- •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
hemorrhages may resemble those associated with diabetes mellitus.
Severe ischemia causes anterior segment changes that may be confused with intraocular inflammation. The patient may have decreased visual acuity; a red, painful eye with episcleral vascular injection; and aqueous flare (ischemic uveitis). Even though neovascularization of the chamber angle and iris is common, intraocular pressure (IOP) may be low, normal, or high. Low or normal IOP in this setting is the result of impaired ciliary body perfusion. Fundus changes may include dilated retinal veins, narrowed retinal arteries with microaneurysm formation, midperipheral dot-and-blot hemorrhages, and macular edema (Fig 5-3). These changes have been termed venous stasis retinopathy (VSR) and may be caused by vascular occlusion anywhere between the heart and the eye.
Figure 5-3 Ocular ischemic syndrome. A, Fundus demonstrates retinal venous dilation, scattered hemorrhages, and mild
optic disc edema. B, Midperipheral dot-and-blot hemorrhages. (Reprinted with permission from John E. Carter, MD. From Carter JE. Panretinal photocoagulation for progressive ocular neovascularization secondary to occlusion of the common carotid artery. Ann Ophthalmol. 1984;16(6):572–576.)
Treatment of ocular ischemic syndrome includes CEA, IOP-lowering drugs, corticosteroids for pain, and panretinal photocoagulation. If the preoperative IOP is low, restoration of blood flow by CEA may precipitate dangerously high IOP. Once the patient incurs signs of chronic hypoperfusion, however, improvement is unlikely. In other patients, carotid occlusion may be too advanced for surgical correction. Early detection is crucial because neovascularization and progressive ocular ischemia occur with prolonged hypoperfusion (see also BCSC Section 12, Retina and Vitreous).
Mizener JB, Podhajsky P, Hayreh SS. Ocular ischemic syndrome. Ophthalmology. 1997;104(5):859–864.
Vasospasm, Hyperviscosity, and Hypercoagulability
TMVL can result from vasospasm of the retinal artery. Patients are generally younger than 50 years, have a strong personal or family history of migraine, and experience stereotypic episodes of painless TMVL that are often severe, even involving complete visual loss. The ocular examination findings are usually normal, but occasionally funduscopic examination reveals constriction of the retinal arteries. Diagnosis of vasospastic TMVL is one of exclusion. The workup should include complete blood count, cardiac evaluation, and carotid imaging. If the patient is older than 50 years, tests for
