of a neuro-ophthalmic workup:
when to order what to order
modality
location how to order
specify lesion and region of interest discuss with radiologist before ordering review with radiologist after imaging
When to Order
The decision to order an imaging study should be based on clinical localization and the expectation of particular findings. Further, the information acquired by imaging should have some influence on patient management or provide a more accurate prognosis of the disease’s natural history. Finally, the same information should not be available by simpler or less expensive means.
Transient visual phenomena without residual deficit are most frequently related to large-vessel disease and thereby require assessment of the vascular structures. Suspicion of a neoplastic mass lesion is a common reason for ordering an imaging study. The workup of a patient with decreased vision should include imaging of the course of the optic nerve if there is evidence of optic neuropathy that is not otherwise clinically explained. When unilateral disc edema is present, the differential diagnosis should include anterior ischemic optic neuropathy (AION), papillitis, and intraorbital compression. Imaging is not required in classic nonarteritic AION. In acute optic neuritis, a cerebral MRI should be ordered to look for evidence of cerebral white matter signal abnormalities typical of multiple sclerosis; these abnormalities are best demonstrated on T2-weighted or FLAIR images. In most cases of acute optic neuritis, specific orbital MRI sequences (eg, postcontrast T1-weighted with fat saturation, or T2-weighted) will also disclose signal abnormalities within the affected optic nerve (Fig 2-9D). A CT scan has very limited value in demyelinating disease. (See Chapter 14 for further discussion of multiple sclerosis and imaging studies.) When central visual function is affected without disc edema, or when field defects respect the vertical midline (as can occur with parachiasmal or retrochiasmal pathology), appropriate imaging studies should be obtained.
Bitemporal visual field defects localize to the chiasm or parasellar region and necessitate neuroimaging. Some of the most frequent chiasmal-compressive masses include
pituitary adenoma (Fig 2-14) with suprasellar extension meningioma
craniopharyngioma chiasmatic glioma aneurysm
Figure 2-14 MRI scans from a 45-year-old man who presented with a bitemporal defect, impotence, and an elevated prolactin level consistent with the clinical diagnosis of a prolactinoma. A, Coronal T1-weighted MRI postcontrast image shows a large pituitary adenoma stretching and compressing the chiasm (visible as a gray ribbon superior to the adenoma [arrow]). B, MRI appearance several months after treatment with a dopamine agonist shows near resolution of the prolactinoma; the enhancing structure in the suprasellar space is a normal-appearing infundibulum (arrow), with the
chiasm above. (Courtesy of Eric Eggenb erger, DO.)
Rarer compressive lesions in the parasellar region include metastases, chordomas, dysgerminomas, lymphomas, histiocytoses, epidermoids, Rathke cleft cysts, and granulomatous inflammatory disease.
Homonymous visual field defects imply pathology in the retrochiasmal visual pathway. Such defects are most commonly vascular in origin but should be imaged unless clearly associated with a prior stroke syndrome.
When a patient reports binocular double vision, anatomic localization is again crucial, and the pattern of misalignment becomes all-important. As with afferent system dysfunction involving the optic nerve, both CT and MRI studies may provide the necessary information regarding the extraocular muscles. MRI is somewhat more sensitive to the various changes associated with inflammation or infiltration, but CT delineates the size of the extraocular muscles well, particularly on direct (ie, not reformatted) coronal images (Fig 2-1B).
When the pattern of deviation fits an ocular motor cranial nerve palsy (CN III, CN IV, or CN VI), the decision about imaging depends on the clinically suspected pathophysiology and whether the palsy is isolated. The acute onset of an isolated ocular motor cranial nerve palsy in a patient in the age group at higher risk of vasculopathy (usually >50 years), especially when associated with a history of diabetes mellitus, hypertension, or vascular disease, is most likely due to microvascular ischemia, and emergent imaging may not be required. However, a pupil involving CN III palsy requires immediate imaging. With simultaneously occurring ocular motor cranial nerve palsies, especially when the fifth nerve is involved, a cavernous sinus location is most likely.
Skew deviation is a supranuclear lesion producing a vertical misalignment that does not fit the pattern of CN III or CN IV palsy. MRI of the posterior fossa should be obtained.
Certain common clinical situations are associated with negative results on imaging, such as AION (nonarteritic or arteritic), isolated microvascular ischemic ocular motor cranial neuropathy, and eye pain in a patient with normal findings on neuro-ophthalmic examination. However, when pain is associated with findings such as ptosis and miosis (Horner syndrome), carotid artery dissection
should be suspected; MRI/MRA or CT/CTA through the carotid artery is often sufficient for confirmation.
Chi SL, Bhatti MT. The diagnostic dilemma of neuro-imaging in acute isolated sixth nerve palsy. Curr Opin Ophthalmol. 2009;20(6):423–429.
Lee AG, Hayman LA, Brazis PW. The evaluation of isolated third nerve palsy revisited: an update on the evolving role of magnetic resonance, computed tomography, and catheter angiography. Surv Ophthalmol. 2002;47(2):137–157.
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What to Order
With some important exceptions (see Table 2-1), MRI is usually more valuable than CT in detecting a lesion and narrowing the differential diagnosis. The specific choice of imaging modality—including the sequence, orientation, and direction—depends on a combination of the suspected location and the expected pathology. In suspected large-vessel disease, MRA, CTA, and digital angiography may also be considered.
Imaging should be done of the brain and the orbits. Orbital imaging provides details of the optic nerves and the surrounding tissues that are often not detected with brain imaging alone.
When pathology is localized to the orbit, either CT or MRI can provide useful information. Orbital fat produces excellent contrast with the other orbital components on CT and MRI, and both modalities give excellent anatomical localization (see Fig 2-1, 2-2, 2-5).
More important than the choice of imaging modality is the selection of orientation and specific sequences. Direct coronal images are useful in most orbital disorders (Fig 2-15). With MRI, direct coronal imaging is not a problem, but CT may require specific positioning (eg, neck extension) that may be difficult to achieve with older patients. MRI of the orbit should be done with fat-saturation techniques designed to eliminate the high-intensity T1 signal from fat (see Fig 2-7, 2-9D).
