- •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
invasive. These advantages can be helpful not only in research but also in planning neurosurgical procedures to avoid eloquent areas of cerebral function such as those involved with language or vision.
Physiologic information is also available through the use of positron emission tomography (PET) or single-photon emission computed tomography (SPECT). In PET, injection of a radioisotope with a short half-life is followed by imaging of the positrons produced during their decay. The uptake of short-lived radioactive isotopes such as fluorine (18F), carbon (11C), nitrogen (13N), or oxygen (15O) is related to metabolic activity. PET with [18F]-fluoro-2-deoxyglucose has shown increased visual cortex metabolism during ictal visual hallucinations and has detected regions of hypoperfusion in cases associated with visual cortex ischemia. PET is usually combined with CT to enhance resolution. SPECT uses an iodinated radiotracer or technetium-99m as a cerebral perfusion and extraction agent. Because of their biological actions as tracers, these agents reveal patterns of blood flow and cerebral metabolism and thus can be used to study stroke, epilepsy, and dementia. SPECT is more widely available than PET, but it lacks sufficient resolution and specificity. Even after nondiagnostic or normal MRI results, functional imaging may reveal altered regional cerebral blood flow in patients with cerebral visual impairment from various causes.
Sonography
Sonography is an excellent noninvasive technique for imaging the orbit and the carotid arteries. It does not use ionizing radiation and is relatively inexpensive, quick, and office-based; sonography does, however, require expertise in image acquisition and interpretation. The images are based on the reflection of 8–20 MHz ultrasound waves at acoustic interfaces. A particular form of ultrasonography, carotid Doppler is generally accurate at detecting cervical carotid artery stenosis, but it does not provide information about more proximal or distal vessels. The major use of carotid Doppler imaging in neuro-ophthalmology is in detecting cervical carotid artery stenosis in patients with transient monocular blindness suggestive of retinal or optic nerve ischemia. Although carotid Doppler sonography is generally accurate at detecting cervical carotid artery stenosis, it is not accurate at detecting carotid artery dissection. Orbital Doppler sonography is a useful technique for evaluating suspected carotid-cavernous fistula, by detecting a reversal of normally retrograde venous blood flow.
Orbital ultrasound provides useful data concerning the optic nerve and retrobulbar structures, including muscle, optic nerve, and vessels, but it does not provide accurate imaging of the orbital apex. Ultrasound is useful in the globe and may help distinguish disc edema from optic nerve head drusen, which are strongly echogenic (see Chapter 4, Fig 4-13).
Retinal and Nerve Fiber Layer Imaging
Several techniques for noninvasive imaging of the optic nerve head and retinal nerve fiber layer are in clinical use, including Heidelberg retinal tomography (HRT), scanning laser polarimetry (GDx), and optical coherence tomography (OCT). These techniques use laser or short coherence light to image the retina and optic nerve head rapidly and are increasingly being used to evaluate or monitor certain optic nerve diseases such as glaucoma, in addition to being used in research in neuroophthalmic diseases. These techniques are discussed further in BCSC Section 10, Glaucoma.
