- •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 1-2 A, Bony anatomy of the right orbital apex. The optic foramen transmits the optic nerve, ophthalmic artery, and oculosympathetic nerves. The superior orbital fissure, a gap between the greater and lesser wings of the sphenoid bones, transmits CNs III, IV, VI, V1, and the superior ophthalmic vein. B, Intracranial view of the left optic canal. Within the lesser
wing of the sphenoid bone is the optic foramen, which leads to the optic canal. The optic strut separates the optic canal from the superior orbital fissure. C, Anatomy of the orbital apex. The 4 rectus muscles arise from the annulus of Zinn. CNs II, III (superior and inferior branches), VI, and the nasociliary nerve all course through the annulus of Zinn. CN IV and the frontal and lacrimal nerves and the ophthalmic veins are located outside the annulus. D, Anatomical dissection just
anterior to the superior orbital fissure. (Parts A and C illustrations b y Dave Peace; parts B and D courtesy of Alb ert L. Rhoton, Jr, MD.)
The Orbit
The orbit—connected posteriorly to the parasellar region—makes up the anterior aspect of the skull and is composed of 7 craniofacial bones (Fig 1-3):
1.maxillary
2.zygomatic
3.frontal
4.lacrimal
5.sphenoid
6.palatine
7.ethmoidal
Figure 1-3 Bony anatomy of the right orbit. A, The orbital roof is composed of 2 bones: the frontal bone and the lesser wing of the sphenoid bone. The frontal sinus lies within the anterior orbital roof. The supraorbital notch, located within the medial one-third of the superior orbital rim, transmits the supraorbital nerve, a terminal branch of the frontal nerve of the ophthalmic division (V1) of CN V. Medially, the frontal bone forms the roof of the ethmoidal sinus and extends to the
cribriform plate. B, The lateral orbital wall is formed by the zygomatic bone and the greater wing of the sphenoid bone. The junction between the lateral orbital wall and the roof is represented by the frontosphenoid suture. Posteriorly, the wall is bordered by the inferior and superior orbital fissures. The sphenoid wing makes up the posterior portion of the lateral wall and separates the orbit from the middle cranial fossa. Medially, the lateral orbital wall ends at the inferior and superior orbital fissures. C, The orbital floor is composed of 3 bones: the orbital plate of the maxillary bone, the maxillary process of the zygomatic bone and the palatine bone. The nasolacrimal duct sits in the anterior middle area of the orbital floor, medial to the origin of the inferior oblique muscle. D, The medial orbital wall is formed by 4 bones: maxilla (frontal process), lacrimal, sphenoid, and ethmoid. The largest component of the medial wall is the lamina papyracea of the ethmoidal bone. Superiorly, the anterior and posterior foramina at the level of the frontoethmoidal suture transmit the anterior and posterior ethmoidal arteries, respectively. The anterior medial orbital wall includes the lacrimal sac fossa, which is formed by both the maxillary and lacrimal bones. The lacrimal bone is divided by the posterior lacrimal crest. The anterior part of the lacrimal sac fossa is formed by the anterior lacrimal crest of the maxillary bone. (Illustrations b y Dave Peace.)
The orbit is surrounded by several important structures. The 4 paranasal sinuses surround the floor
(maxillary sinus) and the medial wall (ethmoidal and sphenoid sinuses) of the orbit (Fig 1-4). The frontal sinus has a variable relationship to the anterior orbital roof. The other major structures around the orbit are the anterior cranial fossa superiorly (containing the frontal lobe) and the
temporal fossa laterally (containing the temporalis muscle). The roof of the ethmoidal complex, delineated by the frontal ethmoidal suture (top of the ethmoidal bone, or lamina papyracea), marks the inferior boundary of the anterior cranial fossa. It is important to realize that surgical intervention above this anatomical landmark—as occurs, for example, during endoscopic sinus surgery—can result in entry into the anterior cranial fossa or a cerebrospinal fluid (CSF) leak.
Figure 1-4 Coronal (A), sagittal (B), and axial (C) views of the anatomical relationship of the 4 paranasal sinuses to the
orbit. (Illustrations b y Dave Peace.)
The sphenoid sinus forms the medial wall of the optic canal (Fig 1-5). Surgery within the sphenoid sinus has the potential to damage the optic nerve; alternatively, the sphenoid sinus is a surgical route facilitating decompression of the optic chiasm. In approximately 4% of patients, the bone may be incomplete, leaving only mucosa separating the sinus from the optic nerve. The pterygomaxillary area, which contains the sphenopalatine ganglion and the internal maxillary artery, underlies the apex of the orbit. This area communicates posteriorly through the foramen rotundum and the vidian canal to the middle cranial fossa, anteriorly through the infraorbital canal to the cheek and lower eyelid, and superiorly through the inferior orbital fissure to the orbit.
Figure 1-5 Coronal section, anterior view into the sphenoid sinus demonstrating the relationship of the internal carotid artery and optic nerve within the lateral wall of the sinus. (Courtesy of Alb ert L. Rhoton, Jr, MD.)
The orbit is approximately 45 mm wide and 35 mm in maximal height. The total volume of the orbit is approximately 30 cm3. The medial wall is approximately 40 mm from the rim to the optic canal. The medial walls are roughly parallel, whereas the lateral walls form an angle of almost 90°. The orbital rim is made up of the frontal bone superiorly, which connects to the zygomatic bone (at the frontozygomatic suture) laterally. The inferior orbital rim is made up of the zygomatic bone inferolaterally and the maxillary bone inferonasally (meeting at the zygomaticomaxillary suture). Medially, the orbital rim consists of the maxillary and lacrimal bones, which join the frontal bone superiorly. Three additional bones contribute to the orbit: the ethmoidal bone medially, the palatine bone inferiorly in the posterior orbit, and the sphenoid bone laterally and superiorly in the orbital apex.
Canals and fissures
The orbit communicates with the surrounding areas through several bony canals and fissures. Posteriorly, the orbit is contiguous with the cavernous sinus through the superior orbital fissure (see Fig 1-2). The medial wall of the orbit continues as the lateral wall of the sphenoid bone, marking the medial extent of the cavernous sinus. Therefore, when sharp objects enter the medial orbit, they are directed through the superior orbital fissure, where they can lacerate the carotid artery.
The orbit is connected superiorly and posteriorly to the anterior cranial fossa by way of the optic
